Transcatheter device occlusion of the left ventricular outflow tract as treatment for severe aortic regurgitation in hypoplastic left heart syndrome

Aortic regurgitation is a rare but dangerous condition in patients with hypoplastic left heart syndrome (HLHS). We report the case of a 2-year-old girl with HLHS with aortic/mitral stenosis (AS-MS) subtype after stage II palliation via the bilateral bidirectional Glenn (BBDG) procedure, when aortic regurgitation presented with the clinical manifestation of a paroxysmal coronary steal phenomenon provoked by exertion. Transcatheter closure of the left ventricular outflow tract (LVOT) with an AmplatzerTM Vascular Plug 4 minimized and finally abolished aortic reflux without compromising either coronary perfusion or atrioventricular (AV) conduction. Midterm follow-up over 3 years revealed complete disappearance of her symptoms, and follow-up catheterization displayed complete LVOT closure. This case illustrates the promising potential of modern cardiac catherization techniques in certain cases and emphasizes that precise assessment of the native aorta and coronary system is critical in HLHS patients. © 2015 Wiley Periodicals, Inc.