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

Pulmonary Circulation
Basil Al-SabeqRyan Davey

Abstract

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.

References

Sep 2, 2005·The European Respiratory Journal·M E van AlbadaR M F Berger
Apr 28, 2006·Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual·David A Ott
Dec 22, 2009·American Journal of Physiology. Lung Cellular and Molecular Physiology·Mirjam E van AlbadaRolf M F Berger
May 24, 2016·The Annals of Thoracic Surgery·Awad El-Ashry, Anthony L Estrera
Jun 14, 2016·Heart Views : the Official Journal of the Gulf Heart Association·Neeraj K GanjuSunite A Ganju
Sep 20, 2016·Hellenic Journal of Cardiology : HJC = Hellēnikē Kardiologikē Epitheōrēsē·George Giannakoulas, Michael A Gatzoulis
Aug 9, 2017·Echocardiography·Pei-Chun McGregorJayashri R Aragam

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