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.