A case of ruptured sinus of Valsalva aneurysm and reversible flow-induced pulmonary hypertension

Pulmonary hypertension (PH) in adults with congenital heart disease (CHD) and significant systemic-to-pulmonary shunting is a significant cause of morbidity and mortality. Its pathophysiology is incompletely understood, but involves a flow-induced pulmonary arteriopathy characterized by endothelial...

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Main Authors: Basil Al-Sabeq, Sabe De, Ryan Davey
Format: Article
Language:English
Published: Wiley 2018-03-01
Series:Pulmonary Circulation
Online Access:https://doi.org/10.1177/2045894018760656
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author Basil Al-Sabeq
Sabe De
Ryan Davey
author_facet Basil Al-Sabeq
Sabe De
Ryan Davey
author_sort Basil Al-Sabeq
collection DOAJ
description Pulmonary hypertension (PH) in adults with congenital heart disease (CHD) and significant systemic-to-pulmonary shunting is a significant cause of morbidity and mortality. Its pathophysiology is incompletely understood, but involves a flow-induced pulmonary arteriopathy characterized by endothelial cell dysfunction and vascular remodeling that alters pulmonary arterial vasoreactivity. There is a paucity of literature linking PH with left-to-right shunting due to ruptured sinus of Valsalva aneurysms (SOVA). We present a unique case of reversible, flow-associated PH due to a ruptured congenital right SOVA fistulizing into the right atrium (RA), with emphasis on non-invasive and invasive assessment of pulmonary hemodynamics before and after surgical intervention.
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spelling doaj.art-ab5ddf8729d94168a44df6c6455939742022-12-22T03:37:47ZengWileyPulmonary Circulation2045-89402018-03-01810.1177/2045894018760656A case of ruptured sinus of Valsalva aneurysm and reversible flow-induced pulmonary hypertensionBasil Al-SabeqSabe DeRyan DaveyPulmonary hypertension (PH) in adults with congenital heart disease (CHD) and significant systemic-to-pulmonary shunting is a significant cause of morbidity and mortality. Its pathophysiology is incompletely understood, but involves a flow-induced pulmonary arteriopathy characterized by endothelial cell dysfunction and vascular remodeling that alters pulmonary arterial vasoreactivity. There is a paucity of literature linking PH with left-to-right shunting due to ruptured sinus of Valsalva aneurysms (SOVA). We present a unique case of reversible, flow-associated PH due to a ruptured congenital right SOVA fistulizing into the right atrium (RA), with emphasis on non-invasive and invasive assessment of pulmonary hemodynamics before and after surgical intervention.https://doi.org/10.1177/2045894018760656
spellingShingle Basil Al-Sabeq
Sabe De
Ryan Davey
A case of ruptured sinus of Valsalva aneurysm and reversible flow-induced pulmonary hypertension
Pulmonary Circulation
title A case of ruptured sinus of Valsalva aneurysm and reversible flow-induced pulmonary hypertension
title_full A case of ruptured sinus of Valsalva aneurysm and reversible flow-induced pulmonary hypertension
title_fullStr A case of ruptured sinus of Valsalva aneurysm and reversible flow-induced pulmonary hypertension
title_full_unstemmed A case of ruptured sinus of Valsalva aneurysm and reversible flow-induced pulmonary hypertension
title_short A case of ruptured sinus of Valsalva aneurysm and reversible flow-induced pulmonary hypertension
title_sort case of ruptured sinus of valsalva aneurysm and reversible flow induced pulmonary hypertension
url https://doi.org/10.1177/2045894018760656
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