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Objective: The optimal shunt size for patients who have the Norwood operation with a right ventricle-to-pulmonary artery conduit is controversial. The goal of this study is to compare outcomes of 2 shunt sizes in this population., Methods: Between 2002 and 2010, 75 consecutive patients diagnosed with hypoplastic left heart syndrome and its variants underwent the Norwood procedure with a right ventricle-to-pulmonary artery conduit. The outcomes of 20 neonates weighing <3 kg were analyzed., Results: The cumulative 30-day stage 1 survival [corrected] was 97% (95% confidence interval, 88%-99%) for all patients (73/75), 20 of whom weighed <3 kg. Nine patients had a 6-mm (group 1) and 11 patients had a 5-mm (group 2) right ventricle-to-pulmonary artery conduit. Thirty-day stage 1 survival was 88% (8/9) in group 1 and 90% (10/11) in group 2 (P = .88). The central pulmonary artery confluence size at prebidirectional cavopulmonary shunt catheterization was 4.5 ± 1.2 mm in group 1 and 2.5 ± 1.0 mm in group 2 (P = .009). The mean transpulmonary gradient was higher in group 2 (7.8 ± 3.1 mm Hg vs 4.2 ± 1.9 mm Hg; P = .036). The incidence of pulmonary artery intervention was 16% (1/7) in group 1 and 75% (6/8) in group 2 (P = .030). Survival rate at 6 months was 66% (6/9) in group 1 and 70% (7/10) in group 2 (P = .89)., Conclusions: Use of a 6-mm right ventricle-to-pulmonary artery conduit showed better central pulmonary artery growth and less need for pulmonary artery intervention in the authors' experience., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Low birth weight (LBW) is a risk factor for mortality in neonatal and infant heart surgery. The purpose of this study was to determine the contemporary outcomes and risk factors of cardiac surgery in low weight babies. The records of 75 consecutive infants weighing <2.5 kg having heart surgery were reviewed. The median weight was 2100 g (range 800-2500 g) and median age was 11 days (range 2-86 days). Half (n=38) of the infants were premature. Diagnoses included: arch obstruction (n=14), hypoplastic left heart syndrome (HLHS) (n=12), tetralogy of Fallot (ToF) or pulmonary atresia (PA)/ventricular septal defect (VSD) (n=11), transposition of the great arteries (TGA) (n=7), total anomalous pulmonary venous return (TAPVR) (n=5), and other (n=20). There were two early deaths. Follow-up was available on all infants with a median duration of 1320 days (range 6-3055 days). Cumulative Kaplan-Meier survival at one year was 90% [95% confidence interval (CI), 80-95%] and at five years was 88% (95% CI, 77-94%). Overall mortality amongst patients with genetic/chromosomal abnormalities was higher, 28% vs. 5.4% amongst patients without such abnormalities (P=0.008). Age, prematurity, preoperative mechanical ventilation, prostaglandins, non-cardiac organ dysfunction, extra-cardiac malformations, perioperative extracorporeal membrane oxygenation (ECMO), and type of procedure were not associated with significant differences in mortality. Cardiac surgery in LBW infants can be performed with low early and mid-term mortality. LBW infants with chromosomal/genetic anomalies have a higher risk.

Objectives: Bidirectional cavopulmonary anastomosis has been performed without cardiopulmonary bypass for some single-ventricle heart defects. Limited data are available for the outcomes of off-pump bidirectional cavopulmonary anastomosis in infants with hypoplastic left heart syndrome. The purpose of this study is to determine the early outcomes for stage II palliation of hypoplastic left heart syndrome without cardiopulmonary bypass., Methods: This is a retrospective review of infants having surgical palliation of hypoplastic left heart syndrome from April 2003 to March 2010 at a single institution., Results: Seventy-five infants had a modified Norwood procedure, 65 with a right ventricle-pulmonary artery conduit, 10 with an aortopulmonary shunt, 2 with atrioventricular valve repair, and 3 with extracorporeal life support. Sixty-eight patients had hypoplastic left heart syndrome or one of its variants, and 7 had other single-ventricle lesions. There were 2 stage I deaths. Stage I survival was 97% (95% confidence interval, 88%-99%). Another 5 infants succumbed in the interstage period. Of the 68 stage I and interstage survivors, 61 had bidirectional cavopulmonary anastomoses, 20 without cardiopulmonary bypass. Median age was 6 months (range, 4-13 months), and median weight was 6.1 kg (range, 5.2-9.0 kg). There were no conversions to cardiopulmonary bypass when off-pump bidirectional cavopulmonary anastomosis was attempted. There were no hospital deaths. Median ventilation duration was 10 hours (range, 6-18 hours), and length of stay was 5 days (range, 4-9 days). Follow-up was available on all infants at a median duration of 17 months (range, 3-43 months), with no unplanned reinterventions., Conclusions: Bidirectional cavopulmonary anastomosis without the use of cardiopulmonary bypass can be performed safely and with low mortality for selected infants with hypoplastic left heart syndrome. Midterm to long-term outcomes remain to be determined., (Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Preoperative brain injury, particularly stroke and white matter injury, is common in neonates with congenital heart disease. The objective of this study was to determine the risk of hemorrhage or extension of preoperative brain injury with cardiac surgery., Methods: This dual-center prospective cohort study recruited 92 term neonates, 62 with transposition of the great arteries and 30 with single ventricle physiology, from 2 tertiary referral centers. Neonates underwent brain magnetic resonance imaging scans before and after cardiac surgery., Results: Brain injury was identified in 40 (43%) neonates on the preoperative magnetic resonance imaging scan (median 5 days after birth): stroke in 23, white matter injury in 21, and intraventricular hemorrhage in 7. None of the brain lesions presented clinically with overt signs or seizures. Preoperative brain injury was associated with balloon atrial septostomy (P = .003) and lowest arterial oxygen saturation (P = .007); in a multivariable model, only the effect of balloon atrial septostomy remained significant when adjusting for lowest arterial oxygen saturation. On postoperative magnetic resonance imaging in 78 neonates (median 21 days after birth), none of the preoperative lesions showed evidence of extension or hemorrhagic transformation (0/40 [95% confidence interval: 0%-7%]). The presence of preoperative brain injury was not a significant risk factor for acquiring new injury on postoperative magnetic resonance imaging (P = .8)., Conclusions: Clinically silent brain injuries identified preoperatively in neonates with congenital heart disease, including stroke, have a low risk of progression with surgery and cardiopulmonary bypass and should therefore not delay clinically indicated cardiac surgery. In this multicenter cohort, balloon atrial septostomy remains an important risk factor for preoperative brain injury, particularly stroke., (2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)