Familial primary pulmonary hypertension.
N Z Med J. 2004 Jun 18;117(1196):U936
Park D, Beckert L.
Department of Respiratory Medicine, Christchurch Hospital, Christchurch, New
Zealand. Lutz.Beckert@chhb.govt.nz
Publication Types:
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Case
Reports
Living-related lobar transplantation and simultaneous
atrial septal defect closure in a young patient with irreversible pulmonary
hypertension: a case report.
Heart Vessels. 2004 Jul;19(4):203-7
Matsuda H, Minami M, Ichikawa H, Fukushima N, Ohta M, Saito
M, Kita T, Matsushita T.
Department of Surgery, 2-2 Yamada-oka, Suita, 562-0027, Osaka, Japan,
matsuda@surg1.med.osaka-u.ac.jp
A 6-year-old boy was diagnosed as having atrial septal defect with oversystemic
pulmonary hypertension, and gradually developed hypoxia and heart failure. At
the age of 11, living-related bilateral lobar lung transplantation from his
parents was indicated, because of his critical condition. The estimated forced
vital capacity calculated by the donors' lower lobes was 104% of his age. The
operation was carried out with simultaneous closure of the atrial septal defect
under full cardiopulmonary bypass. The postoperative course was complicated
with pulmonary edema and phrenic nerve palsy, which were eventually resolved.
Six months after surgery he was free from heart failure and rehabilitating at
home without oxygen. The final diagnosis was primary pulmonary hypertension
with atrial septal defect. Living-related bilateral lobar lung transplantation
with simultaneous intracardiac repair may be an optional strategy for children
with Eisenmenger syndrome or primary pulmonary hypertension with intracardiac
defect when cadaveric transplantation is not possible.
Stenting in primary pulmonary hypertension with compression
of the left main coronary artery.
Rev Esp Cardiol. 2004 Jul;57(7):695-8
[Article in English, Spanish]
Gomez Varela S, Montes Orbe PM, Alcibar Villa J, Egurbide MV, Sainz I,
Barrenetxea Benguria JI.
Servicio de Cardiologia. Hospital de Cruces. Baracaldo. Vizcaya. Spain.
Primary pulmonary hypertension is often associated with angina-like chest pain
of uncertain etiology. Left main coronary artery compression by the pulmonary
artery is a treatable cause of angina and should be considered in these
patients. We describe a patient presenting with primary pulmonary hypertension,
clinical angina and extrinsic compression of the left main coronary artery by
the pulmonary artery, who was treated with direct stenting.
Chronic thromboembolic pulmonary arterial hypertension:
correlation of postoperative results of thromboendarterectomy with preoperative
helical contrast-enhanced computed tomography.
J Thorac Imaging. 2004 Apr;19(2):67-73
Oikonomou A, Dennie CJ, Muller NL, Seely JM, Matzinger FR,
Rubens FD.
Department of Radiology, The Ottawa Hospital, Ontario, Canada.
aoikonom@med.duth.gr
INTRODUCTION: Pulmonary thromboendarterectomy is the treatment of choice for
patients with chronic thromboembolic pulmonary arterial hypertension (CTEPH).
Some patients do poorly after this procedure and may be better candidates for
heart-lung transplant. The purpose of this study was to correlate preoperative
findings on helical contrast-enhanced computed tomography (CT) with surgical
outcome. METHODS: Thirty-seven patients (mean age 52.9, range 22-71) who
underwent pulmonary thromboendarterectomy and had preoperative helical
contrast-enhanced CT followed by High Resolution CT (HRCT) scans were included
in the study. The CTs were evaluated for the presence of central and segmental
disease and for the presence of mosaic perfusion pattern. RESULTS: The presence
of central disease, as well as the presence of segmental disease, correlated
negatively with the postoperative mean pulmonary arterial pressure [r(c) =
-0.401, P = 0.015, r(s) = -0.38, P = 0.024)] and the pulmonary vascular
resistance [(r(c) = -0.37, P = 0.027, r(s) = -0.39, P = 0.019]. No correlation
was found between the clinical variables and the presence of mosaic perfusion
pattern. CONCLUSION: Patients with CTEPH and evidence of chronic PE in the
central or segmental pulmonary arteries have a better clinical outcome after
pulmonary thromboendarterectomy than patients without these findings. The
presence of mosaic perfusion pattern is not helpful in predicting postoperative
outcome.
Phosphodiesterases in the vascular system.
J Smooth Muscle Res. 2003 Aug;39(4):67-86
Matsumoto T,
Kobayashi T, Kamata K.
Department of Physiology and
Morphology, Institute of Medicinal Chemistry, Hoshi University, Shinagawa-ku,
Tokyo 142-8501, Japan.
Cyclic adenosine 3',5'-monophosphate (cAMP) and cyclic guanosine
3',5'-monophosphate (cGMP) are second messengers involved in the intracellular
signal transduction of a variety of extracellular stimuli in several tissues.
In the vascular system, these nucleotides play important roles in the
regulation of vascular tone and in the maintenance of the mature contractile
phenotype in smooth muscle cells. Given that cyclic nucleotide signaling
regulates a wide variety of cellular functions, it is not surprising that
cyclic nucleotide phosphodiesterases (PDEs). In paticular, the accumulating
data showing that there are a large number of different PDE isozymes have
triggered an equally large increase in interest about these enzymes. At least
11 different gene families of PDEs are currently known to exist in mammalian
tissues. Most families contain several distinct genes, and many of these genes
are expressed in different tissues as functionally unique alternative splice
variants. This article reviews many of the important aspects about the
structure, cellular localization, and regulation of each family of PDEs.
Particular emphasis is placed on new information obtained in the last few years
about vascular disease. The development of novel methods to deliver more potent
and selective PDE inhibitors to individual cell types and subcellular locations
will lead to new therapeutic uses for this class of drugs in diseases of the
vascular system.
Publication Types:
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Review
·
Review,
Tutorial
Vasopressin during spinal anesthesia in a patient with
primary pulmonary hypertension treated with intravenous epoprostenol.
Anesth Analg. 2004 Jul;99(1):36-7.
Braun EB, Palin CA, Hogue CW.
Department of Anesthesiology, Washington University School of Medicine, St.
Louis, Missouri, USA.
Primary pulmonary hypertension (PPH) is a progressive disease with frequent
morbidity and mortality, including the risk of cardiac decompensation and
death, during general anesthesia. Administration of IV epoprostenol (Flolan)
improves symptoms and survival of patients with PPH and thus is an increasingly
used long-term treatment for this condition. This therapy is associated with impaired
platelet aggregation, which may complicate the perioperative management of
patients with PPH. We present a case report of a patient with severe PPH
receiving a continuous epoprostenol infusion undergoing skin grafting for a leg
ulcer under spinal anesthesia. An IV infusion of vasopressin was given to
prevent systemic hypotension resulting from sympathetic blockade while avoiding
increases in pulmonary vascular resistance that may have resulted from
catecholamine usage.
Pulmonary arterial hypertension: a look to the future.
J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):89S-90S
Rubin LJ, Galie N.
Pulmonary Vascular Center, University of California-San Diego School of
Medicine, La Jolla, California, USA. ljrubin@ucsd.edu
The Third World Symposium on Pulmonary Arterial Hypertension served not only as
a forum for the presentation of state-of-the art overviews of the pathobiologic
and clinical aspects of pulmonary arterial hypertension (PAH), but also
afforded an opportunity to the international scientific community to explore
future directions of research and collaboration. This summary provides a brief
overview of future directions in the field.
Publication Types:
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Review
·
Review,
Tutorial
Comparative analysis of clinical trials and evidence-based treatment
algorithm in pulmonary arterial hypertension.
J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):81S-88S
Galie N, Seeger W, Naeije R, Simonneau G, Rubin LJ.
Institute of Cardiology, University of Bologna, Bologna, Italy.
n.galie@bo.nettuno.it
The numerous controlled clinical trials performed recently in pulmonary
arterial hypertension (PAH) can allow us to abandon a clinical-based treatment
strategy and adopt an evidence-based therapy. Both uncontrolled and controlled
clinical trials with different compounds and procedures are reviewed and
compared in order to define the efficacy-to-side-effect ratio of each
treatment. A grading system for the level of evidence of treatments based on
the number of favorable controlled clinical trials performed with a given
compound is adopted; a treatment algorithm based on the evidence derived by
clinical trials is proposed. It includes drugs approved by regulatory agencies
for the treatment of patients with PAH and/or drugs available on the market for
other indications. The algorithm is restricted to patients in New York Heart
Association (NYHA) functional class III or IV because they represent the
largest population included in controlled clinical trials. In addition, the
different treatments have been evaluated mainly in sporadic, idiopathic PAH and
in PAH associated with scleroderma or to anorexigen use. Extrapolation of these
recommendations to the other PAH subgroups should be done with caution. Oral
anticoagulation is proposed for all patients, whereas diuretic treatment and
supplemental oxygen are indicated in cases of fluid retention and hypoxemia,
respectively. High doses of calcium channel blockers are indicated only in the
minority of patients who are responders to acute vasoreactivity testing.
Nonresponders to acute vasoreactivity testing, or responders who remain in NYHA
functional class III, should be considered candidates for treatment with either
an endothelin receptor antagonist or a prostanoid. Continuous intravenous
administration of epoprostenol is proposed as rescue treatment in NYHA
functional class IV patients. Phosphodiesterase-V inhibitors should be
considered in patients who have failed or are not candidates to other
therapies. Combination therapy can be attempted in selected cases. Both balloon
atrial septostomy and lung transplantation are indicated for refractory
patients or where medical treatment is unavailable.
Publication Types:
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Review
·
Review,
Tutorial
Interventional and surgical modalities of treatment for pulmonary arterial
hypertension.
J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):73S-80S
Klepetko W, Mayer E, Sandoval J, Trulock EP, Vachiery JL,
Dartevelle P, Pepke-Zaba J, Jamieson SW, Lang I, Corris P.
Department of Cardio-Thoracic Surgery, Vienna University Hospital, Vienna,
Austria. walter.klepetko@meduniwien.ac.at
Beyond medical therapy, different interventional and surgical approaches exist
for treatment of pulmonary arterial hypertension (PAH). Atrial septostomy has
been applied in patients with lack of response to medical therapy in the
absence of other surgical treatment options. With growing experience,
procedure-related death rates have been reduced to 5.4%, and the most suitable
patient group has been identified among patients with a mean right atrial
pressure between 10 and 20 mm Hg. Pulmonary endarterectomy is the accepted form
of treatment for patients with chronic thromboembolic pulmonary hypertension.
Establishing the diagnosis and the classification of the type of lesions by
pulmonary angiography is crucial for optimal patient selection. Perioperative
mortality rates have been reduced to <10% in experienced centers, and the
hemodynamic improvement is dramatic and sustained. Lung and heart-lung
transplantation remains the procedure of choice for patients unsuitable for
other treatment modalities. Timing of the procedure is difficult because
waiting times vary between centers and usually are in a high range. Early
referral of patients unresponsive to other treatment forms is therefore of
importance to avoid transplantation of patients with established significant
comorbidity. The survival rate during the first five years after
transplantation for PAH is intermediate among the lung diseases, lower than
chronic obstructive pulmonary disease but higher than idiopathic pulmonary
fibrosis.
Publication Types:
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Review
·
Review,
Tutorial
Nitric oxide pathway and phosphodiesterase inhibitors in pulmonary arterial
hypertension.
J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):68S-72S
Ghofrani HA, Pepke-Zaba J, Barbera JA, Channick R, Keogh
AM, Gomez-Sanchez MA, Kneussl M, Grimminger F.
Department of Internal Medicine Pulmonary Hypertension Center, University
Hospital, Giessen, Germany. ardeschir.ghofrani@innere.med.uni-giessen.de
Pulmonary hypertension (PH) is a disease of various origins. Nitric oxide-a
potent vasodilator-is a key player of pulmonary vasoregulation. Nitric oxide
signaling is mainly mediated by the guanylate cyclase/cyclic guanylate
monophosphate pathway. The effects of this second messenger system are limited
by enzymatic degradation through phosphodiesterases (PDEs). Recently,
beneficial effects of the oral PDE-5 inhibitor sildenafil (originally approved
for the treatment of erectile dysfunction) were reported for the treatment of
PH. We provide a brief overview of the experimental and clinical application of
PDE inhibitors in the field of PH. In particular, studies reporting the
clinical effectiveness of sildenafil are highlighted. This agent, despite oral
application, displays characteristics of