Angiotensin-Converting Enzyme Inhibitor Prescription for Patients With Single Ventricle Physiology Enrolled in the NPC-QIC Registry

Background The routine use of angiotensin-converting enzyme inhibitors (ACEI) during palliation of hypoplastic left heart syndrome is controversial. We sought to describe ACEI prescription in the interstage between stage 1 palliation (stage I Norwood procedure) discharge and stage 2 palliation (stage II superior cavopulmonary anastomosis procedure) admission using the NPC-QIC (National Pediatric Cardiology Quality Improvement Collaborative) registry. Methods and Results Analysis of all patients (n=2180) enrolled in NPC-QIC from 2008 to 2016 included preoperative anatomy, risk factors, and echocardiographic data. ACEI were prescribed at stage I Norwood procedure discharge in 38% of patients. ACEI prescription declined from 2011 to 2016 compared with pre-2010 (36.8% versus 45%; P=0.005) with significant variation across centers (range 7-100%; P