Lesion-specific outcomes in neonates undergoing congenital heart surgery are related predominantly to patient and management factors rather than institution or surgeon experience: A Congenital Heart Surgeons Society Study.

Objective: To identify the role of institution and surgeon factors, including case volume and experience, on survival of neonates with complex congenital heart disease.

Methods: A total of 2421 neonates from 4 groups-transposition of the great arteries (n = 829), pulmonary atresia with intact ventricular septum (n = 408), Norwood (n = 710), and interrupted aortic arch (n = 474)-were prospectively enrolled from Congenital Heart Surgeons Society institutions. Multivariable analysis of risk-adjusted survival was performed for each group, entering each institution or surgeon into the multivariable analysis separately.

Matching procedure to morphology improves outcomes in neonates with tricuspid atresia.

Objective: This study was undertaken to characterize morphologic substrate of tricuspid atresia with ventriculoarterial concordance and discriminate management strategies that lead to successful definitive repair.

Methods: From 1999 to 2004, a total of 150 babies with type I tricuspid atresia were enrolled from first diagnosis at 26 institutions. Antegrade pulmonary blood flow was absent in 19%, restricted in 54%, and unrestricted in 28%.

Does the combination of aprotinin and angiotensin-converting enzyme inhibitor cause renal failure after cardiac surgery?

Background: Aprotinin use in cardiac surgery has been associated with mild elevations in serum creatinine but generally has not been associated with an increase in the risk of acute renal failure. In the presence of angiotensin-converting enzyme (ACE) inhibitors, however, aprotinin may contribute to significant reductions in glomerular perfusion pressure. The purpose of this study was to test the hypothesis that the combination of ACE inhibitors and aprotinin cause renal failure after cardiac surgery.

Interdigitating arch reconstruction eliminates recurrent coarctation after the Norwood procedure.

Background: We sought to determine whether evolving techniques of aortic arch reconstruction used during the Norwood procedure decreased the incidence of postoperative aortic arch obstruction.

Methods: Our technique for aortic arch reconstruction in patients undergoing the Norwood procedure has evolved from using an allograft patch (classic group, n = 26) to primary connection of the pulmonary artery and arch (autologous group, n = 20). More recently, we have used a novel technique involving coarctation excision, an extended end-to-end anastomosis on the back of the arch, and a counterincision on the anterior descending aorta to sew in an allograft patch for total arch reconstruction (interdigitating group, n = 33).

Prevention of early sudden circulatory collapse after the Norwood operation.

Background: After modifications in our perioperative management protocol, we have observed a decrease in sudden circulatory collapse after the Norwood operation. The current study examines early outcomes after the Norwood operation in our unit in an attempt to identify variables that may have altered the risk of unexpected circulatory collapse.

Methods And Results: We studied 105 consecutive neonates who underwent a Norwood operation in our institution.

Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum.

Objective: We sought to define the prevalence of definitive end states and their determinants in children given a diagnosis of pulmonary atresia and intact ventricular septum during the neonatal period.

Methods: Between 1987 and 1997, 408 neonates with pulmonary atresia and intact ventricular septum were entered into a prospective study by 33 institutions. Competing risks analysis was used to demonstrate the prevalence of 6 end states.

Atrioventricular septal defects: effect of bridging leaflet division on early valve function.

Background: Bridging leaflet division may facilitate repair of atrioventricular septal defects (AVSD). However, the consequences of bridging leaflet division on early valve function and mortality are not well defined.

Methods: Records of children undergoing AVSD repair between January 1995 and January 2002 were reviewed.

Late results of palliative atrial switch for transposition, ventricular septal defect, and pulmonary vascular obstructive disease.

Background: Palliative atrial switch (PAS) procedures that reroute pulmonary and systemic venous drainage and leave a ventricular septal defect (VSD) open have been used in the treatment of deeply cyanotic patients who have severe pulmonary vascular obstructive disease (PVOD). Palliative atrial switch is beneficial for patients with transposition of the great arteries or other complex lesions with VSD who show higher arterial oxygen saturation in the pulmonary artery than in the aorta (transposition hemodynamics/unfavorable streaming). We reviewed the early and late results of PAS (Mustard, n = 25; Senning, n = 3) in patients at two institutions.

Quality of life of children after repair of transposition of the great arteries.

Background: We sought to assess quality of life of children with transposition of the great arteries (TGA) enrolled during transition in management strategy from atrial to arterial switch operation.

Methods And Results: Neonates enrolled by the Congenital Heart Surgeons Society in a prospective study of TGA between 1985 and 1989 were eligible. A Child Health Questionnaire was sent for completion by the child between February and June 2000.

Outcomes of 829 neonates with complete transposition of the great arteries 12-17 years after repair.

Objective: Between 1985 and 1989, the surgical management of neonates with complete transposition (TGA) underwent a transition from atrial to arterial repair. We sought to examine the intermediate outcomes and their associated risk factors in neonates repaired during the era of transition.

Patients And Methods: Twenty-four institutions entered 829 neonates age less than 15 days in a prospective study.

Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia.

Objective: This study was undertaken to determine the demographic, anatomic, institutional, and surgical risk factors associated with outcomes after the Norwood operation.

Methods: A total of 710 of 985 neonates with critical aortic stenosis or atresia enrolled in a prospective 29-institution study between 1994 and 2000 underwent the Norwood operation. Admission echocardiograms were independently reviewed for 64% of neonates.

Electrophysiologic surgery in patients with congenital heart disease.

As patients with congenital heart disease increase in number and age, arrhythmia is becoming a more prevalent and important clinical problem. Although catheter-based therapy has revolutionized the management of arrhythmia, there remains an increasing patient population with congenital heart disease presenting for repair or reoperation with associated atrial or ventricular arrhythmias. Arrhythmia ablation may be safely and effectively included as an adjunct to repair of underlying structural cardiac lesions.

Unusual presentations of gastroschisis.

Gastroschisis is a defect of the ventral abdominal wall nearly always located to the right of the umbilicus. A relatively low incidence of associated anomalies exists with the most common being nonduodenal intestinal atresia occurring in 10 per cent of patients. Although multiple hypotheses have been proposed the pathogenesis of gastroschisis is unproven and controversial.