Lymphangitis carcinomatosis of unknown origin presenting as severe pulmonary hypertension.

Am J Med Sci. 2004 May;327(5):255-7

Maza I, Braun E, Plotkin A, Guralnik L, Azzam ZS.

Department of Internal Medicine B, Rambam Medical Center, Haifa, Israel.

An apparently healthy 46-year-old woman was admitted because of progressive shortness of breath that had begun 2 months before her admission. Physical examination revealed a patient with respiratory distress, tachycardia, and mild jugular venous distention; otherwise, results were unremarkable. Our investigation revealed hypoxia and severe pulmonary hypertension with signs of right heart dysfunction, but no primary cause was found. The patient died 5 days after admission. Autopsy revealed pulmonary lymphangitis carcinomatosis caused by papillary carcinoma. No primary tumor was found.

Peripartum substitution of inhaled for intravenous prostacyclin in a patient with primary pulmonary hypertension.

Anesthesiology. 2004 Jun;100(6):1603-5

Hill LL, De Wet CJ, Jacobsohn E, Leighton BL, Tymkew H.

Department of Anesthesiology and Division of Cardiothoracic Surgery, Washington University School of Medicine, St. Louis, Missouri 63110, USA.

Publication Types:

● Case Reports

Paradoxical effect of prostacyclin infusion in a patient with primary pulmonary hypertension-a case report.

Angiology. 2004 May-Jun;55(3):341-4

Younger JR, Lui CY.

Department of Cardiology, Sarver Heart Center, University of Arizona, Tucson, AZ 85724, USA.

Prostacyclin treatment successfully delays the need for lung transplantation in many patients with progressive primary pulmonary hypertension by vasodilating pulmonary arteries. However, the treatment of pulmonary hypertension with prostacyclin may cause a paradoxical increase in pulmonary artery pressure, as shown in this case.

Publication Types:

● Case Reports

A case of scleroderma spectrum disorder with anticentriole antibody and pulmonary hypertension.

Clin Rheumatol. 2004 Jun;23(3):266-8. Epub 2004 Apr 14

Hayakawa I, Sato S, Hasegawa M, Echigo T, Takehara K.

Department of Dermatology, Kanazawa University, Graduate School of Medical Science, 13-1 Takaramachi, Ishikawa 920-8641, Kanazawa, Japan.

We describe the case of a patient with anticentriole antibody-positive scleroderma spectrum disorder (SSD) who developed pulmonary hypertension. A 54-year-old woman had noticed Raynaud's phenomenon and digital ulcers during the winter for the past 10 years. Although sclerodactyly was not present, digital ulcers, swelling of her hands, and phalangeal contracture were observed. An indirect immunofluorescence test revealed anticentriole antibody. Other SSc-specific antoantibodies were negative. An echocardiogram demonstrated that the estimated right ventricular systolic pressure was increased to 51 mmHg. She was diagnosed as SSD with pulmonary hypertension. This is the first case of SSD with anticentriole antibody to develop pulmonary hypertension.

Hepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant database.

Liver Transpl. 2004 Feb;10(2):174-82

Krowka MJ, Mandell MS, Ramsay MA, Kawut SM, Fallon MB, Manzarbeitia C, Pardo M Jr, Marotta P, Uemoto S, Stoffel MP, Benson JT.

Mayo Clinic, Rochester, Minnesota 55905, USA. krowka@mayo.edu

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PortoPH) are pulmonary vascular consequences of advanced liver disease associated with significant mortality after orthotopic liver transplantation (OLT). Data from 10 liver transplant centers were collected from 1996 to 2001 that characterized the outcome of patients with either HPS (n = 40) or PortoPH (n = 66) referred for OLT. Key variables (PaO2 for HPS, mean pulmonary artery pressure [MPAP], pulmonary vascular resistance [PVR], and cardiac output [CO] for PortoPH) were analyzed with respect to 3 definitive outcomes (those denied OLT, transplant hospitalization survivors, and transplant hospitalization nonsurvivors). OLT was denied in 8 of 40 patients (20%) with HPS and 30 of 66 patients (45%) with PortoPH. Patients with HPS who were denied OLT had significantly worse PaO2 compared with patients who underwent transplantation (47 vs. 52 mm Hg, P <.005). Transplant hospitalization survival was associated with higher pre-OLT PaO2 (55 vs. 37 mm Hg; P <.005). MPAP was significantly higher (53 vs. 45 mm Hg; P <.015) and PVR was significantly worse (614 vs. 335 dynes. s. cm(-5); P <.05) in patients with PortoPH who were denied OLT compared with patients who underwent transplantation. Transplant hospitalization mortality was 16% (5/32) in patients with HPS and 36% (13/36) in patients with PortoPH. All of the deaths in patients with PortoPH occurred within 18 days of OLT; 5 of the 13 deaths in patients with PortoPH occurred intraoperatively. We concluded that patients with HPS (based on a combination of low PaO2 and nonpulmonary factors) and patients with PortoPH (based on pulmonary hemodynamics) were frequently denied OLT because of pre-OLT test results and comorbidities. For patients who subsequently underwent OLT, transplant hospitalization mortality remained significant for both those with HPS (16%) and PortoPH (36%).

Publication Types:

● Multicenter Study

Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism.

Pengo V, Lensing AW, Prins MH, Marchiori A, Davidson BL, Tiozzo F, Albanese P, Biasiolo A, Pegoraro C, Iliceto S, Prandoni P; Thromboembolic Pulmonary Hypertension Study Group.

N Engl J Med. 2004 May 27;350(22):2257-64.

Department of Clinical and Experimental Medicine, Division of Clinical Cardiology, University Hospital of Padua, Padua, Italy.

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTPH) is associated with considerable morbidity and mortality. Its incidence after pulmonary embolism and associated risk factors are not well documented. METHODS: We conducted a prospective, long-term, follow-up study to assess the incidence of symptomatic CTPH in consecutive patients with an acute episode of pulmonary embolism but without prior venous thromboembolism. Patients with unexplained persistent dyspnea during follow-up underwent transthoracic echocardiography and, if supportive findings were present, ventilation-perfusion lung scanning and pulmonary angiography. CTPH was considered to be present if systolic and mean pulmonary-artery