title: Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome.
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. 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.
authors.affiliation: The Heart Center, Columbus Children's Hospital, Columbus, OH 43205, USA. galantm@chi.osu.edu
authors.forename: M
authors.initials: M
authors.lastname: Galantowicz
authors.forename: J P
authors.initials: JP
authors.lastname: Cheatham
mesh_terms: D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures; D003131:Combined Modality Therapy / Q000639:trends; D018729:Fontan Procedure; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000601:surgery / Q000628:therapy*; D007231:Infant, Newborn; D012189:Retrospective Studies; D015607:Stents; D016896:Treatment Outcome