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Pulmonary Hypertension Research News RSS Feed
Elusive Origin of Pulmonary Hypertension Uncovered

From Brigham and Women’s Hospital - A new study published in EMBO Molecular Medicine by researchers from Brigham and Women’s Hospital (BWH) sheds light on the disease’s surprising cause, with crucial implications for diagnosis, treatment, and prevention of PH in persons at risk. Read more.

Selexipag (Uptravi) Long-term Outcome Data in PAH Presented

From - Yesterday, key long-term outcome data from Actelion's pivotal selexipag (Uptravi®) Phase III GRIPHON study were shared during an oral presentation at the American College of Cardiology (ACC) Congress in San Diego, Calif. The presentation highlighted that the investigational drug selexipag significantly reduced the risk of a morbidity/mortality event by 40 percent versus placebo (p<0.0001) in patients with pulmonary arterial hypertension (PAH). Read article

Quebec Breathes New Hope into Lives of Those Affected by Pulmonary Arterial Hypertension

(CNW) - The Pulmonary Hypertension Association of Canada (PHA Canada) was pleased to learn that a new treatment option for pulmonary arterial hypertension (PAH) is now being publicly funded in the province of Quebec. On October 1, the Institut national d'excellence en santé et services sociaux (INESSS) recommended that the drug Opsumit (macitentan) be added to the list of drugs covered by the prescription drug insurance plan administered by the Régie de l'assurance maladie du Québec (RAMQ). Read the full press release

PHA Accredits First Six PH Care Centers; Application Process Now Open

We are excited to announce that we have accredited our first six Pulmonary Hypertension Care Centers (PHCC). This initiative aims at improving overall quality of care and ultimately improving outcomes of patients with pulmonary hypertension.

Two types of centers will be accredited: Centers of Comprehensive Care (CCC) - the first six are listed below - and Regional Clinical Programs (RCP). For interested centers, the PHCC Application is now open and available for download. Many PH programs have already submitted their application, and you can track their progress on the Applicant Center page.

Quality Care from Experts in the Field

“The PHCC will give PH patients security in knowing they are receiving quality care from the experts in the field,” said Dr. Murali Chakinala, from the Washington University School of Medicine and PHCC Committee Chair. “Patients can feel confident that the PHCCs have been vetted by a rigorous process that includes a detailed application and a comprehensive site visit with ultimate adjudication by a Committee of more than 20 health professionals with vast experience in caring for PH patients.”

PHA's Scientific Leadership Council and the PHCC Oversight Committee set high parameters for PHCC accreditation. PHCC accreditation signifies that a PH program has demonstrated a dedication to making proper diagnosis and capacity to appropriately and comprehensively manage PH patients through a set of criteria established by the PHA Scientific Leadership Council – 28 global leaders in the field of pulmonary hypertension. These criteria have also been developed with input from many PH stakeholders including physicians, allied health care professionals, patients and PHA leadership and are considered essential to delivering high-quality care for these complex patients.

Patient Registry for Determining Effective Care

Accredited PH Care Centers will also contribute to a PH patient registry that will track diagnostic and treatment patterns at a national level, ultimately demonstrating quality improvement for the care of PH patients.

“The PH patient registry will provide important data on the care and outcomes of patients with pulmonary hypertension across the country,” said Dr. Steven Kawut of the Perelman School of Medicine at the University of Pennsylvania. “We can then begin to understand best practices in the treatment of PH and continue to improve the quality of life and survival of individuals with this disease.”

Linking Centers through a national network will increase collaboration in the PH community, through clinical, quality improvement and investigative partnership.

First Six Accredited Centers

  1. Inova Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Fairfax, Va.
  2. Penn Pulmonary Hypertension / Pulmonary Vascular Disease Program, University of Pennsylvania Health System and Perelman School of Medicine, Philadelphia, Pa.
  3. Kentuckiana Pulmonary Associates, Louisville, Ky.
  4. UC Health Pulmonary Hypertension Program, University of Cincinnati, Cincinnati, Ohio.
  5. Cottage Pulmonary Hypertension Center, Cottage Health System, Santa Barbara, Calif.
  6.  Stanford Pulmonary Hypertension Program, Stanford University, Stanford, Calif.

For more information on PHCC visit or email


Screen Scleroderma Patients Annually for Pulmonary Hypertension

Scleroderma patients should be screened every year for pulmonary hypertension even if they are recently diagnosed and have no respiratory symptoms. Noninvasive tests can catch pulmonary problems early, and treatments are available to slow the progression of scleroderma. Read more and watch the video

Researchers Find New Targets for Treating Pulmonary Hypertension

Jiwang Chen, research assistant professor of critical care medicine, sleep and allergy in the UIC College of Medicine, and his colleagues investigated the molecular mechanisms behind the abnormal proliferation of smooth muscle cells in the pulmonary artery and discovered two ways that the proliferation could be suppressed.

Dr. Chen as well as collaborating author Guofei Zhou have received research grants from PHA in the past. Dr. Roberto Machado, one of the senior authors, is a member of PHA's Scientific Leadership Council and is the PHA Medical Education On-Demand National Chair.

Read article

CHEST Endorses Accreditation of PH Care Centers by PHA


The Pulmonary Hypertension Association and its Scientific Leadership Council have spearheaded a quality improvement initiative to accredit Pulmonary Hypertension Care Centers (PHCC) across the country. These centers are committed to providing high-quality care based on broadly accepted standards of care and consensus guidelines. These centers aspire to improve outcomes of patients with pulmonary hypertension.

The American College of Chest Physicians (CHEST) has endorsed this accreditation process and sees it as another indicator of high quality care are for pulmonary hypertension patients.

“The PHCC are a testament to the growth in research and awareness of pulmonary hypertension,” said Rino Aldrighetti, PHA President and CEO. “We have grown from one treatment in 1996 to 12 in 2014, with more on the horizon. These centers prove that a small rare disease organization can save lives and move mountains in the race toward a cure.”

Currently there are six pilot centers that have gone through the accreditation process. The online application process will open in mid-September 2014. For more information on PH Care Centers, go to


PHA Endorses CHEST Treatment Guidelines

The American College of Chest Physicians (CHEST) recently released guidelines for the management and treatment of pulmonary arterial hypertension (PAH) in adults, Pharmacological Therapy for Pulmonary Arterial Hypertension in Adults: CHEST Guideline and Expert Panel Report, in the CHEST Journal. The guidelines are free to view for clinicians, and provide the latest and most accurate treatment guidelines for PAH.

The Pulmonary Hypertension Association (PHA) endorses these guidelines and sees them as a step in the right direction. “Many times physicians are not clearly informed on the latest ways to treat PAH,” said Rino Aldrighetti, PHA President and CEO. “These guidelines help physicians understand the broad range of therapies available and the need for more research to create more.”

The CHEST Guideline and Expert Panel Report provides 79 recommendations and expert consensus statements aimed at assisting physicians in the management of PAH using the latest drug therapies. The guidelines were reviewed and endorsed by the PHA Scientific Leadership Council.

Read the guidelines

Less Invasive Alternative for Treating Severe Pulmonary Hypertension in Infants

Health e Galaxy - Subcutaneous treprostinil improves outcomes in selected infants with severe pulmonary hypertension and chronic lung disease (CLD) of infancy that have not responded adequately to conservative therapies, researchers say. This is a report of one center’s experience with an extremely small number of carefully selected patients.

One of the co-authors on this paper is Erika Berman-Rosenzweig, MD, a member of PHA's Board of Trustees and Scientific Leadership Council. Read article

Hidden Origins of Pulmonary Hypertension Revealed by Network Modeling

Medical Xpress - In a groundbreaking study, researchers from Brigham and Women's Hospital (BWH) have identified a related family of molecules believed to be a major root cause of pulmonary hypertension, a deadly vascular disease with undefined origins. This is one of the first studies to leverage advanced computational network modeling to decipher the molecular secrets of this complex human disease.

The study is published online June 24, 2014 in The Journal of Clinical Investigation. The senior corresponding author, Stephen Y. Chan, MD, PhD, BWH Divisions of Cardiovascular Medicine and Network Medicine, was a 2010 recipient of PHA's K08 Supplemental Award. Read article

Possible New Type of Targeted Therapy for Pulmonary Hypertension

Reuters - Actelion Pharmaceuticals US, Inc. released today the initial, positive results from the Phase III GRIPHON study of selexipag in 1,156 patients with pulmonary arterial hypertension (PAH). Selexipag is the first non-prostacyclin analogue that acts as a stimulator for the prostaglandin receptor (IP). Read article

New Treatment Fact Sheet on Macitentan

PHA's Scientific Leadership Council recently finalized their official fact sheet on macitentan (Opsumit®).

Read and download the new fact sheet

Reata Enrolls First Patient In Promising, New Pulmonary Arterial Hypertension Trial

BioNews Texas - Irving-based Reata Pharmaceuticals, a privately held company aiming to translate the company’s groundbreaking research into new therapies, has enrolled the first patient in a Phase 2 dose-ranging study that will examine the safety, tolerability, and efficacy of bardoxolone methyl in patients with pulmonary arterial hypertension (PAH).
Read article | Learn more about other PH clinical trials

Bayer HealthCare To Evaluate Riociguat In New Indication - Quick Facts

RTTNews - Bayer HealthCare announced the start of a randomized, double-blind, placebo-controlled Phase IIb study. The RISE-IIP study or Riociguat in patients with Symptomatic Pulmonary Hypertension associated with Idiopathic Interstitial Pneumonias is designed to investigate the efficacy and safety of riociguat in patients with symptomatic pulmonary hypertension or PH associated with idiopathic interstitial pneumonia or IIP.
Read article | Learn more about other PH clinical trials

New Research Links Iraq Dust to Ill Soldiers

USA Today - Titanium and other metals found in dust at a base in Iraq have been linked to the dust found in six sick soldiers' lungs, according to a study set to be released Monday.

"We biopsied several patients and found titanium in every single one of them," said Anthony Szema, an assistant professor at Stony Brook School of Medicine who specializes in pulmonology and allergies.

Titanium and iron are both associated with pulmonary fibrosis and pulmonary hypertension in humans, Szema said. Read article

New Releases of Free CMEs/CEUs on PHA Online University
Physicians and allied health professionals can take advantage of PHA’s medical education hub, PHA Online University, and patients can encourage them to look into its vast resources. As an online initiative that provides long-distance yet accessible medical education for physicians and allied health professionals, PHA Online University supports the need to raise awareness for PH, which, as you know, is oftentimes under-diagnosed or misdiagnosed within the medical community.

Through this online learning platform, physicians and allied health nurses can:
  • Earn free online CME credits/CEUs
  • Find valuable resources provided by PHA, such as information on treatment, research programs, abstracts and presentations from past meetings
  •  Access PHA’s medical journal, Advances in Pulmonary Hypertension
The following are some of our new course releases:

Quality of Life and Palliative Care
Examine the psychosocial determinants of a patient’s quality of life and end-of-life experiences.

Screening Systemic Scleroderma for PAH
Review the classification and staging of pulmonary hypertension, SSc phenotypes, and how to properly screen patients with PAH.

Mechanical Support and RV Failure
Discuss lung transplantation information and the impact of Extracorporeal Life Support among PAH patients.

Pulmonary Hypertension in Sickle Cell Disease
Recognize multifactorial aspects of emodynamic etiology of PH, how patients with SCD have severe impairments in aerobic exercise capacity, and the relationship between anemia and PH.

Clinical Vignettes
PHA Online University is excited to release clinical vignettes that comprise of case studies and provide clinical teachings on various treatments, patient care and a medical understanding of all types of pulmonary hypertension. Cardiology | Psychosocial | Rheumatology

To learn about more course offerings and to explore PH medical education and resources, visit
Renal Dysfunction Affects NT-proBNP Predictive Thresholds in Pulmonary Hypertension

medwireNews - N-terminal pro-brain natriuretic peptide (NT-proBNP) remains a valid predictor in patients with pulmonary arterial hypertension (PAH) who have concomitant renal dysfunction, say researchers. However, they had to use higher than normal thresholds for patients with renal dysfunction, particularly for predicting survival. Read article

Inotropic Agents Support Epoprostenol Initiation in Severe PAH
5/20/2014 - Researchers have published a protocol for use of inotropic agents during initiation of epoprostenol therapy in patients with severe pulmonary arterial hypertension (PAH).Satoshi Akagi (National Hospital Organization Okayama Medical Center, Japan) and colleagues report their experiences using dobutamine and dopamine for haemodynamic support of 46 patients undergoing epoprostenol therapy for World Health Organization functional class IV PAH. Read article

Learn About Pediatric Research from the Researchers

On May 27, meet the researchers who were awarded the inaugural grants for PHA's Robyn J. Barst Pediatric PH Research and Mentoring Fund. Dr. Mehdi Fini and Dr. Melanie Nies will discuss their research and the potential applications for pediatric PH treatments in this live webinar.

Register now for the webinar
Want to learn about the Barst Fund Awardees early? Read their interview on PH Kids | Read more information on the Barst Fund

Triple Therapy Shows Promise for Severe PAH
5/13/2014 - Results of a small pilot study among patients with severe pulmonary arterial hypertension (PAH) support the long-term benefits of upfront triple combination therapy, French researchers report. Read article

Ambrisentan Cleared for Use with Phenprocoumon
5/12/2014 - Starting treatment with ambrisentan does not necessitate adjusting the dose of the oral anticoagulant phenprocoumon in patients with pulmonary hypertension (PH) who require both medications, report researchers.

Read article

News: Northwestern Medicine Study Provides New Insights on Tissue Scarring in Scleroderma
4/25/2014 - A discovery by Northwestern Medicine scientists could lead to potential new treatments for breaking the cycle of tissue scarring in people with scleroderma. Read article

News: Researchers Find “Off” Switch for Scleroderma

University of Michigan researchers are in the news for their role in identifying a signaling pathway that switches on scleroderma, a rare and sometimes fatal disease that causes skin and other tissue to thicken and has no cure.

Read article

PHA's Research Room: Leaving Your Mark in the Future of PH

PHA's International PH Conference and Scientific Sessions includes a Research Room dedicated to helping researchers further their studies by allowing for the collection of data from PH patients, as well as their family and friends. This event gives researchers the rare opportunity to collect data from the largest gathering of patients and families living with PH in the world.

Join this webinar to learn about past Research Room successes, and what individuals planning to participate in the Research Room may expect. While patient participation is valuable, there is often the opportunity for family and friends to participate as well.


  • Eric Austin, MD, MSCI
  • Micheala Aldred, PhD
  • Wendy Chung, MD, PhD
  • C. Gregory Elliott, MD

Register now

World PH Day Webinar on New PH Treatments



Join us April 29 at 12 p.m. ET/9 a.m. PT for Dr. Michael McGoon’s popular annual webinar “PH Treatments: What’s on the Horizon.” This one-hour webinar will cover the new treatments recently approved by the FDA, as well as look at treatments in the pipeline for approval in the coming years.
FDA Meeting Registration Deadline Just Two Weeks Away

On May 13, in White Oak, Md., the Food and Drug Administration will host an informal, “talk show” style meeting where PAH patients can share what it’s really like to live with pulmonary arterial hypertension. FDA will use this information when considering whether to approve PH drugs in the future.

Please help us make a better tomorrow for PAH patients by attending in person or listening in and submitting comments via webcast. Register by April 30.

  1. Register with FDA. Whether you are attending in person or by webcast, you must register on FDA’s website. All members of the PH community are welcome, but PAH patients and the parents of young patients will do the talking. 
  2. Claim your seat on the bus. For those who would prefer not to drive to the FDA campus, buses are available from pick up points in New York, Philadelphia and Maryland.

Don't miss out on this unique opportunity!

Questions? Contact Katie at 301-565-3004 x749.

PROMIS Quality of Life Survey

Vivek Nagaraja, MD and Dinesh Khanna, MD, MSc at the University of Michigan Health System, are conducting a piece of research called PROMIS (Patient Reported Outcomes Measurement Information System) in rheumatology. The research involves an online survey catered to patients with various rheumatologic conditions. Interested patients are highly encouraged to take this survey as it provides important information about the burden of their rheumatologic condition on different aspects of life. This survey is completely voluntary and confidential.

Click here to take the survey:

View further instructions on taking the survey:

Bayer Begins Riociguat Trial for Resistant PAH

healio - Bayer HealthCare announced enrollment of the first participant in the phase 3b RESPITE study. The open-label, multicenter, international pilot study will assess the soluble guanylate cyclase stimulator riociguat (Adempas) in patients with pulmonary arterial hypertension who are resistant to treatment with phosphodiesterase-5 (PDE5) inhibitors.

Read article

What Do You Want from New PAH Drugs?

The Food and Drug Administration is making changes in how they approve drugs. For the first time ever, they are asking, “What do patients want from new drugs?”

Hundreds of disease communities have asked for a chance to answer that question. PAH is one of only sixteen diseases selected by the FDA for this discussion. Please help us make a better tomorrow for PAH patients by attending in person or listening  in and submitting comments via webcast on May 13.

Take these two steps to RSVP for this exciting opportunity:

  1. Register with the FDA. Whether you are attending in person or by webcast, you must register on FDA’s website. All members of the PH community are welcome, but PAH patients and parents of young patients will do the talking.
  2. Claim your seat on the bus. For those who would prefer not to drive to the FDA campus, buses are available from Maryland, New York and Philadelphia. Complete this form to claim your seat on the bus.
Yale Study Offers Insight Into Function of Cells Linked to PH

Science Codex - Most of us draw roughly 25,000 breaths a day without any thought. But for patients with pulmonary hypertension, a life-threatening increase in blood pressure in the lungs, even the smallest task can leave them gasping for air. A new study by researchers at Yale School of Medicine offers insight into the function of cells linked to this incurable and often fatal illness.

Read article

Improved Right Heart Imaging Approved by FDA

The Food and Drug Administration (FDA) has approved a device called the VMS™ heart analysis system which can be used in echocardiography allowing your physician to better measure right heart volumes and function.
Meet the Barst Pediatric Research Fund Awardees

In 2012, PHA established the Robyn J. Barst Pediatric PH Research and Mentoring Fund in memory of Dr. Barst, a pioneer in the field of pediatric pulmonary hypertension research and treatment.

On May 27, 2014, the 2014 grantees, Dr. Mehdi Fini and Dr. Melanie Nies, will discuss their research and the potential applications for pediatric PH treatments in this live webinar.

Dr. Mehdi Fini has been awarded the Cordelia's Pediatric Mentoring and Research Grant through the Barst Fund.

Dr. Melanie Nies has been awarded the Matthew and Michael Wojciechowski Pulmonary Hypertension Pediatric Proof-of-Concept Grant through the Barst Fund.

Register now for the webinar! 

Want to learn about the Barst Fund Awardees early? Read their interview on PH Kids | Read more information on the Barst Fund

Discovery of Genetic Mutations that Cause PCH

A team of researchers, led by physicians and scientists at Intermountain Healthcare's Intermountain Medical Center and ARUP Laboratories, has made a medical breakthrough by discovering genetic mutations that cause a rare and deadly lung disease.

The disease, pulmonary capillary hemangiomatosis or PCH, is a rare cause of pulmonary hypertension, which occurs predominantly in young adults. PCH affects less than one in a million people, and has been extremely difficult and expensive to diagnose, as well as challenging to treat. Read PHA's full release

Chest Radiography Highly Predictive of Pulmonary Hypertension

( - Chest radiography is very accurate for predicting pulmonary hypertension (PH), report researchers. “We felt inappropriate to rule in a radiologic diagnosis of PH only on the basis of a prominent main pulmonary artery,” the researchers write in Thrombosis Research. For this reason, they required the presence of at least one additional finding for a PH diagnosis: isolated enlargement of the right ventricle; a right descending pulmonary artery diameter greater than 16 mm; or pruning of peripheral pulmonary vessels. “By adopting these criteria, chest radiography yielded high diagnostic accuracy for PH,” they report. Read article

In the News: ECG Abnormalities Progress During Course of PAH

(medwireNews) - Researchers have identified a number of electrocardiography (ECG) variables that progress between diagnosis and death in patients with pulmonary arterial hypertension (PAH). Their study “suggests potential new ECG predictors of outcome that deserve further investigation,” say Adriano Tonelli and colleagues, from the Cleveland Clinic in Ohio. Read article

Riociguat Approved in Japan

(MarketWatch) Bayer recently received approval for their pulmonary hypertension drug riociguat (Adempas) in Japan. Read article

Another Piece of the Pulmonary Hypertension Puzzle gets Plugged into Place

(Stanford Medicine Scope Blog) - Pulmonary hypertension, a dangerous increase in the pressure of blood vessels in the lung, is one nasty disease, as I wrote in a Stanford Medicine article a few years ago. As many as three times as many women – many of them quite young – as men are diagnosed with the spontaneous form of PH (which can also arise from scleroderma or bad pharmaceuticals).

But recent work has strongly implicated inflammation in pushing predisposed tissues over the edge into the diseased state. And this week, a Journal of Experimental Medicine study led by PH specialist Marlene Rabinovitch, MD, and her colleagues at Stanford’s PH-focused Vera Moulton Wall Center plunks a potentially pivotal piece of the puzzle into place. Rabinovitch and her associates showed that levels of a pro-inflammatory growth factor usually designated by the acronym GM-CSF (if you really must know, it’s “granulocyte-macrophage colony stimulating factor”) rise substantially when a cell-surface receptor with a heavyweight acronym of its own, BMPR2 (for “bone morphogenic protein receptor”) isn’t functioning properly. That can be due to mutations in the gene that codes for the receptor (as occurs in familial versions of PH), to various environmental causes, or the interaction of the two. Read article

New Research May Boost Drug Efficacy

(From - The development of new, more effective vasodilators to treat pulmonary arterial hypertension (PAH) has been hampered because of their systemic toxicity and adverse side effects. An international team of investigators seeking to surmount these problems and increase drug efficacy have determined that a vascular homing peptide can selectively target hypertensive pulmonary arteries to boost the pulmonary but not systemic effects of vasodilators. Importantly for potential clinical use, this peptide retains its activity when given sublingually. The results using a rat model of PAH are published in the American Journal of Pathology. Read article

New Pulmonary Hypertension Drug Approved!

Friday, the U.S. Food and Drug Administration (FDA) approved United Therapeutics' oral treprostinil, an extended-release tablet commercially known as Orenitram, for the treatment of pulmonary arterial hypertension (PAH). Orenitram is the first orally administered prostacyclin analogue approved by the FDA for any disease.

"PHA is always pleased as new treatments become available. They increase options for patient treatment, both individually and in combination with others," says PHA President Rino Aldrighetti. "This is the twelfth treatment for PAH, the third new therapy to be FDA approved in the last two months. During the past 10 years, the average number of drugs approved per year for all rare diseases is fewer than thirty. To have this kind of advancement for PAH is extraordinary and speaks to the medical and research structure that has been built in this field."

Most Effective Use of Treatments Requires Early Diagnosis

While huge strides have been made in the advancement of therapeutic options, the time it takes to reach a correct PH diagnosis has not changed over the last 20 years. Many patients will see three or more different physicians over a three year period before they are properly diagnosed with PH, and almost three-fourths have advanced PH by the time they are diagnosed.

“Misdiagnosis and delayed diagnosis have an impact on patient survival and quality of life,” explains PHA Senior Director of Volunteer Services Debbie Castro. "Because PHA sees early diagnosis as a huge priority, we are working with medical professionals and patients to improve diagnosis through our early diagnosis campaign, Sometimes it’s PH."

More Needs to be Done for Children

While there are 12 treatments for pulmonary arterial hypertension (PAH), none are specifically approved for pediatric patients. PHA has launched the only pediatric PH research program in the United States, the Robyn Barst Pediatric Research and Mentoring Fund.

And, says Rino, “Ultimately, our goal is a cure. With increasing numbers of researchers engaged in more studies every year, we are hopeful and encouraged as we see that hope turning into action.”

Learn more: Read article | PH treatments in the U.S. | PH treatments worldwide | Sometimes it’s PH website | Robyn Barst Pediatric Research and Mentoring Fund

Our Scientific Leadership Council is working on a forthcoming treatment fact sheet with more details. When completed the fact sheet will be available here:

New Mitochondrial Research Promising for PH

(Medical Xpress) Queen's University [in Canada] professor Stephen Archer's research has revealed that in serious human diseases (such as pulmonary hypertension, lung cancer, cardiac arrest and neurologic disease) the cell's power source, known as mitochondria, displays an abnormal structure.

Dr. Archer discussed his findings in a recent New England Journal of Medicine article.

"This explosion in the understanding of the once secret lives of mitochondria will almost certainly advance our understanding of an important mechanism for cell death and cell growth," says Dr. Archer. "This basic understanding offers new molecular targets for therapies of neurologic diseases, cancer and pulmonary hypertension." Read the full article

Angina Drug Shows Promise for Type of Pulmonary Hypertension

(MedPage Today) The anti-angina agent ranolazine (Ranexa) may work in a condition for which there are few options in terms of drug therapy: pulmonary hypertension (PH) tied to heart failure with preserved ejection fraction (HF-pEF), researchers found. Read article

Pulmonary Hypertension Spotlighted at CHEST

Five staff members represented the Pulmonary Hypertension Association at this year’s meeting of the American College of Chest Physicians.

  • Rino Aldrighetti, President & CEO
  • Carl Hicks, Executive Vice President
  • Lisa Beth Gansberg, Executive Director, Midwest Chapter
  • Michael Patrick Gray, Director, Medical Services
  • Briana Rivas-Morello, Patient Education Associate

Many physicians with whom PHA works closely gave talks on various diagnostic and management issues related to PH to a particularly receptive audience, given the increased PH presence at this year’s meeting. Read this MedPage Today article on how pulmonary hypertension was in the spotlight at CHEST

New Criteria Could Lead to Earlier Diagnosis of Scleroderma

(National Pain Report) A new classification criterion for systemic sclerosis, a chronic autoimmune disease also known as scleroderma, could help physicians diagnose and begin treating patients sooner. The new criteria, developed by a joint committee formed by the American College of Rheumatology (ACR) and the European League Against Rheumatism, are published in the ACR journal, Arthritis & Rheumatism. They are the first changes in the criteria since 1980. Read article

Eleventh PH Drug Approved, The Second in Just Ten Days

Today the U.S. Food and Drug Administration (FDA) approved the eleventh pulmonary arterial hypertension (PAH) treatment in the U.S., Actelion Pharmaceutical’s macitentan (commercially known as Opsumit), an oral dual endothelin-receptor antagonist. This comes just ten days after the approval of riociguat (commercially known as Adempas) for the treatment of PAH and some cases of chronic thromboembolic pulmonary hypertension (CTEPH). These two new drugs are the first in five years to be approved by FDA for treating PH.

According to Healio, the phase 3 SERAPHIN drug trial for macitentan was the first to test a PAH drug for its effects on morbidity and mortality. Patients who took the drug showed lower rates of morbidity and mortality as compared to patients who received a placebo, showed improved six-minute walk tests and more showed improvement in their WHO functional class.

Acknowledging the achievement of adding an eleventh drug to the choices for PAH patients in a rare disease state, PHA President Rino Aldrighetti said, “Of the 7,000 rare diseases, only three have as many or more treatments available. We are fortunate to have a strong and growing research community with great communication and cooperation. That has helped foster industry interest in exploring new options for patients.”

PHA itself has committed more than $12.5 million to support cutting-edge research focused on pulmonary hypertension. PHA has been able to leverage such substantial funding through partnerships with the National Heart, Lung, and Blood Institute (NHLBI), American Thoracic Society (ATS), American Heart Association (AHA), and Pfizer.

We know that your ultimate wish is for a cure, and that is why PHA will continue to support the work of researchers until the day when no one suffers from PH.

Learn more: FDA press release | macitentan study findings | PH treatments in the U.S. | PH treatments worldwide | PHA Research Program

Researchers Identify Possible Treatment Avenues for Scleroderma

(Science Codex) Using mice, lab-grown cells and clues from a related disorder, Johns Hopkins researchers have greatly increased understanding of the causes of , showing that a critical culprit is a defect in the way certain cells communicate with their structural scaffolding. They say the new insights point the way toward potentially developing drugs for the disease, which affects approximately 100,000 people in the United States. Read article

New Drug Approved for PAH and CTEPH Patients in U.S.

On Tuesday, the U.S. Food and Drug Administration (FDA) approved Bayer’s riociguat pill for the treatment of pulmonary arterial hypertension (PAH) and for chronic thromboembolic pulmonary hypertension (CTEPH).  Riociguat, commercially known as Adempas, will be used for CTEPH patients after pulmonary thromboendarterectomy (PTE) surgery or if surgery is not possible. This will be the first drug available for CTEPH patients and tenth for PAH.

PHA President Rino Aldrighetti says, “Treatment opportunities for patients are growing rapidly. Eighteen years ago there were no treatments for pulmonary arterial hypertension. Twelve years ago there was one. Today there are ten, with a possible eleventh later this year (Actelion’s macitentan). Of the 7,000 rare diseases, only three have as many or more treatments available. We are fortunate to have a strong and growing research community with great communication and cooperation. That has helped foster industry interest in exploring new options for patients.”

Better Prognosis for PH Patients

The strong progress in the field of pulmonary hypertension over the past two decades has led to a better prognosis for PH patients. Whereas physicians treating PH once delivered terminal diagnoses, they now expect their patients to live with their condition. Many patients survive for years -- even 10-20 years or more -- with proper PH treatment.

Patients Not Receiving Treatments in a Timely Manner

“The problem we now face is getting these treatments to patients sooner. While we have made huge strides over the past 20 years, the time it takes to reach a correct PH diagnosis has not changed,” explains PHA Vice President for Community Engagement Mollie Katz. “With missed or late diagnosis, the disease continues to get worse, affecting patients’ survival and quality of life. Many patients will see three or more different physicians over a three year period before they are properly diagnosed with PH, and almost three-fourths have advanced PH by the time they are diagnosed. Because PHA sees early diagnosis as a huge priority, second only to a cure, we are working with medical professionals and patients to improve diagnosis through our early diagnosis campaign, Sometimes it’s PH. 

Not There Yet

While there are 10 treatments for pulmonary arterial hypertension (PAH) and now one for CTEPH, there are none approved for pediatric patients. PHA has launched the only pediatric PH research program in the United States, the Robyn Barst Pediatric Research and Mentoring Fund.

And, says Rino, “Ultimately, our goal is a cure. With increasing numbers of researchers engaged in more studies every year, we are hopeful and encouraged as we see that hope turning into action.”

Learn more: FDA decision | riociguat study findings | PH treatments in the U.S. | PH treatments worldwide | Sometimes it’s PH website


Galectin-3 Strong Prognostic Marker in Patients with Pulmonary Hypertension

(Healio) Patients with pulmonary hypertension and elevated galectin-3 were more likely to have a higher mortality rate at 4 years compared with patients with lower levels of the biomarker, according to data presented at the Annual Scientific Meeting of the Heart Failure Society of America. Read article

Epoprosenol for Injection Now Available in Canada

This month, CARIPUL® (epoprostenol) for Injection became available in Canada by Actelion Pharmaceuticals Canada Inc. for the treatment of idiopathic pulmonary arterial hypertension (IPAH) and pulmonary hypertension secondary to scleroderma spectrum of diseases in NYHA functional Class III and IV patients who have not responded adequately to conventional therapy. Read article

Blocking Molecular Pathway Reverses Pulmonary Hypertension in Rats

A new study from researchers at the Stanford University School of Medicine published recently in Science Translational Medicine suggests that pulmonary hypertension may be caused by an inflammation-producing molecular pathway that damages the inner lining of blood vessels. The results suggest that medications to block this pathway could lead to a possible cure for pulmonary hypertension as well as a better understanding of other diseases involving inflammation of blood vessels, such as coronary artery disease. Read article

Macitentan Trial Shows Reduced Morbidity and Mortality in Pulmonary Hypertension Patients

(Healio) - The phase 3 SERAPHIN drug trial for macitentan showed lower morbidity and mortality rates in pulmonary hypertension patients than those who received the placebo. Macitentan, which will be marketed as Opsumit if it is approved by the FDA, is an investigational dual endothelin-receptor antagonist being developed by Actelion Pharmaceuticals. Read article

Study findings were presented at the European Society of Cardiology (ESC) this past weekend and will be presented at the European Respiratory Society (ERS) Annual Congress in Barcelona, Spain, on Sept. 9 and 10. Learn more

Read about PH drugs approved in the U.S. | PH treatments around the world

Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study

Read a study about the DETECT algorithm for PAH detection in systemic sclerosis. This algorithm is a sensitive, non-invasive tool that minimizes missed diagnoses, identifies milder disease and addresses resource usage. Read the study

Pulmonary Hypertension in U.S. Veterans Underdiagnosed

A study in the Journal of the American College of Cardiology (JACC) reported that severe pulmonary hypertension is under recognized and associated with substantial mortality in U.S. veteran patients. Veterans show elevated rates of cardiopulmonary diseases that promote PH. The study recommends enhanced awareness among clinical cardiologists about PH diagnostic testing. Read article

Research on Two New Pulmonary Hypertension Drugs Presented at ATS

The American Thoracic Society recently held their annual conference and evidence from studies on two new pulmonary hypertension drugs in the pipeline were presented.

Riociguat increased walk distance by pulmonary arterial hypertension patients. Read article

Macitentan improves health-related quality of life in pulmonary arterial hypertension (PAH) and reduces PAH-related hospitalizations. Read article 

Race, Sex Differences May Affect Response to PAH Drugs

In a recent study analyzing 1,130 patients with PAH who were enrolled in one of the original clinical trials of endothelin receptor antagonists (ERAs), it was found that there were distinct response to treatment between men and women and patients of different race. Read article

Fetal Growth Restriction and Pulmonary Hypertension in Premature Infants with Bronchopulmonary Dysplasia

Study findings published in the Journal of Perinatology...

Birth weight for gestational age is an important predictor of pulmonary hypertension in premature infants with moderate to severe bronchopulmonary dysplasia. Our findings contribute to the growing evidence supporting fetal mechanisms of later onset pulmonary vascular disease. Read more

Relationship Between Serum Uric Acid Levels and Patient Conditions and Prognosis in Idiopathic Pulmonary Arterial Hypertension

Study findings in

The serum level of uric acid correlates significantly with patient conditions and prognosis in IPAH. And a higher serum level of uric acid predicts worse conditions and prognosis. Read more

Link Between Abnormal Bone Marrow Cells and Pulmonary Arterial Hypertension

Investigators have uncovered a new clue to the cause of pulmonary arterial hypertension, a progressive and frequently lethal disease that in many cases arises mysteriously. A report published in June 2012 describes findings suggesting that bone marrow-derived endothelial progenitor cells play a role in causing the vascular injury in the lung that underlies the disease.

This study was released by the National Heart, Lung, and Blood Institute (NHLBI) on a list of their top research findings of 2012. Read More

Pulmonary Hypertension Could Be Late Effect of Childhood Cancer Therapy

(Doctors Lounge) - Gregory T. Armstrong, MD, of St. Jude Children's Research Hospital in Memphis, Tenn., and colleagues conducted a cross-sectional assessment involving 498 adult survivors of childhood cancer to examine the prevalence of pulmonary hypertension, a late effect of cancer therapy. Read article

Disruption of Cellular Signaling Identified in Pulmonary Arterial Hypertension

(Medical Xpress) - Impairment of a key signaling cascade in the pulmonary blood vessels plays an important role in pulmonary arterial hypertension (PAH), a Yale study has found.

Recent studies have described the role of the gene apelin in the signaling process that maintains normal pulmonary vascular function. Apelin levels have been found to be significantly reduced in patients with PAH. Read article

FDA Accepts Actelion's Filing for PAH Drug Opsumit

(PharmPro) - Actelion announced that the Food and Drug Administration (FDA) accepted the company's filing seeking approval of Opsumit (macitentan) for the treatment of patients with pulmonary arterial hypertension (PAH). Read article

Silencing of STIM1 Attenuates Hypoxia-induced PASMCs Proliferation via Inhibition of the SOC/Ca2+/NFAT Pathway

(7thSpace Interactive) - Stromal interaction molecule 1 (STIM1) is a newly discovered Ca2+ sensor on the endoplasmic reticulum which is an indispensable part in the activation of store-operated Ca2+ channels (SOC). Recent studies demonstrate that SOC of pulmonary smooth muscle cells (PASMCs) were upregulated by chronic hypoxia which contribute to the enhanced pulmonary vasoconstriction and vascular remodeling. Read article

Bayer to Present Data on Investigational Pulmonary Hypertension Compound at CHEST 2012

(Herald Online) - Bayer HealthCare announced today that data on its investigational pulmonary hypertension compound riociguat (BAY 63-2521) will be presented in a scientific forum at the American College of Chest Physicians (ACCP) annual meeting October 20 – 25, in Atlanta, GA.  Data from two pivotal, Phase III trials researching riociguat in patients with for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) will be presented as late-breaking abstracts in oral presentation sessions. Read article

Actelion Submits a New Drug Application to the FDA for Macitentan (Opsumit) for the Treatment of Patients With Pulmonary Arterial Hypertension

(Reuters) - Actelion today announced that it has submitted a New Drug Application (NDA) to the US Food and Drug Administration (FDA) seeking approval for macitentan (Opsumit®) for the treatment of patients with pulmonary arterial hypertension. Read article

Breakthrough Could Help Sufferers of Pulmonary Arterial Hypertension

(The University of Sheffield News) - For the first time scientists have found an antibody treatment that not only stops PAH getting worse, but also reverses the condition in mice and rats. The research was funded by the British Heart Foundation and the Medical Research Council (MRC) and is published in the Journal of Experimental Medicine. Read article

17-Beta Estradiol Attenuates Hypoxic PH VIA Estrogen Receptor-Mediated Effects

In a study of 17-Beta Estradiol (E2) and its effects in hypoxia-induced pulmonary hypertension (HPH), results indicate that in an environment characterized by low oxygen concentrations, E2 may exert beneficial effects on the pulmonary artery and right ventricle.  Read more

New Treatment Target Brings Hope for Scleroderma Patients

(News-Medical.Net) - Research presented at the European Academy of Dermatology and Venereology in Prague, Czech Republic, suggests that inhibition of the SMAD protein may be a viable therapeutic target in scleroderma patients. Read article

Revatio (sildenafil): Drug Safety Communication - Recommendation Against Use in Children

The FDA has issued a decision regarding sildenafil (Revatio) for pediatric usage.  More information can be found in FDA's Medwatch and Pfizer's statements (PDF)

In addition, PHA's Scientific Leadership Council will be releasing a statement in the near future.

Older Women May Be at Risk for Pulmonary Hypertension

(Medpage Today) - Of patients with systemic hypertension but preserved ejection fraction, those with pulmonary hypertension tended to be older and female, a retrospective study found. 72% of the patients with PH were women compared with 49% of those without PH, reported Monica Mukherjee, MD, from George Washington University in Washington, D.C., and colleagues. Moreover, the mean age of those with PH was 77, compared with 64 in those without PH, according to Mukherjee's presentation at the American Society of Echocardiography meeting. Read article

Actelion Pharmaceuticals Announces U.S. FDA Approval of Supplemental New Drug Application for Second Generation VELETRI(R) (epoprostenol) for Injection

(Market Watch) - Actelion Pharmaceuticals US, Inc. announced today that the U.S. Food and Drug Administration (FDA) approved their supplemental new drug application (sNDA) for second generation VELETRI, for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity. Read article

VentriPoint Multi-Centre Clinical Trial Focuses on Ultrasound Diagnostic Tool for Heart Analysis

(Sys-Con Media) - VentriPoint Diagnostics Ltd. is pleased to announce that Mayo Clinic has joined its multi-centre clinical trial to evaluate the use of an ultrasound diagnostic tool (the VMS(TM) heart analysis system) for patients with pulmonary arterial hypertension (PAH). Robert Frantz, M.D., a consultant in the Cardiovascular Division and Associate Professor of Medicine, College of Medicine, Mayo Clinic is the lead investigator for the clinical trial site in Rochester, MN. Read article

Inhibition of PBEF is a Possible Therapeutic Target for Pulmonary Hypertension

(Science Codex) - Inhibition of pre-B Cell Colony-Enhancing Factor (PBEF) could be a potential therapeutic target for pulmonary hypertension (PH), according to a preclinical study in an animal model of PH. Read article

Mylan Canada Receives Health Canada Approval for Generic Version of Tracleer®

(Canada Newswire) - Mylan Inc. today announced that its Canadian subsidiary, Mylan Pharmaceuticals ULC, has received approval from Health Canada for Mylan-Bosentan Tablets, 62.5 mg and 125 mg. Mylan-Bosentan is the generic version of Actelion Pharmaceuticals Ltd.'s Tracleer®, used for the treatment of pulmonary arterial hypertension. "Providing Canadians access to high quality, affordable medicines is our primary goal," said Dick Guest, Mylan Canada's President and CEO. "By providing access to more affordable generic drugs, Mylan helps to reduce the burden on our health care system, benefiting all Canadians." Read article

Promising New Research May Have Impact on Scleroderma Population

(Scleroderma Foundation) - A member of the Scleroderma Foundation's Board of Directors has led a team of researchers who have identified a peptide that can block fibrosis of the skin and lungs, according to a report published today. "Lung fibrosis currently is the number one cause of death in patients with scleroderma," said the report's senior author Carol A. Feghali-Bostwick, Ph.D. "Identifying a way to stop this process from happening could have enormous impact on mortality and quality of life." Read article

Scleroderma Malignancy Risk Linked to Antinuclear Antibodies

5/16/2012 (Internal Medicine News) - The presence of specific autoantibodies may help to predict which patients with systemic sclerosis are likely to develop cancer within a few years of their diagnosis, according to the findings of a U.K.-based registry study. Read article

The Trajectory to Diagnosis With Pulmonary Arterial Hypertension: A Qualitative Study

(BMJ Open) - Funded by Pulmonary Hypertension Association UK, objectives of this study were to investigate the patient's experience of the trajectory to receiving a diagnosis of pulmonary arterial hypertension (PAH) and inform the provision of care for this patient group. Participants were interviewed in their own homes across England.

Conclusions: Limited awareness of PAH outside specialist centres leads to misdiagnosis, delays in treatment and an increasing sense of anger, frustration and confusion for many patients. Repeated tests and visits to numerous medical professionals, including GPs and cardiac or respiratory consultants over a number of years, represents a significant use of resources and consequent cost to the NHS. Read Article

Culprit Responsible for Severe Systemic Scleroderma Complications in African-Americans Found

(EurekAlert) - A new analysis finds that compared to Caucasians, African-Americans with systemic scleroderma have more antibodies in the blood that are linked to severe complications and an increased likelihood of death. They say this finding, published today in Arthritis & Rheumatism, suggests physicians can use these disease markers to screen and treat scleroderma patients proactively. Read article

VentriPoint Launches Clinical Trial in Pulmonary Arterial Hypertension (PAH) With Top PH Centres in the United States and Canada

(SYS-CON Media) - VentriPoint Diagnostics today announced the commencement of the pivotal clinical trial to demonstrate that the VentriPoint heart analysis system is equivalent to cardiac MRI in patients with pulmonary arterial hypertension (PAH). The trial will be lead by Dr. Robyn Barst. Read article

Actelion's Macitentan Meets Primary Endpoint in Pivotal Phase III Seraphin Outcome Study in patients with pulmonary arterial hypertension

(Reuters) - Actelion announced today that initial analysis indicates that the pivotal, long-term, event-driven study SERAPHIN with macitentan, a novel dual endothelin receptor antagonist, in 742 patients suffering from pulmonary arterial hypertension (PAH) and treated for up to three and a half years, has met its primary endpoint. Read article

Webinar for Medical Professionals: 17-Beta Estradiol Attenuates Hypoxic PH via Estrogen Receptor-Mediated Effects

4/23/2012 - PHA Online University Webinar Thursday, May 10, 2:00 p.m. ET/11:00 a.m. PT
In this webinar, Tim Lahm, MD, from Roudenbush VA Medical Center in Indianapolis, Ind., will discuss his recent research involving 17-Beta Estradiol and the role it plays in tempering hypoxic pulmonary hypertension.
Register now

Scientists Lead Trek to Investigate High-Altitude Maladies

4/19/2012 - (Castanet) - Canada’s international scientific expedition to Mount Everest is preparing to depart for Nepal on Sunday April 22. Everest 2012, a six-week, six-country research investigation into the effects of heart and brain blood flow and oxygen deprivation at high altitudes, will settle into the Pyramid Laboratory near Everest base camp for a six-week stay through the end of May. Expedition members will themselves be the test subjects for the experiments. Some experiments, like one measuring pulmonary arterial hypertension, involves invasive minor surgery to place arteriovenous shunts in wrist and neck arteries to measure acute hypoxia (oxygen deprivation) following ascent to high altitude. Read article | Follow the Everest expedition

New Data Shows GeNO LLC Nitric Oxide Delivery System Provided Nitric Oxide With Low Levels of Nitrogen Dioxide for Heart Transplant Patient Evaluation

(Yahoo Finance) - New data presented today studied the safety of the GeNO  system for delivering inhaled nitric oxide (NO) to patients being evaluated for cardiac transplant. The study evaluated 10 heart failure patients who underwent RHC prior to cardiac transplant or LVAD implant. Read article

Pulmonary Hypertension Predicts Heart Failure After Acute MI

(DoctorsLounge) - Pulmonary hypertension (PH) is a strong independent predictor of heart failure in patients with acute myocardial infarction (AMI), according to a study published in the May 1 issue of The American Journal of Cardiology. Read article

Lower Dose of Thrombolytics can Treat Moderate Pulmonary Embolism

(News-Medical) - Pulmonary embolism is estimated to cause over 100,000 deaths each year in the U.S. Although thrombolytics, or "clot-buster" drugs, are currently reserved to treat only the most severe cases of pulmonary embolism, new data suggest that when used at lower doses, these drugs are also safe and effective for more common, moderate cases of pulmonary embolism, according to research presented today at the American College of Cardiology's 61st Annual Scientific Session. Read article

Studies Shed New Light on how Nitric Oxide is Produced

1/20/2012 (Vanderbilt University Medical Center) - Studies that started as part of an exploratory collaboration for Judy Aschner, M.D., took an unexpected turn and have led to a whole new understanding of how nitric oxide — an important signaling molecule in the body — is produced. “I think we’re on the cusp of something that could change the face of how we treat infants at risk for chronic lung disease or with chronic forms of pulmonary hypertension,” Aschner said. Read article

Pulmonary Hypertension Registry IDs Features of Pediatric PH

1/11/2012 (Doctors Lounge) - An analysis of data from the Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension registry has identified clinical features specific to the care of children with pulmonary hypertension, according to research published online Jan. 11 in The Lancet. Read article

Heart Rate Recovery Predicts Clinical Worsening in Pulmonary Hypertension

11/18/2011 (EurekAlert) - Heart rate recovery at one minute after a six-minute walking distance (6MWD) test is highly predictive of clinical worsening and time to clinical worsening in patients with idiopathic pulmonary arterial hypertension (IPAH), according to a new study. Read article

New Guidelines for Treating Pulmonary Hypertension fnd Venous Thromboembolism

11/4/2011 (URMC) - URMC doctor R. James White, M.D., Ph.D., helps establish new guidelines for treating pulmonary hypertension and venous thromboembolism. Watch the video

UK (University of Kentucky) Healthcare Surgeons First to Perform Novel Procedure for Transplant Patient

10/29/2011 (KyForward) - The patient “feels like a miracle,” after surgeons at UK HealthCare recently became the first ever to perform two specific procedures together as a bridge to lung transplantation. Read article

CHEST: Study Looks at Sildenafil Dose for Youth With PAH

10/24/2011 (Doctors Lounge) - Medium doses of sildenafil may be beneficial for children with pulmonary artery hypertension, according to a study presented at the annual meeting of the American College of Chest Physicians, held from from Oct. 22 to 26 in Honolulu. Read article

FDA Drug Safety Communication: Sprycel (dasatinib) and Risk of Pulmonary Arterial Hypertension

10/11/2011 (FDA) - The U.S. Food and Drug Administration (FDA) is warning the public that the leukemia drug Sprycel (dasatinib) may increase the risk of a rare, but serious condition in which there is abnormally high blood pressure in the arteries of the lungs (pulmonary arterial hypertension [PAH]). Information about this risk has been added to the Warnings and Precautions section of the Sprycel drug label. Read article

No Need to Perfuse Brain in Pulmonary Endarterectomy

(MedPage Today) - During pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension, continuously maintaining brain perfusion provides no advantage in cognitive function compared with periods of deep hypothermic circulatory arrest of up to 20 minutes, a randomized trial showed. Read article

Women with Pulmonary Arterial Hypertension Have Greater Response to Treatment than Men, Penn Study Finds

9/27/2011 (Penn Medicine) - Pulmonary arterial hypertension (PAH) patients of different sexes and races may respond differently to treatment with commonly used medications for the disease, says a new study from researchers at Perelman School of Medicine at the University of Pennsylvania. The results of the study are published online ahead of print in the journal CHEST. Read article

Sildenafil Appears safe and Effective for Eisenmenger Syndrome

9/23/2011 (Heartzine) - In a study published by the journal Heart, researchers based in China led by Professor Zhi-Cheng Jing of Tongji University School of Medicine report that over a 12 month period, sildenafil treatment (oral administration) for those with Eisenmenger syndrome (a shunt-related congenital heart defect which leads to PH) was both well tolerated and appeared to improve key performance measures. Read article

Novartis Study Shows QTI571 Significantly Improved Walking Distance in Patients with Life-Threatening PAH

9/26/2011 (Pipeline Review) - Novartis announced new data today from the pivotal Phase III IMPRES clinical trial showing that the investigational therapy QTI571 (imatinib) significantly improved exercise capacity in patients with pulmonary arterial hypertension (PAH) after 24 weeks compared with placebo(1). Evidence indicates that QTI571 targets an underlying cause of PAH by counteracting uncontrolled growth of arterial smooth muscle cells(2). Read article

Estrogen Treatment May Help Reverse Severe Pulmonary Hypertension

9/15/2011 (eurekalert) - UCLA researchers have found that the hormone estrogen may help reverse advanced pulmonary hypertension. Published in the Sept. 15 issue of the American Journal of Respiratory and Critical Care Medicine, the preclinical study shows that in rats, estrogen treatment can reverse the progression of pulmonary hypertension to heart failure and can restore lung and ventricle structure and function. Read article

New Study Will Explore Impact of Exercise on Pulmonary Hypertension

8/26/2011 (Univ. of Wisconsin) - Funded by a four-year, $2.5 million grant from the National Institutes of Health, Naomi Chesler, an associate professor of biomedical engineering at UW-Madison, will investigate the relationships between small artery narrowing, large artery stiffening and their interactions with the right side of the heart in patients with PAH. Read article

United Therapeutics Will Move Forward With FDA Filing For New Drug

8/24/11 (Metro Business Media) - United Therapeutics Corporation released its FREEDOM-C(2) Phase 3 clinical trial results today but the company announced that the study did not meet its primary endpoint. Oral treprostinil (treprostinil diethanolamine) is next in the pipeline of drugs to treat PAH. United Therapeutics said that the tablet form of treatment for PAH did not post any statistical significance in the FREEDOM-C(2) trial. Read article

High Blood Pressure in Lungs Can Pose a Danger for Women

8/11/11 ( - A recent study has shown that pulmonary hypertension, or abnormally high blood pressure in the arteries of the lungs, affects about four times as many women as men in the United States. One of the most alarming findings of the study is that those with the disease were likely diagnosed many months after the first sign of symptoms. Read article by PHCR member Dr. Neil Ettinger

Evidence for the Role of Haptoglobin Phenotypes in Toxic Oil Syndrome

8/15/11 ( - It is now 30 years since toxic oil syndrome struck in Spain with devastating consequences. It is a food-borne disease that is estimated to have killed at least 2500 people with thousands more affected to this day with different degrees of disability. There was a swathe of acute symptoms which developed into chronic conditions such as motor neuropathy, musculoskeletal pain, sclerodermia, pulmonary hypertension, liver impairment, hypothyroidism and diabetes mellitus. Read article

Details of Updated UK Heart Failure Guidelines Raise Some Eyebrows

8/17/11 (Forbes) - Much of the controversy revolves around the relative weight given to echocardiography and natriuretic peptides in the diagnosis and treatment of heart failure. The updated NICE guidelines recommend that for the diagnosis of heart failure in patients with no history of MI, echocardiography should be used only if natriuretic peptides are raised. Peterson and Rumsfeld point out that both the ESC and ACC/AHA guidelines recommend that all patients with the signs and symptoms of heart failure should have an echocardiogram. The NICE position, they say, “may be questioned because of the utility of echocardiography for not only measuring left ventricular function but also for detecting structural or valvular heart disease, pulmonary hypertension, and pericardial effusion.” Read article

Conference Research Room Helps Researchers Develop New Tests for PH Patients

Georg Hansmann, MD, PhDAn interdisciplinary group of researchers from Boston have developed a microfluidic device that can test for the number of beneficial circulating endothelial progenitor cells (EPCs) in the blood stream of patients with cardiovascular disease. The number of EPCs in the blood can be used as a biomarker for risk assessment, disease progression and the response to therapy.  Up to now, the measurement of EPCs in the bloodstream was very difficult and laborious and therefore, not very useful to physicians working with PH Patients at the bedside.

Dr. Hansmann and colleagues have attended the research room at PHA’s 2010 International Conference to apply the so called "EPC Capture Chip"  in over 40 patients with PAH. They found the EPC numbers in PAH patients to be half the number in healthy controls.  The new EPC capture chip has tremendous potential for serving as a rapid bedside tests to monitor treatment and disease progression. The research team includes: Georg Hansmann; B.D. Plouffe; A. Hatch; A. Von Gise; H. Sallmon; Roham T. Zamanin; Shashi Murthy. Read abstract

NIH Funds New Stem Cell Projects

(RT Magazine) - The National Institutes of Health (NIH) has provided $85 million over 5 years to nine new studies aimed at developing induced pluripotent stem (iPS) cells from patients with genetic variations that have been associated with pulmonary hypertension, coronary artery disease, clotting disorders, diabetes, and other conditions. The studies will build upon previous genome-wide association study (GWAS) findings and investigate how gene variants lead to the physical manifestations of these diseases. Read article

Study: PH Patients at Increased Risk after Total Knee Arthroplasty

7/15/2011 (Ortho SuperSite) - A study by researchers at the Hospital for Special Surgery has found patients who have a history of significant medical issues — especially congestive heart failure or pulmonary hypertension — are at increased risk for major complications following total knee arthroplasty. Read article

Graduate Student One Step Closer to Finding Cure for Cardiovascular Disease

7/10/2011 (The Gateway) - A doctoral student at the University of Alberta has made a discovery that could lead to a cure for a disease which usually leaves approximately three years of life once diagnosed. Gopinath Sutendra has been researching pulmonary arterial hypertension, a disease characterized by excessive cancer-like growth of the cells in the pulmonary vessels of the heart, which provide blood to and from the lungs. These vessels end up getting blocked, resulting in right heart failure. Read article

Right Ventricular Structure Differs by Patient Age, Sex, Race

6/6/2011 (Internal Medicine News) - Right ventricular mass, volume, and ejection fraction differ significantly according to patient age, sex, and race, according to a prospective imaging study of more than 4,000 healthy people that was reported in Circulation on June 6. Read article

FREEDOM-M Trial of Oral Treprostinil in PAH Meets Primary Endpoint

(The Sacramento Bee) - United Therapeutics Corporation announced today the completion of its FREEDOM-M Phase 3 trial of treprostinil diethanolamine (oral treprostinil), an investigational sustained release oral formulation of treprostinil, a stable synthetic form of prostacyclin, in patients with pulmonary arterial hypertension (PAH).  Preliminary analysis demonstrates that the trial has met its primary endpoint. Read article

ATS: Heart Drugs Fail to Show Benefit in PAH

5/19/2011 (MEDPAGE Today) - Two drugs that are beneficial in heart disease were ineffective in treating pulmonary arterial hypertension (PAH), a researcher said. In a randomized trial, neither aspirin nor simvastatin (Zocor) improved performance on the standard six-minute walk test, according to Steven Kawut, MD, of the University of Pennsylvania in Philadelphia, and colleagues. Read article

Pfizer’s Revatio Receives European Approval to Treat PAH in Children

5/9/2011 (InPharm) - The European Commission has given the green light to phosphodiesterase-5 inhibitor Revatio (sildenafil) for pediatric patients. Read article

Pulmonary Hypertension After a Lung Transplant Predicts RRT Need

4/29/2011 (Renal & Urology News) - Pulmonary hypertension is a strong predictor of the need for renal replacement therapy (RRT) after lung transplantation, according to researchers. Read article

Study Supports the Safety of Transitioning PAH Patients from Inhaled Iloprost to Tyvaso

(RT Magazine) - An open-label study demonstrated that the rapid transition from inhaled iloprost to Tyvaso® (treprostinil) was well tolerated and associated with maintenance of exercise capacity and improved quality of life in pulmonary arterial hypertension (PAH) patients. Read article

REVEAL Registry: PAH Exacerbated by Presyncope, Syncope

4/18/2011 (Cardiology Today) - For patients with pulmonary arterial hypertension, a history of presyncope and syncope worsened the severity of disease, according to data from the REVEAL registry. What this indicates, according to researchers, is the importance of validating the prognosis and effect of syncope among patients with PAH as treatment guidelines continue to be defined. Read article

Dr. Larissa Shimoda to Discuss Mechanisms of Hypoxic Pulmonary Hypertension

4/10/2011 (Eurekalert) - When muscles and organs are deprived of an adequate supply of oxygen the body's usual responses include increased circulation and a slight drop in blood pressure in the blood vessels serving the affected tissue. However, the blood vessels in the lungs react differently: blood pressure in the lungs rises, often with deleterious effects on the lungs' tissue and the heart. Larissa A. Shimoda, PhD, Associate Professor of Medicine at the Johns Hopkins University School of Medicine in Baltimore, Md., has dedicated her research to learning why this happens. Read article

PHA-Funded Study Explores Role of MIF in Hypoxia-Induced Pulmonary Vascular Remodeling

Dr. Yinzhong ZhangResearch has long been the key to unlocking PH mysteries, and each year new discoveries occur that move us further along in our understanding and treatment of PH. Dr. Yinzhong Zhang of the Feinstein Institute for Medical Research is doing his part to propel PH research forward. Dr. Zhang was a 2008 recipient of the PHA/American Heart Association Post-Doctoral Fellowship Award for his research project entitled “Role of Macrophage Migration Inhibiting Factor (MIF) in Hypoxia-Induced Pulmonary Vascular Remodeling.”

Read more

GeNO LLC Initiates Study of Inhaled NITROSYL™ Nitric Oxide in Idiopathic Pulmonary Fibrosis and Pulmonary Arterial Hypertension

3/23/2011(Medical News Today) - GeNO LLC, a privately held, advanced development-stage technology company, announced commencement of the PHiano Study: A Phase 2, Dose-Escalation Trial for the Treatment of Pulmonary Hypertension in patients with Pulmonary Arterial Hypertension (PAH) and Pulmonary Hypertension secondary to Idiopathic Pulmonary Fibrosis (PH-IPF) using Inhaled NITROSYL™ nitric oxide (NO). The first patient was successfully dosed at Creighton University School of Medicine. Read article

CHMP Post-Authorisation Summary of Positive Opinion for Revatio

LONDON, 3/17/2011(PharmaLive) -  On 17 March 2011 the Committee for Medicinal Products for Human Use (CHMP) adopted a positive opinion recommending a variation to the terms of the marketing authorisation for the medicinal product Revatio. The marketing authorisation holder for this medicinal product is Pfizer Ltd. Read article

Raynaud's Responds to Sildenafil

3/9/2011 (MedPage Today) - Sildenafil (Viagra) may also help treat Raynaud's phenomenon. In a small randomized, double-blinded, and placebo-controlled trial, the drug led to a significant percentage reduction in the number of weekly attacks of Raynaud's phenomenon, according to Ariane Herrick, MD, of the University of Manchester in England, and colleagues. Read article

FDA Modifies Boxed Warning For Pulmonary Arterial Hypertension Drug Letairis

3/4/2011 (PRNewswire) - The U.S. Food and Drug Administration today announced that monthly liver enzyme tests are no longer required for those taking Letairis tablets (ambrisentan), used to treat pulmonary arterial hypertension. Citing data from clinical trials and postmarket reports, the FDA said that the drug poses only a low risk of liver injury. Information related to potential serious liver injury and the need to monitor for such serious injury is being removed from the drug's boxed warning. Read article

Findings Illuminate Role of Right Ventricular Failure in LV Free Wall Mass in Patients With CTEPH

2/16/2011 (Cardiology Today) - Right ventricular failure in patients with chronic thromboembolic pulmonary hypertension was linked with a reversible reduction in left ventricular free wall mass, study data suggest. Read article

Device in Artery Helps Track Blood Pressure in Heart Patients

2/10/2011 (The Columbus Dispatch) - As many as 2 million Americans with congestive heart failure could benefit from a pressure monitor inserted in the pulmonary artery to help doctors track early signs of trouble, according to a study published this week in the medical journal The Lancet. Read article

Longer Time to Treatment for Child IPAH Patients

2/6/11 (MedPageToday) - According to a study of almost 3,000 patients at centers around the U.S., adults began treatment within two months of being diagnosed with IPAH -- compared with 11-months for the children, according to Robyn Barst, MD, of Columbia University, and colleagues. Read article

Society of Thoracic Surgeons: Lung Emboli Surgery Safer

2/3/11 (MedPageToday) - Outcomes from pulmonary endarterectomy for the treatment of chronic thromboembolic pulmonary hypertension are improving, despite the number of increasingly complex cases, according to a large single-center study. Read article

What Works for Raynaud's Phenomenon With Ischemic Finger

1/26/11 (Internal Medicine News) - Until further testing confirms or disproves the promise of an oral formulation of treprostinil that is making its way through the developmental pipeline for management of Raynaud’s phenomenon and the ischemic finger, rheumatologists will have to continue to make do with modestly efficacious agents that all are best employed in conjunction with background therapy using a long-acting calcium channel blocker titrated to the maximum tolerated dose. Read article

Long-Term Hypoxia in Flies Shown to Result in Permanent DNA Changes

1/24/2011 (EurekAlert) - In an important study that may shed light on human ability to adapt to hypoxia, or inadequate levels of oxygen, researchers at the University of California, San Diego School of Medicine have proven that the genome of flies exposed to long-term hypoxia are changed to permanently affect gene expression. Their findings, to be published online by the journal of the Proceedings of the National Academy of Sciences (PNAS) the week of January 24, 2011, may lead to new targets for development of therapies for hypoxia-induced disease in humans. Read article

COPD in Acute MI Patients Spells Trouble

1/24/2011 (Internal Medicine News) - Chronic obstructive pulmonary disease is a powerful risk factor for in-hospital mortality or cardiogenic shock in patients presenting with ST-elevation MI, according to a large retrospective study. The clinical inference is that the reduced cardiopulmonary reserve imposed by COPD – a disease often marked by pulmonary hypertension and right ventricular dysfunction – renders the circulatory system less capable of coping with the effects of an MI, Dr. Kohei Wakabayashi said at the annual scientific sessions of the American Heart Association. Read article

New Diagnostic Tool To Detect Complications In Lung Transplant Patients

1/2/11 (Medindia) - University of Michigan researchers have developed a new diagnostic tool that could help predict a fatal complication in lung transplantation patients. In the complication known as bronchiolitis obliterans syndrome (BOS), scar tissue builds up in the lungs and chokes off the ability to breathe. Read article

EU Agency to Review Other PAH Drugs

12/16/10 (the - The European Medicines Agency is to review the hepatotoxic profile of two other endothelin receptor antagonists (ERAs) for the treatment of pulmonary arterial hypertension following the withdrawal last week of Pfizer's ERA sitaxentan (Thelin) due to two cases of fatal liver injury linked to the drug. Read article

Pfizer Recalls Lung Thelin®, Ends Clinical Trials

(Bloomberg) - Pfizer Inc. is recalling its lung drug Thelin worldwide and halting trials of the medicine after it was linked to potentially life-threatening liver damage. No new patients should be prescribed Thelin and those receiving it should be transferred to other therapies, Pfizer said in a statement today. Read article

PHA-Funded Study Explores Role of MIF in Hypoxia-Induced Pulmonary Vascular Remodeling

Research has long been the key to unlocking PH mysteries, and each year new discoveries occur that move us further along in our understanding and treatment of PH. Dr. Yinzhong Zhang of the Feinstein Institute for Medical Research is doing his part to propel PH research forward. Dr. Zhang was a 2008 recipient of the PHA/American Heart Association Post-Doctoral Fellowship Award for his research project entitled “Role of Macrophage Migration Inhibiting Factor (MIF) in Hypoxia-Induced Pulmonary Vascular Remodeling.” Learn more

FDA Clears Investigational New Drug Application for Clinical Trials With NITROSYL™ Inhaled Nitric Oxide System

12/2/10 (PR Newswire) - GeNO LLC announced today that the U.S. Food and Drug Administration has cleared its Investigational New Drug application for a clinical trial with the GeNO NITROSYL™ (Inhaled Nitric Oxide) System for treatment of pulmonary hypertension in patients with Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis, (PH-IPF WHO Group 3) and Pulmonary Arterial Hypertension, (PAH, WHO Group 1). Read article

Tiny San Diego Biotech Will Resume Work on Experimental Lung Disease Drug

11/11/10 (The San Diego Union-Tribune) - An infusion of $20 million from investors and a potentially lucrative partnership with a major pharmaceutical company is breathing new life into Aires Pharmaceuticals, nearly a year after the San Diego drug developer ran out of money and essentially shut down. Aires said Thursday that Novartis had acquired the option to obtain the worldwide rights to Aironite, the company’s experimental treatment for primary pulmonary hypertension, in a deal worth up to $250 million if certain milestones are reached. Read article

CHEST: Clinical Changes Signal Prognosis in PAH

11/5/10 (Medpage Today) - An increase in New York Heart Association functional class or at least 15% reduction in Six-Minute Walk Distance predicted lower likelihood of major event-free survival at two years in the REVEAL study. Read article

Breakthrough in Diagnosis and Treatment of Pulmonary Hypertension

10/25/10 (Toronto Sun) - A Canadian researcher may have discovered a breakthrough diagnosis - even a cure - for pulmonary hypertension. New research indicates a protein, dubbed PIM-1, is high in patients with the condition. The higher the PIM-1 levels, the more severe the patient's pulmonary hypertension. Read article

Updated U.S. Product Labeling for Veletri (Epoprostenol for Injection)

(PR-USA) - Actelion today announced that the U.S. Food and Drug Administration (FDA) has approved additional in-use conditions for administration of Veletri®, an improved formulation of epoprostenol. Dr. Vallerie McLaughlin, Professor of Medicine and Director of Pulmonary Hypertension at the University of Michigan, commented: "Patients with more advanced PAH struggle daily with debilitating symptoms, such as shortness of breath, chest pain and chronic fatigue. I expect the improved convenience of Veletri's preparation and storage flexibility to significantly benefit these patients and make a difference in their everyday life." Read article

YM BioSciences Licensee Pulmokine Awarded NIH Funding to Develop Small Molecule Drugs for PH
9/29/2010 (PR Newswire) - YM BioSciences Inc. today reported that Pulmokine Inc. (Slingerlands, New York), a licensee of several small molecule compounds from YM, has been awarded two National Institutes of Health (NIH) Small Business Innovation Research (SBIR) grants. The grants, totaling more than US$650,000, are Phase I awards to develop novel treatments for PAH. These compounds, originating from YM BioSciences' small molecule library, have defined mechanisms of action and, based on preliminary experiments, are believed to inhibit key processes in PAH disease development and progression. YM will continue to have a role in the ongoing development of the compounds. Read article
Pulmonary Vascular and Right Ventricular Dysfunction in Adult Critical Care: Current and Emerging Options for Management: a Systematic Literature Review
(7th Space Interactive) - Pulmonary vascular dysfunction, PH and resulting right ventricular failure occur in many critical illnesses and may be associated with a worse prognosis. PH and RV failure may be difficult to manage: principles include maintenance of appropriate RV preload, augmentation of RV function, and reduction of RV afterload by lowering pulmonary vascular resistance (PVR). Read article
Actelion Pharmaceuticals Announces FDA Approval of Brand Name for Commercially Available VELETRI® (epoprostenol for injection) for Pulmonary Arterial Hypertension
(PRNewswire) - Actelion Pharmaceuticals US, Inc., today announced that the U.S. Food and Drug Administration (FDA) has approved the brand name VELETRI® for the company's epoprostenol for injection therapy.  VELETRI has been commercially available since April 2010 as Epoprostenol for Injection for the treatment of moderate to severe pulmonary arterial hypertension (PAH) and PAH associated with the scleroderma spectrum of disease.  Actelion plans to release VELETRI-labeled product by early fourth quarter of 2010. Read article
Potential Treatment For Pulmonary Hypertension
(Medical News Today) - Researchers in the Faculty of Medicine & Dentistry at the University of Alberta are one step closer to a treatment for PAH. Read article
Pfizer's Viagra Weighed by U.S. FDA for Children With PAH
7/27/10 (Bloomberg) - Revatio may be used for children with a rare lung disorder if U.S. regulators can agree on how to test it. Outside advisers to the Food and Drug Administration are set to meet July 29 to evaluate whether Pfizer’s study of Revatio is sufficient to determine its effectiveness in children, the agency said today. Read article
Data Published on Novartis QT1571 for Treatment of Severe Pulmonary Arterial Hypertension
7/9/10 ( - The proof of concept study to assess the safety, tolerability and efficacy of QTI571 (imatinib) in patients with pulmonary arterial hypertension (PAH) was published in the American Journal of Respiratory and Critical Care Medicine. QTI571, a tyrosine kinase inhibitor, was studied in patients with PAH not adequately controlled with currently licensed medications. QTI571 significantly improved exercise capacity and pulmonary haemodynamics compared with placebo in patients with severe PAH. Read press release
Researchers Point to Possible Scleroderma-Cancer Link

7/9/10 (HealthDay News) - People with specific antibodies may develop both cancer and a certain type of scleroderma -- an incurable autoimmune disease -- at nearly the same time, new research finds. The finding, from a small study of 23 patients, hints at a possible link between cancer and autoimmune disorders. Read article

Tezosentan Does Not Prevent Bypass-Related Right Ventricular Failure in PH Patients
June 29, 2010 (Doctor's Guide) - Administration of an endothelin receptor blocker does not lessen rates of right ventricular failure provoked by cardiopulmonary bypass in patients with pulmonary hypertension, according to research presented here at the 2010 Annual Meeting of the Canadian Anesthesiologists' Society (CAS). Read article
Formula For Predicting Survival in Patients With PAH
(The University of Chicago Department of Medicine) - Developed at The University of Chicago, a new regression equation, the PHC calculator, is used to predict survival in patients with idiopathic, familial, and anorexigen-associated PAH. The PHC calculator helps medical professionals prognosticate survival based on cardiac catheterization results at the time of diagnosis. Read article | Access the calculator
Echocardiographic Assessment of Pulmonary Vascular Resistance in PAH
(7thSpace Interactive) - Echocardiographic ratio of peak tricuspid regurgitant velocity to the right ventricular outflow tract time-velocity integral (TRV/TVI rvot) was presented as a reliable non-invasive method of estimating pulmonary vascular resistance (PVR). Studies using this technique in patients with moderate to high PVR are scarce. Read article
Safety Affirmed with Echo Contrast Agent
(MedPage Today) - Microbubble contrast agents used in echocardiography don't elevate cardiovascular risk despite lingering doubts, according to a meta-analysis. Read article
Epigenetic Gene Silencing May Hold Key to Pulmonary Hypertension
6/11/10 ( - A rare but fatal disease of blood vessels in the lung may be caused in part by aberrant silencing of genes rather than genetic mutation, new research reports. Pulmonary arterial hypertension, a syndrome characterized by gradual blockage of blood vessels in the lungs, has been linked to genetic causes in a small percentage of patients. But University of Chicago researchers have now found that a form of epigenetics -- the modification of gene expression -- causes the disease in an animal model and could contribute to the disease in humans. Published in the American Heart Association journal Circulation, the finding opens a promising avenue for research into the origins of vascular disorders. Read press release
European Respiratory Journal Publishes Phase II Study of Investigational Oral Agent Riociguat in Treatment of Pulmonary Hypertension
6/11/10 (PR Newswire) - Bayer HealthCare Pharmaceuticals, Inc. announced that the study "Riociguat for chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension: a phase II study" was published online this week by the European Respiratory Journal. Riociguat (BAY 63-2521) is an investigational oral agent being studied for the potential treatment of PH that targets soluble guanylate cyclase (sGC), a receptor for nitric oxide (NO). Read press release
New Product Has Potential for Early Diagnosis of Pulmonary Hypertension in Pediatric Patients
6/7/10 (GlobeNewswire) - Pulmo BioTech Inc.has announced that early indications are that its PulmoBind(TM) product candidate has the potential for early diagnosis of pulmonary hypertension (PH) in the pediatric population. Read announcement
Right Ventricular Ejection Fraction is Better Reflected by Transverse Rather than Longitudinal Wall Motion in Pulmonary Hypertension
6/4/10 (7th Space) - This study showed that regional transverse wall movements provide important information of right ventricle (RV) function in PH. Compared to longitudinal motion, transverse motion at mid RV reveals a significantly stronger relationship with RV ejection fraction (RVEF) and thereby might be a better predictor for RV function. Read study summary
Hepatic Failue, Neonatal Hemochromatosis and Port-Pulmonary Hypertension in a Newborn with Trisomy 21 - A Case Report
5/19/10 (7th Space) - A report shows for the first time the association of liver failure with porto-pulmonary hypertension secondary to neonatal hemochromatosis (NH) in an infant with Trisomy 21. Read report summary
Urge Your Senators to Support Increased Funding for National Institutes of Health
 The NIH conducts critical research on pulmonary hypertension that improves the health and saves the lives of PH patients. The funding allocated to NIH has not kept pace with inflation and NIH is not as able to award grants to much-needed medical studies. See how much funding your state got from the NIH in 2009

Take action now! Senator Casey (D-PA), the Senate sponsor of the PH Research and Education Act, is circulating a letter requesting an 11.9% increase in funding for NIH for Fiscal Year 2011. Urge your senators to support NIH research by signing onto this letter before June 3.

Use PHA's talking points to contact your Senators today.
Take Action
Gene Therapy May Be Effective in Treating Pulmonary Arterial Hypertension
5/18/10 ( Australia - Gene therapy has been shown to have positive effects in rat models of pulmonary arterial hypertension (PAH), according to researchers at the University of Adelaide in Australia. Read article
Downside of Revatio? Possible Hearing Loss
5/18/10 (MSNBC) - Men who take Viagra (sildenafil) could be at an increased risk for long-term hearing loss, a new study suggests. However, the study only shows an association, and not a direct cause-effect link, and more research is needed to back up the findings. Sildenafil is also used as a treatment for pulmonary hypertension (Revatio). Read article
Bayer Pulmonary Hypertension Drug Riociguat Shows Promise in Long-term Trial
5/18/10 (Reuters) - Bayer's experimental pill riociguat showed long-term benefits in treating a life-threatening lung disease. After treatment for about 17 months on average, patients suffering from two types of pulmonary-hypertension, CTEPH and PAH, were able to endure more physical exercise than before, according to data posted on the American Thoracic Society's website. Read article
Addition of Ambrisentan to Sildenafil Improves Outcomes for Patients With Progressive PAH: Presented at ATS
5/18/10 (Doctor's Guide) New Orleans - For people with pulmonary arterial hypertension (PAH) who have progressive disease while on sildenafil therapy, the addition of ambrisentan appears to provide similar safety and effectiveness as ambrisentan monotherapy in treatment-naïve patients, researchers said here at the American Thoracic Society (ATS) 2010 International Conference. Read article
Actelion Pulmonary Hypertension Drug Selexipag Shows Promise in Small Study
5/17/10 (Reuters) - An experimental drug being developed by Actelion Ltd for a serious lung disorder significantly eased resistance to blood pumping through arteries around the lungs, according to data from a small, mid-stage clinical trial presented on Monday. The Swiss drugmaker is developing selexipag to treat pulmonary arterial hypertension, or PAH, a life-threatening condition in which abnormally high blood pressure in the arteries between the heart and lungs severely compromises the function of both organs. Read article
Sildenafil Improves Quality of Life, Walking Distance in Patients With PAH: Presented at ATS
5/17/10 (Doctor's Guide News) New Orleans - Treatment of pulmonary arterial hypertension (PAH) with sildenafil significantly improves several quality-of-life measures and walking distance in patients, researchers reported today at the American Thoracic Society (ATS) 2010 International Conference. Read article
Real-World Bosentan Therapy Successful in Pulmonary Arterial Hypertension: Presented at ATS
5/17/10 (Doctor's Guide News) New Orleans - Real-life experiences in treating patients with pulmonary arterial hypertension with bosentan appears to provide similar efficacy as seen in clinical trials -- despite patients being somewhat older and having more scleroderma, researchers said today at the American Thoracic Society (ATS) 2010 International Conference. Read article
REVEAL(R) RegistryData May Advance Patient Care in Pulmonary Arterial Hypertension
5/17/10 (PRNewswire) - Actelion Ltd announced today that findings from the REVEAL® (Registry to Evaluate Early And Long-term PAH Disease Management) Registry have confirmed the clinical utility of the REVEAL risk score calculator, a new tool designed to predict 1-year survival in patients with pulmonary arterial hypertension (PAH) according to data presented at the American Thoracic Society's (ATS) Annual International Conference. The predictive tool was developed and validated in patients enrolled in REVEAL, the largest prospective registry in PAH containing data from more than 3,500 patients. Read press release
Tibetans Developed Genes to Help Them Adapt to Life at High Elevations
5/13/10 ( - A detailed understanding of these changes may eventually lead to targeted therapies for common human maladies, including pulmonary hypertension and lung and brain edema, which affect people everywhere. Read article
Pfizer Enters Into Agreement With Ergonex Pharma to Acquire Investigational Treatment for Pulmonary Arterial Hypertension
5/12/10 (PR Newswire) - Pfizer Inc. and Ergonex Pharma GmbH announced that they have entered into an agreement under which Pfizer will acquire terguride, which is in development as a potential treatment for pulmonary arterial hypertension (PAH). Under the terms of the agreement, Pfizer will support the completion of the ongoing Phase 2 trial for terguride and will have exclusive worldwide rights excluding Japan to commercialize terguride for the treatment of PAH. Read press release
High-Altitude Research Advances Low-Altitude Medicine for Pulmonary Hypertension and Pulmonary Edema

5/4/10 (EurekAlert!) Philadelphia, Penn. - High altitude medicine is a "natural research laboratory" for the study of cardiovascular physiology and pathophysiology. Yves Allemann, MD, FESC, Swiss Cardiovascular Center, University Hospital, Bern, and Urs Scherrer, MD, Centre Hospitalier Universitaire Vaudois, Lausanne, have assembled an international group of leading authorities to contribute to a special issue of Progress in Cardiovascular Diseases dedicated to high-altitude medicine and novel insights into disease mechanisms provided by high-altitude research.

The article by Scherrer et al demonstrates how studies at high altitude have provided important insights into fundamental mechanisms underpinning pulmonary hypertension and pulmonary edema in humans. They show how these insights have been translated into novel approaches for the treatment of patients suffering from these problems at low altitude. Finally, it provides some hints on how the natural research laboratory of high altitude may provide novel insight into cardiovascular disease mechanisms in the future.

Read full press release

New Drug-Interaction Warnings for All HIV Protease Inhibitors
4/28/10 (AIDSMEDS) - Package inserts for all of the approved protease inhibitors (PIs) used to treat HIV have been updated to reflect important drug-drug interactions, according to an April 27 e-mail announcement from the U.S. Food and Drug Administration. Revatio is contraindicated among people living with HIV using PIs, meaning that the drug should not be used under any circumstances. Tracleer and Adcirca should be used cautiously. According to the FDA, their doses may need to be adjusted when combined with protease inhibitors. The agency also warns against using Tracleer with the PI Reyataz (atazanavir) if a Norvir (ritonavir) booster is not also included. Read article
Trial Shows Inhaled Drug Improves Pulmonary Arterial Hypertension Outcomes
(MedPage) - For patients with severe pulmonary arterial hypertension, adding inhaled treprostinil (Tyvaso) to other therapies improves exercise capacity and quality of life, a randomized, placebo-controlled trial showed. After 12 weeks of combination treatment with either bosentan (Tracleer) or sildenafil (Revatio), the prostacyclin analog resulted in a significantly improved peak six-minute walk distance compared with placebo (median 20 meters, P=0.0004), according to Vallerie McLaughlin, MD, of the University of Michigan in Ann Arbor, and colleagues. Dr. Mclaughlin is PHA's Scientific Leadership Council (SLC) Chair. Read article | Learn about PHA's SLC
Study Shows Bilirubin a Prognostic Marker in Patients with Pulmonary Arterial Hypertension
(7th Space) - Previous studies demonstrated relationship between the results of liver function test and the prognosis in patients with heart failure. However, few studies have examined this relationship in patients with pulmonary arterial hypertension (PAH), which this study did. Results indicated that elevated serum bilirubin is a risk factor for death in patients with PAH. Read study synopsis
New PH Treatment! New Form of Epoprostenol Now on the Market
(PR Newswire) - Actelion Pharmaceuticals US, Inc. today announced the commercial availability of Epoprostenol for Injection, an improved formulation of epoprostenol that is stable at room temperature, for the treatment of primary pulmonary hypertension and pulmonary hypertension associated with scleroderma spectrum of disease in NYHA Class III and Class IV patients. Simultaneously, the company has launched the PROSPECT registry, a multicenter, observational, U.S.-based registry that will provide additional clinical experience on patients being treated with Epoprostenol for Injection. Read press release
Research Shows Possible Predictors of Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis
4/15/10 (7th Space) - In this study, iron deposition and alveolar septal capillary density (ASCD) in non fibrotic lung tissue showed an association with right ventricular systolic pressure (RVSP), suggesting that these features are possible morphologic predictors of pulmonary hypertension in idiopathic pulmonary fibrosis (IPF). Read article
Valve-in-Valve Implants via Catheter Effective in High-Risk Patients
4/12/10 ( - Replacing failing artificial animal-based heart valves by implanting mechanical valves inside them is an effective option for high-risk patients, according to research reported in Circulation: Journal of the American Heart Association. The procedure is offered only to selected patients with failed surgical valves who would be poor candidates for another operation. Factors that made the patients in the study risky or ineligible as surgical candidates included two or more prior open-chest surgeries, severe pulmonary hypertension and various heart or other complications. Read article
UNC Doctor Uses Award for Pulmonary Hypertension Research
4/9/10 (Medical News Today) - Sean E. McLean, MD, has been selected as a 2010-2014 Harold Amos Medical Faculty Development Program scholar. McLean, an assistant professor of pediatric surgery in the University of North Carolina at Chapel Hill School of Medicine, is one of only 11 nationwide selected this year to receive the award. McLean said he plans to use the $420,000, four-year grant to support a laboratory research project that focuses on how changes in smooth muscle cell phenotypes contribute to pulmonary hypertension, using a mouse model for congenital diaphragmatic hernia. Read article
PH Research Benefits from Federal Stimulus Funding

researcher in labThe Pulmonary Hypertension Association's Washington Rep, Gavin Lindberg, sent us a report recently from the National Institutes of Health (NIH) on the investment made in pulmonary hypertension research with funds from the economic stimulus bill. PHA joined with the biomedical research community last year in encouraging Congress to provide an additional $10 billon to the NIH as part of the American Recovery and Investment Act (ARRA).

The NIH has been producing "ARRA Investment Reports" on diseases that benefited significantly from stimulus funding. Only a handful of rare diseases have been the subject of these investment reports and PH is one of them. Read more on PHA President Rino Aldrighetti's blog

Minimally Invasive Heart Valve Therapy Improves Heart Function, Quality of Life for Mitral Regurgitation Patients
3/14/10 (PRNewswire) Evanston, Ill. - NorthShore University HealthSystem interventional cardiologist, Ted Feldman, MD, presented data at the American College of Cardiology annual scientific sessions from the EVEREST II trial (Endovascular Valve Edge-to-Edge REpair STudy) demonstrating the MitraClip, a novel, first-in-class catheter-based device,  is a safe and effective alternative to open heart surgery for select patients with mitral regurgitation. Patients in the study had at least moderate-to-severe functional or degenerative mitral regurgitation and were either symptomatic or asymptomatic with evidence of compromised left ventricular function, atrial fibrillation, or pulmonary hypertension. Read article
FDA Clears GeNO LLC Investigational New Drug Application for Nitric Oxide Delivery System
(PRNewswire) COCOA, Fla. - GeNO LLC ( a privately held, advanced development-stage technology company, today announced that the U.S. Food and Drug Administration (FDA) has granted clearance of its Investigational New Drug (IND) application for its stand-alone gas cylinder Nitrosyl™ Delivery Platform (NDP). The initial indication to be studied for nitric oxide delivered via the GeNO Nitrosyl delivery system is as a diagnostic agent for administration as an adjunct to right heart catheterization in patients with Pulmonary Arterial Hypertension (WHO Group 1) to add information to improve clinical decision making. Read article
When Should an IVC Filter Be Used to Treat a DVT?
(The Hospitalist) - There is insufficient data to support the use of IVC filters for such situations as recurrent VTE on anticoagulation, recurrent PE with pulmonary hypertension, extensive free-floating ilio-femoral thrombus, and post-thrombolysis of ilio-caval thrombus. Read article
Treatment of Portal Hypertensive Pulmonary Lesions Induced by Schistosomiasis
3/4/2010 (EureaAlert) - To evaluate efficacy of Calculus Bovis compound preparation (ICCBco) in the treatment of lung lesions in portal hypertensive rabbits with schistosomiasis as the experimental animal model, a research group in China performed a randomized, double-blind, controlled trial to observe pathological changes and pathological effect mechanism of expression of fibronectin and laminin in the lung tissue of portal hypertensive rabbits with schistosomiasis. Read article
Acupuncture Benefit Seen in Pregnancy to Treat Depression
2/23/10 (The Wall Street Journal) - Acupuncture designed to treat depression appears to improve symptoms in pregnant women, suggesting it as an alternative to antidepressant medication during pregnancy, a study found. A previous study showed that the risk of persistent pulmonary hypertension, a potentially serious lung condition, is significantly greater in newborns whose mother took antidepressants later in pregnancy. This new study indicates acupuncture may be a possible alternative to using antidepressants. Read article
New Medical Device, Shape-HF, Measures Patient Physiology to Define Shortness of Breath
2/23/10 (PRWeb) Saint Paul, Minn. - According to Dr. Myron Licht, defining the specific cause of shortness of breath is now more simple because of a new medical device available at the Apex Heart Care clinic. That device is called the Shape-HF and it measures a patient's physiology in an easy, six-minute test that helps physicians identify the predominant source of shortness of breath, primarily the heart or lungs. In addition to defining a patient's physiological limitation, the Shape-HF also measures responses to pharmacotherapy and cardiac resynchronization therapy and unmasks exercise-induced changes in pulmonary vasculature. Dr. Licht uses his Shape-HF to measure patients with a variety of heart and lung ailments including heart failure, heart shunts, chronic obstructive pulmonary disease, pulmonary hypertension, and even a double lung transplant. Read article
Six States To Study Sickle Cell Disease And Thalassemias In National Pilot Project
2/20/10 (Medical News Today) - Medical researchers are developing a new surveillance system to determine the number of patients diagnosed with a family of inherited blood disorders known as hemoglobinopathies, including sickle cell disease, thalassemias, and hemoglobin E disease. Sickle cell disease is associated with pulmonary hypertension. Read article
United Therapeutics Withdraws PH Drug Application in Europe
2/19/10 (Washington Business Journal) - Silver Spring, Md.-based United Therapeutics Corp. has pulled the plug on its European marketing application to sell Tyvaso (TM) (treprostinil), an inhaled version of its pulmonary arterial hypertension drug, because its chances for approval in the European market seemed unlikely. Read article
Sarcoidosis-Associated Pulmonary Hypertension: A Role for Endothelin Receptor Antagonists?
2/15/10 (Medscape) - Data on the treatment of sarcoidosis-associated pulmonary hypertension are scarce, while the variety of underlying pathophysiologic mechanisms are a major limitation in the implementation of a universal therapy. Addition of bosentan, a dual endothelin receptor antagonist, resulted in marked improvement in functional class and exercise capacity of the patient, allowing gradual tapering of steroids. Read article (requires free registration)
PHA "Path to a Cure" Climbers Garner News Coverage and Awareness of PH

As Dr. Robert Frantz, Jessica Lazar, PA, and Dr. Raymond Benza (pictured right in Tanzania) climb Mt. Kilimanjaro, their efforts are getting coverage in the news, raising much needed awareness about pulmonary hypertension. They have raised over $100,000 so far for PH research and patient services. Check out the latest stories:

Read full list of news coverage | Learn more about Path to a Cure campaign | Donate

Artificial Lung Tissue Developed for Premies with Hypoplasia, Which Can Lead to PH
2/11/10 (The Johns Hopkins news-Letter) - Although there have been various state-of-the-art treatments for pulmonary hypoplasia, later in life, infants can still suffer from pulmonary hypertension, hyaline membrane disease and acute respiratory distress syndrome. The laboratory of Fizan Abdullah combined with the Pediatric Surgery and Mechanical Engineering departments of Johns Hopkins worked to create an artificial alveolar-capillary membrane that could one day put an end to pulmonary hypoplasia. Read article
Research Results: Can Moderate-Severity Autologous Pulmonary Embolism in Rats Produce Persistent PH
2/12/10 (7th Space Interactive) - Objective: Test if moderate-severity autologous pulmonary embolism (PE) in Sprague-Dawley (SD) and Copenhagen (Cop) rats can produce persistent pulmonary hypertension. Conclusion: Neither strain developed persistent PH. Experimental models of PE designed to induce sustained PH and a robust inflammatory response appear to require significant, persistent pulmonary vascular occlusion. Read full summary
The Role of the Protein HIF-2-Alpha in Chuvash Polycythemia, a Disease Associated with PH
2/9/10 (Journal of Clinical Investigation) - Individuals with a condition known as Chuvash polycythemia, which is caused by a specific mutation in the protein VHL, have a greater proportion of their blood volume occupied by red blood cells than do healthy individuals. They also have pulmonary hypertension and increased respiratory rates, although the mechanistic basis for these symptoms has not been determined. Now, Celeste Simon and colleagues at the University of Pennsylvania School of Medicine, Philadelphia, have identified a role for the protein HIF-2-alpha in the lung complications of Chuvash polycythemia by studying mice that model the condition. Of particular interest, HIF-2-alpha activity was found to be increased in lungs from mice that model Chuvash polycythemia. Further, as loss of one copy of the gene responsible for generating HIF-2-alpha in mice that model Chuvash polycythemia suppressed both the polycythemia and pulmonary hypertension, the authors suggest that inhibiting HIF-2-alpha might provide a new approach to treat Chuvash disease. Read full article
Aviptadil Successful in Phase II Clinical Trials for PAH, Plans to Test for Treatment of Secondary PH
(Gerson Lehrman Group) - Aviptadil, a vasoactive intestinal peptide (VIP), is a novel target for the treatment of primary pulmonary hypertension (PAH) that was shown to have beneficial effects on hemodynamics and safety in a phase II clinical trial for PAH. mondoBIOTECH and Lung Rx announced a partnership to bring Aviptadil to market, presumably to target the larger market of lung diseases with secondary pulmonary hypertension. Read article
Adcirca® (tadalafil) Approved in Canada for PAH Patients
2/3/10 (PharmaLive) - Adcirca® (tadalafil) is now available in Canada for the treatment of pulmonary arterial hypertension (PAH). Adcirca® is the first once-a-day PDE-5 inhibitor oral therapy for the treatment of PAH. It is a vasodilator, which means it relaxes or opens blood vessels. “As a physician who treats this serious and life-threatening disease, I am confident that the convenience of this new oral treatment will help to promote compliance among patients, thus making a difference in their health-related quality of life,” said Dr. Sanjay Mehta, Professor of Medicine and Director of the Southwest Ontario PH Clinic in London, Ontario, and Medical Director, PHA Canada. Read article | Read Tadalafil (Adcirca®) Fact Sheet
Transcatheter Aortic Valve Replacement in Patients with High Surgical Risk
1/21/10 (Cardiology Today) - Transfemoral and transapical transcatheter aortic valve implantation in patients at prohibitive or high surgical risk were associated with comparable mortality as predicted by surgical risk calculators, results from a study suggested. If there is truth in repetitive data, it looks like transcatheter aortic valve replacement works. Read article
Novel Equation Effective at Predicting Survival in Pulmonary Arterial Hypertension
(Cardiology Today) - A newly formed equation that can predict survival in patients with pulmonary arterial hypertension was more effective than the equation traditionally used by the National Institutes of Health to predict survival, study results suggested. Read Article
Researchers Revisit Pulmonary Arterial Hypertension Survival
(EureaAlert) - Setting out to determine the survival of patients with pulmonary arterial hypertension (PAH), researchers at the University of Chicago Medical Center and their colleagues also discovered that an equation used for more than 20 years to predict survival is outdated. Accordingly, they developed and recently published a new survival prediction equation that will impact clinical practice and the drug development process. Read article
NeoPharm to Investigate Treatment for Deadly Lung Disease
(onemedplace) - NeoPharm, a biopharmaceutical company specializing in cancer treatments, has filed an investigational new drug application with the U.S. Food and Drug Administration (FDA) for the treatment of a deadly lung disease. The drug candidate IL13-PE has the potential to be the first effective therapy for idiopathic pulmonary fibrosis (IPF), which kills an estimated 40,000 people in the United States each year. Read article
Pfizer Gets European OK for Intravenous Revatio
(ABC News) – Drug developer Pfizer Inc. said Monday European regulators approved an intravenous version of its high blood pressure drug Revatio. Specifically, the European Commission has now approved the intravenous version for patients now prescribed oral Revatio and temporarily unable to take oral medicine but otherwise clinically stable. Read article
Cardiologist Wins Stimulus Grant to Identify Biomarkers of Idiopathic Pulmonary Hypertension
( – Johns Hopkins Children's Center cardiologist Allen Everett recently won more than $460,000 in stimulus grant funding to identify the biomarkers of idiopathic pulmonary hypertension. Read article
Breathlessness Eased in Patients With Pulmonary Arterial Hypertension
(ScienceDaily) – Patients with a rare, deadly disease that mostly affects women felt a dramatic reduction in breathlessness using an approved drug, according to study results published online December 17 in The Journal of Heart and Lung Transplantation. Read article
Clinical Programs With Riociguat In PH Well On Track
(Medical News Today) – A first Phase II trial with Bayer Schering Pharma's oral agent riociguat in pulmonary hypertension owing to interstitial lung disease (PH-ILD) has successfully been completed.  Read article
Pfizer Granted FDA Approval For Intravenous Formulation Of Revatio
(RTTNews) – Health care major Pfizer Inc. said Friday that the U.S. Food and Drug Administration approved an intravenous formulation of Revatio or sildenafil for continued treatment of patients with pulmonary arterial hypertension, who are unable to take oral medication. Read article
Bosentan Improves Cardiovascular Parameters in Patients With Pulmonary Hypertension Associated With COPD: Presented at CHEST 2009
(Doctor's Guide) – In patients with moderate to severe chronic obstructive pulmonary disease (COPD) and PH, bosentan plus best conventional care produced greater improvement in cardiovascular parameters than best conventional care alone. Read article
Gilead Sciences, GlaxoSmithKline Reveals Phase IV Combination Study Of PH Drugs
(RTTNews) – Gilead Sciences Inc. in association with GlaxoSmithKline announced plans for a Phase-IV clinical trial to evaluate first-line combination use of ambrisentan and tadalafil, in patients with PAH, as compared to monotherapy. Read article
UC San Diego Researchers Reverse Pulmonary Arterial Hypertension in Mouse Models
(EurekAlert) – Researchers at the University of California, San Diego, have identified a key protein that promotes the development of pulmonary arterial hypertension in humans and mice. This groundbreaking discovery has implications for future drug therapies that may extend the life of patients with pulmonary arterial hypertension and prevent the need for lung transplantation, currently the only cure for this debilitating disease. Read article
Cancer Drug May Help Scleroderma
(HealthDay News) – A new study suggests that the cancer drug Gleevec may benefit people with scleroderma, a chronic connective tissue disease. Read article
Healing Effects of Carbon Monoxide Being Studied in an Amazing Variety of Diseases and Conditions
(SmartPlanet) – For years, researchers have quietly been studying the healing effects of carbon monoxide. Inhalation of CO at doses between 10 and 1000 ppm protected animals against restenosis after arterial balloon injury, pulmonary hypertension and more. Read article
First Drug For Scleroderma Treatment Maybe on Horizon
(EmaxHealth) – Until now, no drug has been found to be effective for treating scleroderma. Results of a new study to be presented at the annual meeting of the American College of Rheumatology in Philadelphia on October 18 may change that. Read article
UW to Lead an Effort to Identify Gene Variations That Influence the Severity of Lung Diseases Including PH
(7thSpace Interactive) – The National Heart, Lung, and Blood Institute (NHLBI) announced Oct. 1 that the University of Washington (UW) will receive two of the six "Grand Opportunity" NHLBI Large-Scale DNA Sequencing Project awards. The multi-institutional genomics project will examine the genetic connections to heart, lung, and blood diseases that account for three of the leading causes of death in the United States. Read article
Tracleer Receives Label Extension in Canada for the Treatment of Patients With Mildly Symptomatic WHO Functional Class II PAH
(Marketwire) – Actelion Pharmaceuticals Canada Inc. announced today that Tracleer® (bosentan), a dual endothelin receptor antagonist, has been approved in Canada for the treatment of patients with mildly symptomatic WHO Functional Class II (FC II) pulmonary arterial hypertension (PAH). Read article
Sildenafil Research Shows Benefits To Children With PAH
(Ukmedix) – Sildenafil has been shown to improve the peak oxygen consumption in children suffering from Pulmonary Arterial Hypertension. The research which was submitted at the Annual Congress of the European Respiratory Society in Vienna, Austria is a big step forward in the treatment of Pulmonary Arterial Hypertension and gives more information on exactly how and why Sildenafil is effective in treating patients with this condition. Read article
Long-Term Tadalafil Therapy Is Effective in Pulmonary Arterial Hypertension: Presented at ERS
(Doctor's Guide) – Results of the Pulmonary Arterial Hypertension and Response to Tadalafil (PHIRST)-1 and 2 study were presented by Ronald J. Oudiz, MD, Los Angeles Biomedical Research Institute, Harbor UCLA Medical Center, at the 19th Annual Congress of the European Respiratory Society (ERS). Read article
International Conference on Endothelin
(EurekaAlert) – As the scientific and medical communities involved in endothelin move towards 25 years of understanding the protein, which future developments hold potential? At what risk? Do medicinal compounds look promising? Donald Kohan, Professor of Medicine in the Division of Nephrology at the University of Utah Sciences Center and a leading global expert on endothelins is addressing these and other issues at the 11th International Conference on Endothelin being held September 9-12, 2009 in Montreal, CN. Read article
Actelion Launches Increased Strength Formulation of Ventavis (Iloprost) for PAH in the United States
(Reuters) – Actelion Pharmaceuticals US, Inc., today announced the first commercial sales of a new 20 microgram per milliliter (mcg/mL) formulation of Ventavis(R), for the treatment of New York Heart Association Class III and IV pulmonary arterial hypertension (PAH). Read article
New European Guidelines on Pulmonary Hypertension Provide New 6-Group Clinical Definition
(EurekaAlert) – New 2009 Guidelines for the diagnosis and treatment of pulmonary hypertension are made public today. The Guidelines have been jointly produced by a Task Force of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS); the Task Force also included experts from the International Society of Heart and Lung Transplantation (ISHLT). Read article
Cellular Crosstalk Linked to Lung Disease, Including PH
(EurekAlert!) – Crosstalk between cells lining the lung (epithelial cells) and airway smooth muscle cells is important in lung development. However, it has also been shown to contribute to several lung diseases, including asthma and pulmonary hypertension. Read article
FDA Expands Access to Investigational Drugs
(FDA) – On August 12, 2009, FDA announced changes to the rules to make them broader and clearer for the patient and the treating physician, while still preserving the integrity of clinical trials designed to find out whether a drug has a desired effect on some disease or condition. Read article
Actelion's Tracleer Gets US Approval For Wider Use
(Reuters) – Europe's biggest biotech company Actelion Ltd said on Monday its Tracleer drug had been granted approval for mildly symptomatic pulmonary arterial hypertension (PAH). Read article
Ventavis (Iloprost) Receives US Approval for Increased 20 mcg/ml Strength Formulation for Treatment of PAH
(Actelion) – Actelion Ltd announced today that the US Food and Drug Administration has approved a new 20 microgram per milliliter (mcg/ml) formulation of Ventavis® as a therapy for New York Heart Association Class III and IV pulmonary arterial hypertension (PAH). This new increased 20 mcg/ml strength formulation will deliver the same dose in half the volume which is expected to reduce inhalation time and further support patient compliance. Read article
Drug Heals Lung Damage in Ex-Smokers
(Los Angeles Times) – Iloprost, a drug routinely used to treat pulmonary hypertension – high blood pressure in the lungs – can heal the lungs of ex-smokers, but provides no benefit to those who continue to smoke, Colorado researchers reported today at a San Francisco meeting of the International Assn. for the Study of Lung Cancer. Read article
FDA Approves Tyvaso, Which is Inhaled Treprostinil, for the Treatment of Pulmonary Arterial Hypertension
(PR Newswire) – United Therapeutics Corporation announced today that the United States Food and Drug Administration (FDA) has approved Tyvaso, which is inhaled treprostinil, for the treatment of pulmonary arterial hypertension (PAH) using the TYVASO Inhalation System. Read article
Actelion To Start Late Stage Trial With PGI2 By End-2009
(Nasdaq – Switzerland) – Actelion Ltd. said it plans to start late-stage trials for its compound PGI2, a potential treatment for pulmonary arterial hypertension, by the end of the year after the compound proved safe and effective in a phase II trial. Read article
Hopkins Study Finds Double-Lung Transplant Better Than Single-Lung
(Examiner) - Lung transplant recipients who received two lungs are twice as likely to be alive ten years later than those who receive only one, researchers at the Johns Hopkins University School of Medicine have reported. Read article
Pulmonary Hypertension in Hemodialysis Patients Increases Death Risk
(Renal and Urology News) - Pulmonary hypertension independently predicts greater mortality in hemodialysis (HD) patients, Israeli researchers report. Read article
Study Results Present Efficacy and Safety Findings From the PHIRST-1 Study of Patients With Pulmonary Arterial Hypertension Taking Tadalafil Tablets Once Daily
(PR Newswire) – United Therapeutics Corporation and Eli Lilly and Company today announced the results of a pivotal 16-week study showing that a once-daily dose of tadalafil was generally well tolerated, improved exercise capacity and improved time to clinical worsening in patients with pulmonary arterial hypertension. Read article
New Pathway Found Underlying Pulmonary Hypertension
(ScienceDaily) – Pulmonary hypertension is an unremitting disease caused by a progressive increase in blood pressure in the blood vessels of the lung; it leads to heart failure and ultimately death. Currently there are limited treatment options. However, You-Yang Zhao and colleagues, at the University of Illinois College of Medicine, Chicago, have identified in mice a new molecular pathway underlying pulmonary hypertension that they hope might provide novel therapeutic targets. Read article
Research Presented at EHA Congress Shows That Soliris® Significantly Reduced Hemolysis in Never-Transfused Patients With PNH
(Product Design & Development) – Clinical investigators observed that Soliris(R) (eculizumab), a first-in-class terminal complement inhibitor developed by Alexion Pharmaceuticals, Inc., reduced hemolysis (red blood cell destruction) and improved symptoms in nine patients with paroxysmal nocturnal hemoglobinuria (PNH) who had received no blood transfusions prior to initiating Soliris therapy. — Abstract 1110: "Eculizumab Reduces Pulmonary Hypertension Through Inhibition of Haemolysis-Associated Nitric Oxide Consumption in Patients with Paroxysmal Nocturnal Hemoglobinuria," Dr. Anita Hill. Read article
New Use for Active Ingredient of Cialis
(WebMD) – Tadalafil, the Active Ingredient in ED Drug Cialis, Approved as Adcirca to Treat Pulmonary Arterial Hypertension. Read article
FDA approves Adcirca (Tadalafil) Tablets for Pulmonary Arterial Hypertension
(News-Medical.Net) – United Therapeutics Corporation announced today that the United States Food and Drug Administration (FDA) has approved Adcirca (tadalafil) tablets for oral administration, with a recommended dose of 40 mg, as the first once-daily phosphodiesterase type 5 (PDE5) inhibitor for the treatment of pulmonary arterial hypertension (PAH). Read article
Bayer Schering Presents Positive Results of Phase II Study With Riociguat
(Pharmacy Europe) – Positive data from a phase II trial with Bayer Schering Pharma’s oral agent riociguat (BAY 63-2521) in chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension (PAH) were presented today at the American Thoracic Society (ATS) international conference in San Diego, USA. Read article
New Data for Gilead Sciences' Ambrisentan Show Clinical Improvements in a Diverse Pulmonary Hypertension (PH) Population
(PharmiWeb) – Gilead Sciences, Inc. today announced results from ARIES-3, an open-label, single-arm, Phase III study evaluating the efficacy and safety of ambrisentan in patients with pulmonary hypertension (PH), which showed a mean 21-meter improvement from baseline in six-minute walk distance (6MWD) at 24 weeks. Read article
Sleep Apnea Thickens Blood Vessels, Increases Heart Disease Risk
(ScienceDaily) – Obstructive sleep apnea, or periodic interruptions in breathing throughout the night, thickens sufferers' blood vessels. Moreover, it increases the risk of several forms of heart and vascular disease. Emory researchers have identified the enzyme NADPH oxidase as important for the effects obstructive sleep apnea has on blood vessels in the lung. Read article
Sildenafil (Viagra) Eases Newborns’ Pulmonary Hypertension
( – Using sildenafil (Viagra) to treat severe, persistent pulmonary hypertension of the newborn can help them adapt to breathing outside the womb, according to a researcher. Read article
Pulmonary Hypertension May Predict Nonresponse of Heart Failure to Resynchronization Therapy
( – The presence of secondary pulmonary-artery hypertension may identify patients with systolic heart failure who are eligible or cardiac resynchronization therapy (CRT) but who are unlikely to gain any benefit from it, according to a preliminary study unveiled here at the International Society for Heart and Lung Transplantation 2009 Scientific Sessions. Read article
Bosentan (Tracleer®) Receives Positive CHMP Opinion For Pediatric Formulation
( – The Committee for Medicinal Products for Human Use has issued a positive opinion for Actelion Ltd.’s pediatric formulation of Bosentan (Tracleer®) for the treatment of pulmonary arterial hypertension. Currently, no drug is indicated for the treatment of PAH in children below 12 years of age; once licensed, Bosentan will be the only PAH treatment with a pediatric formulation. Read article
New Study Shows Long-Term Benefits for Patients with Pulmonary Arterial Hypertension

( – Results of a recent two-year study show promising long-term survival benefits for patients living with Pulmonary Arterial Hypertension. Research of new treatments has focused mainly on outcomes other than survival in shorter term three-to-12 month studies. Read article

Pulmonary Hypertension In Heart Failure With Preserved Ejection Fraction: A Community-Based Study
( – A recent study has found pulmonary hypertension is highly prevalent and often severe in heart failure due to diastolic dysfunction. Read article
Nippon Shinyaku Signs Agreement With Eli Lilly Japan and Eli Lilly And Company For Cialis
( – Pharmaceutical company Nippon Shinyaku Co., Ltd., Eli Lilly Japan K.K., and U.S.-based pharmaceutical company Eli Lilly and Company have signed a licensing agreement for Cialis. Under the terms of the agreement, Cialis would be exclusively promoted and sold by Nippon Shinyaku in Japan starting July 1. Read article
Darusentan encouraging in hard-to-treat hypertensives
( – The first of two phase 3 trials with a new endothelin receptor antagonist darusentan has shown promise in patients with resistant hypertension, Gilead Sciences in Foster City, Calif., reports. Read article
Biomarker Predicts Outcomes in Pulmonary Hypertension
( - High blood levels of a marker of inflammation and tissue damage, C-reactive protein (CRP), are associated with disease severity and survival in patients with pulmonary arterial hypertension (PAH), according to a report in the April 7 issue of the Journal of the American College of Cardiology. Read article
Limitations to the 6-Minute Walk Test in Interstitual Lung Disease and Pulmonary Hypertension in Scleroderma
( – The Journal of Rheumatology presents an abstract on a 6-minute walking test to determine factors that influence the 6-minute walk distance in patients with scleromerma-interstitial lung disease, SSc-pulmonary hypertension, and idiopathic pulmonary fibrosis. Read article
United Therapeutics Expects Delay On Drug
(Triangle Business Journal) – United Therapeutics said regulators at the FDA have concerns about the patient instructions on the nebulizer used to deliver the drug, known as Tyvaso. Read article
New AGH Institute Strives For Groundbreaking Cardiovascular Research
(WTAE-TV4, Pittsburgh) A new research facility in Pittsburgh could make a big difference in the lives of people with a history of pulmonary hypertension in their family. Allegheny General Hospital is opening a new research institute, which focuses on genetic problems and solutions. Read article
Pulmonary Hypertension in Children May Result from the Reduced Activity of Gene Regulator
( Too little activity be gene regulators called PPARS appears to be a major player in the irreversible lung damage that can occur in children with heart defects, researchers say. Read article
FDA Approves SYMBICORT(R) for Chronic Obstructive Pulmonary Disease (COPD)
(PR Newswire) AstraZeneca today announced that the U.S. Food and Drug Administration (FDA) has approved SYMBICORT (budesonide/formoterol fumarate dihydrate) 160/4.5 mcg for the twice daily maintenance treatment of airflow obstruction in patients with chronic obstructive pulmonary disease (COPD), including chronic bronchitis and emphysema. Read article
Actelion Announces Acquisition of a New Formulation of Intravenous Epoprostenol with Improved Therma
(ALLSCHWIL/BASEL, SWITZERLAND ) Actelion announced today it has entered into a definitive agreement to acquire an improved, thermostable formulation of epoprostenol sodium for the intravenous treatment of pulmonary arterial hypertension (PAH) from privately-held GeneraMedix Inc. (GXI). Read article (pdf)
Bayer Starts Phase III Study of Lung Drug riociguat
(Reuters, United Kingdom) - Bayer AG (BAYG.DE) has entered phase III of testing its experimental pulmonary-hypertension drug riociguat after the pill showed promise in an earlier trial, the company said on Monday. Read article
23andMe and mondoBIOTECH Partner to Advance Research of Rare Diseases
(PR News wire, Switzerland) - 23andMe and mondoBIOTECH will work together to facilitate research of the genetic bases of rare and potentially fatal diseases, such as Pulmonary Arterial Hypertension, Sarcoidosis, and Pulmonary Fibrosis, the genetics of which are poorly understood. Read article

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