BACKGROUND The Norwood procedure with a modified Blalock–Taussig (MBT) shunt, the first palliative stage for single-ventricle lesions with systemic outflow obstruction, is associated with high mortality. The right ventricle–pulmonary artery (RVPA) shunt may improve coronary flow but requires a ventriculotomy. We compared the two shunts in infants with hypoplastic heart syndrome or related anomalies. METHODS Infants undergoing the Norwood procedure were randomly assigned to the MBT shunt (275 infants) or the RVPA shunt (274 infants) at 15 North American centers. The primary outcome was death or cardiac transplantation 12 months after randomization. Secondary outcomes included unintended cardiovascular interventions and right ventricular size and function at 14 months and transplantation-free survival until the last subject reached 14 months of age. RESULTS Transplantation-free survival 12 months after randomization was higher with the RVPA shunt than with the MBT shunt (74% vs. 64%, P = 0.01). However, the RVPA shunt group had more unintended interventions (P = 0.003) and complications (P = 0.002). Right ventricular size and function at the age of 14 months and the rate of nonfatal serious adverse events at the age of 12 months were similar in the two groups. Data collected over a mean (±SD) follow-up period of 32±11 months showed a nonsignificant difference in transplantation-free survival between the two groups (P = 0.06). On nonproportional-hazards analysis, the size of the treatment effect differed before and after 12 months (P = 0.02). CONCLUSIONS In children undergoing the Norwood procedure, transplantation-free survival at 12 months was better with the RVPA shunt than with the MBT shunt. After 12 months, available data showed no significant difference in transplantation-free survival between the two groups. (ClinicalTrials.gov number, NCT00115934.)

Background— Recent data suggest that the Berlin Heart EXCOR Pediatric ventricular assist device is superior to extracorporeal membrane oxygenation for bridge to heart transplantation. Published data are limited to 1 in 4 children who received the device as part of the US clinical trial. We analyzed outcomes for all US children who received the EXCOR to characterize device outcomes in an unselected cohort and to identify risk factors for mortality to facilitate patient selection. Methods and Results— This multicenter, prospective cohort study involved all children implanted with the Berlin Heart EXCOR Pediatric ventricular assist device at 47 centers from May 2007 through December 2010. Multiphase nonproportional hazards modeling was used to identify risk factors for early (<2 months) and late mortality. Of 204 children supported with the EXCOR, the median duration of support was 40 days (range, 1–435 days). Survival at 12 months was 75%, including 64% who reached transplantation, 6% who recovered, and 5% who were alive on the device. Multivariable analysis identified lower weight, biventricular assist device support, and elevated bilirubin as risk factors for early mortality and bilirubin extremes and renal dysfunction as risk factors for late mortality. Neurological dysfunction occurred in 29% and was the leading cause of death. Conclusions— Use of the Berlin Heart EXCOR has risen dramatically over the past decade. The EXCOR has emerged as a new treatment standard in the United States for pediatric bridge to transplantation. Three-quarters of children survived to transplantation or recovery; an important fraction experienced neurological dysfunction. Smaller patient size, renal dysfunction, hepatic dysfunction, and biventricular assist device use were associated with mortality, whereas extracorporeal membrane oxygenation before implantation and congenital heart disease were not.

Patients undergoing transplantation for CHD have a good late survival if they survive the early post-operative period. Risk factors for reduced survival are older age at transplant and a previous Fontan operation.

AP. The total cavopulmonary connection resistance: a significant impact on single ventricle hemodynamics at rest and exercise. Am J Physiol Heart Circ Physiol 295: H2427-H2435, 2008. First published October 17, 2008 doi:10.1152/ajpheart.00628.2008Little is known about the impact of the total cavopulmonary connection (TCPC) on resting and exercise hemodynamics in a single ventricle (SV) circulation. The aim of this study was to elucidate this mechanism using a lumped parameter model of the SV circulation. Pulmonary vascular resistance (1.96 Ϯ 0.80 WU) and systemic vascular resistances (18.4 Ϯ 7.2 WU) were obtained from catheterization data on 40 patients with a TCPC. TCPC resistances (0.39 Ϯ 0.26 WU) were established using computational fluid dynamic simulations conducted on anatomically accurate three-dimensional models reconstructed from MRI (n ϭ 16). These parameters were used in a lumped parameter model of the SV circulation to investigate the impact of TCPC resistance on SV hemodynamics under resting and exercise conditions. A biventricular model was used for comparison. For a biventricular circulation, the cardiac output (CO) dependence on TCPC resistance was negligible (sensitivity ϭ Ϫ0.064 l ⅐ min Ϫ1 ⅐ WU Ϫ1 ) but not for the SV circulation (sensitivity ϭ Ϫ0.88 l ⅐ min Ϫ1 ⅐ WU Ϫ1 ). The capacity to increase CO with heart rate was also severely reduced for the SV. At a simulated heart rate of 150 beats/min, the SV patient with the highest resistance (1.08 WU) had a significantly lower increase in CO (20.5%) compared with the SV patient with the lowest resistance (50%) and normal circulation (119%). This was due to the increased afterload (ϩ35%) and decreased preload (Ϫ12%) associated with the SV circulation. In conclusion, TCPC resistance has a significant impact on resting hemodynamics and the exercise capacity of patients with a SV physiology. mathematical modeling; cardiac function; congenital heart defects; magnetic resonance imaging THE FONTAN PROCEDURE, composing of connecting the systemic and pulmonary circulation in series via the total cavopulmonary connection (TCPC), is the primary palliative option for physiological correction of single ventricle (SV) congenital heart diseases (2, 3). Although postoperative survival is excellent, with some centers having a mortality of Ͻ1%, studies have repeatedly demonstrated poor functional outcome in survivors (15-18), including significantly reduced exercise capacity, diminished cardiac output (CO), and risks of heart failure (5, 10 -12, 16, 32, 35, 36, 40). Among all the parameters that come into play, decreased cardiac function in these patients seems to be one of the key players responsible for the poor functional outcome (26, 35). However, the impact of the surgically altered TCPC on cardiac function or functional outcome has not been well understood.Poor cardiac function in SV patients can be associated with many factors. For example, Fogel et al. (8) demonstrated that the presence of the lateral tunnel baffle restricted the motion of the atrioventric...

Closure of ASD with the HELEX septal occluder is safe and effective when compared with surgical repair, with reduced anesthesia time and hospital stay. (U.S. Multicenter Pivotal Study of the HELEX Septal Occluder for Percutaneous Closure of Secundum Atrial Septal Defects; this study was approved by the Food and Drug Administration before the National Institutes of Health website was active, so there is not a URL or registration number.).