Abstract

BackgroundThis report describes 9 high-risk neonates and infants with hypoplastic left heart syndrome (HLHS) who were stabilized with a combined hybrid approach and ventricular assist device (VAD) insertion (HYBRID+VAD) in preparation for heart transplantation. MethodsA total of 9 patients with HLHS (7 neonates, 2 infants) presented with anatomic or physiologic features associated with an increased risk for conventional univentricular palliation with the Norwood operation (large coronary sinusoids or fistulas, severe tricuspid regurgitation, cardiogenic shock, restrictive atrial septum). These patients underwent combined VAD insertion (Berlin EXCOR, Berlin Heart, Inc, Berlin, Germany) and Stage 1 hybrid palliation (application of bilateral pulmonary bands, stent placement in the patent arterial duct, and atrial septectomy if needed). During this same era, at the Congenital Heart Center, University of Florida, Gainesville, Florida, 46 neonates underwent a Norwood operation, 4 neonates underwent a hybrid approach “Stage 1” without VAD, and 3 patients with HLHS were supported with prostaglandin while awaiting heart transplantation. ResultsAt HYBRID+VAD insertion, the median age was 20 days (range, 13 to 143 days), and median weight was 3.25 kg (range, 2.43 to 4.2 kg). Six patients survive (67%), and 3 patients died (33%). Five survivors are at home doing well after successful heart transplantation, and 1 survivor is doing well in the intensive care unit on VAD support while awaiting transplantation. Only 1 of 6 survivors (16.7%) required intubation more than 10 days after HYBRID+VAD insertion. In 8 patients no longer undergoing VAD support, the median length of VAD support was 119.5 days (range, 56 to 196 days). ConclusionsHigh-risk patients with HLHS who are suboptimal candidates for Norwood palliation can be successfully stabilized with pulsatile VAD insertion along with hybrid palliation while awaiting cardiac transplantation. These patients may be extubated and optimized for transplantation while undergoing VAD support.

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