In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients.

Background-The optimal treatment for dysfunctional right ventricle-to-pulmonary artery (RV-PA) conduits is unknown.Limited follow-up data on stenting of RV-PA conduits have been reported. Methods and Results-Between 1990 and 2004, deployment of balloon-expandable bare stents was attempted in 242 obstructed RV-PA conduits in 221 patients (median age, 6.7 years). Acute hemodynamic changes after stenting included significantly decreased RV systolic pressure (89Ϯ18 to 65Ϯ20 mm Hg, PϽ0.001) and peak RV-PA gradient (59Ϯ19 to 27Ϯ14 mm Hg, PϽ0.001). There were no deaths, and, aside from 5 malpositioned stents requiring surgical removal, there were no serious procedural complications. During follow-up of 4.0Ϯ3.2 years, 9 patients died and 2 underwent heart transplantation, none related to catheterization or stent malfunction. During 155 follow-up catheterizations in 126 patients, the stent was redilated in 83 patients and additional stents were placed in 41. Stent fractures were diagnosed in 56 patients (43%) and associated with stent compression and substernal location but did not cause acute hemodynamic consequences. By Kaplan-Meier analysis, median freedom from conduit surgery after stenting was 2.7 years (3.9 years in patients Ͼ5 years), with younger age, homograft conduit, conduit diameter Յ10 mm, diagnosis other than tetralogy of Fallot, Genesis stent, higher prestent RV:aortic pressure ratio, and stent malposition associated with shorter freedom from surgery. Tricuspid regurgitation and RV function did not change between stent implantation and subsequent surgery. Conclusions-Conduit

Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries.