Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome

Pediatric Cardiology
Mark Galantowicz, John P Cheatham

Abstract

Despite improvements in surgical techniques and perioperative management, the overall results of traditional staged palliation for hypoplastic left heart syndrome (HLHS) remain suboptimal. We report the evolution of a hybrid strategy in 34 patients coupling innovative surgical and transcatheter techniques that requires one open-heart procedure to reach a Fontan completion. Experience has led to modifications in technique, timing of procedures, and follow-up. Current initial palliation is by intraoperative, off-pump, placement of bilateral pulmonary artery bands and a PDA stent. Transcatheter creation of an unrestricted atrial septal defect is performed when necessary. Follow-up includes weekly cardiology assessment, including echocardiography with a surveillance catheterization performed at 6 weeks. At 3-6 months a comprehensive stage 2 procedure (elements of traditional stages 1-3) is performed. At 2 years, transcatheter Fontan completion with a covered stent is performed. Initial hybrid stage 1 was from August 2001 to December 2004 and included 29 newborns (1.8-4.2 kg). There were five hospital deaths and three interstage deaths. Procedural and interstage deaths were eliminated in the latter half of the experience. Comprehens...Continue Reading

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