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 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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Heart Arrest/ Block/ Defects/ Diseases/ Failure/ Rate/ Infarction
Pulmonary Hypertension


Basil Al-Sabeq, Sabe De, Ryan Davey

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Pulmonary Circulation Vol 8: No 2 cover image

June 2018

Pulmonary Circulation Vol 8: No 2

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