Selective Coronary Artery Angiography in Hypoplastic Left Heart Syndrome

Coronary artery disease in palliated hypoplastic left heart syndrome is uncommon. Myocardial infarction from a coronary thrombus, serving as a substrate for ventricular arrhythmia in Fontan physiology, is under-reported despite known hypercoagulopathic state. Traditional risk factors for coronary artery occlusion include intracardiac thrombi, hyperlipidemia, and hypertension. Baffle leaks and abnormal ventriculocoronary fistulae found in these patients are contributing factors. We sought to assess and describe coronary artery involvement in this complex patient population. Our research highlights both the need to assess distal coronary vasculature and to thoroughly evaluate hemodynamics and biventricular function with new-onset troponin leak or ventricular arrhythmias.