Acquired ventricular septal defect in the setting of a catecholaminergic storm

A 58-year-old man was admitted to the cardiac intensive care unit following an episode of chest pain and severe hypertension (277/110 mmHg) during haemodialysis. Electrocardiography showed intermittent left bundle branch block, and high-sensitivity troponin I peaked at 27 582 ng/L. A loud holosystolic murmur was noted the following day. Transthoracic echocardiography revealed a 15 mm ventricular septal defect (VSD; Figure A, Supplementary data online, Video S1), while cardiac catheterization confirmed a significant left-to-right shunt (Qp:Qs 2.2) with normal coronary arteries (see Supplementary data online, Video S2). Cardiac magnetic resonance imaging showed no late gadolinium enhancement (Figure B). Given marked blood pressure lability and recurrent hypertensive crises, secondary causes were investigated. Plasma metanephrines were elevated, and 18F-DOPA positron emission tomography identified a right adrenal lesion consistent with pheochromocytoma (white arrow; Figure C), which was surgically resected, resulting in blood pressure control. At follow-up, signs of left ventricular volume overload and rising pulmonary pressures prompted consideration of VSD closure. Owing to comorbidities, the Heart Team favoured a percutaneous approach with surgical backup. Patient-specific three-dimensional printing enabled detailed characterization of the tortuous VSD trajectory and its relationship with adjacent structures, particularly its proximity to the right ventricular free wall, informing device type, size, and delivery strategy (Figure D, Supplementary data online, Video S3). Percutaneous closure was successfully performed using a dedicated VSD occluder under fluoroscopic and transoesophageal echocardiographic guidance (Figure E–1F, Supplementary data online, Video S4). The post-procedural course was uneventful. At 2-month follow-up, there was significant clinical and haemodynamic improvement (Qp:Qs 1.2). To our knowledge, this is the first reported case of an acquired VSD associated with pheochromocytoma.