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

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. Comprehensive stage 2 included 18 patients. There were four deaths; two patients who died were brought urgently to operation. Transcatheter Fontan completion included 5 patients (not from the initial 29); there were no deaths and all returned home in 24 hours. With this strategy there is one exposure to cardiopulmonary bypass, aortic cross-clamping, and circulatory arrest. There was a significant learning curve, which is highlighted. This initial experience warrants further investigation to determine whether this hybrid strategy will yield better short- and long-term outcomes.