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Multiple peripheral pulmonary artery stenoses in adults: a rare cause of severe pulmonary hypertension necessitating lung transplantation


Ulrich, S; Fischler, M; Pfammatter, T; Vogt, P; Weder, W; Boehler, A; Speich, R (2005). Multiple peripheral pulmonary artery stenoses in adults: a rare cause of severe pulmonary hypertension necessitating lung transplantation. Journal of Heart and Lung Transplantation, 24(11):1984-1987.

Abstract

Stenoses of multiple peripheral pulmonary artery branches represent a rare cause of pulmonary hypertension in children, but the prognosis is very poor for such patients. Herein we describe 2 patients with multiple peripheral pulmonary artery stenoses (MPPAS) presenting with severe pulmonary arterial hypertension in adulthood, which has only once been described previously. Both patients lived without significant health problems for decades; however, after onset of symptoms, their medical condition declined rapidly, necessitating lung transplantation several months after the diagnosis despite vasodilator therapy. Because MPPAS mimics chronic thromboembolic pulmonary hypertension, this entity may being underdiagnosed, decreasing the possibility of adequate therapy.

Abstract

Stenoses of multiple peripheral pulmonary artery branches represent a rare cause of pulmonary hypertension in children, but the prognosis is very poor for such patients. Herein we describe 2 patients with multiple peripheral pulmonary artery stenoses (MPPAS) presenting with severe pulmonary arterial hypertension in adulthood, which has only once been described previously. Both patients lived without significant health problems for decades; however, after onset of symptoms, their medical condition declined rapidly, necessitating lung transplantation several months after the diagnosis despite vasodilator therapy. Because MPPAS mimics chronic thromboembolic pulmonary hypertension, this entity may being underdiagnosed, decreasing the possibility of adequate therapy.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic and Policlinic for Internal Medicine
Dewey Decimal Classification:610 Medicine & health
Date:2005
Deposited On:17 Apr 2013 10:50
Last Modified:07 Dec 2017 20:18
Publisher:Elsevier
ISSN:1053-2498
Publisher DOI:https://doi.org/10.1016/j.healun.2005.02.006
PubMed ID:16297810

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