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Author Poscia, R. ♦ Vizza, C. D. ♦ Pezzuto, B. ♦ Papa, S. ♦ Badagliacca, R. ♦ Gambardella, C. ♦ Fedele, F. ♦ Crescenzi, E.
Source Directory of Open Access Journals (DOAJ)
Content type Text
Publisher European Respiratory Society
File Format HTM / HTML
Date Created 2009-09-02
Copyright Year ©2009
Language English
Subject Domain (in LCC) RC705-779
Subject Keyword Diseases of the respiratory system ♦ Internal medicine ♦ Congenital heart disease ♦ Medicine ♦ Pulmonary arterial hypertension ♦ Specialties of internal medicine
Abstract A 63-yr-old black female, with a 1-yr history of hepatitis C and ascites was referred to an expert centre with suspicion of portopulmonary hypertension (PPHTN). Her poor condition made a rapid diagnosis imperative and precluded a normal diagnostic work-up. Echocardiography confirmed severe pulmonary hypertension (PH). A hepatic scintigraphy and an abdominal echo-Doppler study excluded liver cirrhosis and portal hypertension. Cardiac magnetic resonance imaging showed marked dilation of the right ventricle with significant hypertrophy of the free wall, a finding that is uncommon in idiopathic pulmonary arterial hypertension or PPHTN. Right heart catheterisation demonstrated severe pre-capillary PH without response to acute vasodilator testing. Finally the patient underwent computed tomography angiography, which showed marked dilation of the pulmonary artery without thromboembolic disease and, unexpectedly, a partially calcified large patent ductus arteriosus. The correct diagnosis of the underlying cause of pulmonary arterial hypertension is essential. Patients with underlying heart defects may have an atypical presentation and be referred to expert centres with an incorrect diagnosis. A full investigation is necessary; careful examination of right ventricular anatomy can provide clues about the aetiology of PH, and it is important to exclude intra- and extracardiac shunts during haemodynamic studies.
ISSN 16000617
Age Range 18 to 22 years ♦ above 22 year
Educational Use Research
Education Level UG and PG ♦ Career/Technical Study
Learning Resource Type Article
Publisher Date 2009-09-01
e-ISSN 09059180
Journal European Respiratory Review
Volume Number 18
Issue Number 113
Page Count 3
Starting Page 174
Ending Page 176


Source: Directory of Open Access Journals (DOAJ)