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Background: Mechanical circulatory support using ventricular assist devices (VADs) is a life-saving option for children in heart failure refractory to maximal medical management. The aim of this study was to evaluate the efficacy of standard adult VADs in adolescents and children as well as report our early experience with miniaturized VADs in small children., Methods: A 15-year retrospective review was performed on all patients younger than 18 years of age undergoing insertion of a pulsatile VAD at our institution., Results: Eighteen patients underwent VAD placement during the study period. The mean age was 12 (range, 6 months to 18 years), with a mean body surface area of 1.48 (range, 0.25 to 2.3 m2). Diagnoses included dilated cardiomyopathy (n = 15), myocarditis (n = 2), and postcardiotomy ventricular failure (n = 1). Ten children underwent insertion of biventricular VADs, and 8 had implantation of left ventricular VADs. The mean support duration was 57 days (range, 2 to 175 days). Complications included bleeding requiring reoperation (n = 4), stroke (n = 5), and device-related infection (n = 2). Outcomes of VAD support were as follows: VAD explantation in 1 case, death while receiving mechanical support in 3 patients, and successful transplantation in 14 patients (77%). Survival at 6 months after orthotopic heart transplantation was 93% with 1-year and 5-year survival rates of 83%., Conclusions: Currently available VADs are applicable for use as a bridge to orthotopic heart transplantation or in rare instances for myocardial recovery. The increasing accessibility of miniaturized devices allow for long-term support in smaller children and infants while awaiting orthotopic heart transplantation. Although the perioperative morbidity and mortality of VAD placement is not insignificant, survival for those who receive a heart transplant is excellent.

We describe a child who presented with a traumatic aortic transection in the setting of anomalous origin of the right subclavian artery. This abnormal arch anatomy may influence the diagnosis, anatomic location, and technique of surgical repair of the injury.

Background: Graft failure after cardiac transplantation in children can be managed acutely with mechanical support, most commonly extracorporeal membrane oxygenation (ECMO). The purpose of this study was to evaluation the long-term outcome of ECMO support early and late after pediatric cardiac transplantation., Methods: From February 1982 through October 2002, 168 patients underwent isolated cardiac transplantation. Twenty patients (11.9%) required mechanical support early or late after transplantation. Inpatient and outpatient records of these were reviewed., Results: Indication for transplantation was complex congenital heart disease in 12, cardiomyopathy in 7, and graft failure (retransplant) in 1. One patient was also on ECMO preoperatively. Fifteen patients required circulatory support immediately or shortly (less than 6 weeks) after transplantation. The remaining 5 patients were placed on ECMO for ventricular dysfunction late (3 months to 7 years) after transplantation. In the perioperative ECMO group, 8 (53%) were successfully decannulated and subsequently discharged. Three of 5 (60%) patients placed on ECMO late were successfully decannulated, 1 of whom died in hospital 10 days later and 2 of whom are still alive., Conclusions: Mechanical circulatory support using ECMO can be a useful strategy is the management of graft dysfunction after pediatric cardiac transplantation both early and late postoperatively. The mortality rate is acceptable in this very high risk group of patients and long-term outcome is good.

Background: Regional low-flow perfusion has been shown to provide cerebral circulatory support during neonatal aortic arch operations. However, its ability to provide somatic circulatory support remains unknown., Methods: Fifteen neonates undergoing arch reconstruction with regional perfusion were studied. Three techniques were used to assess somatic perfusion: abdominal aortic blood pressure, quadriceps blood flow (near-infrared spectroscopy), and gastric tonometry., Results: Twelve patients required operation for hypoplastic left heart syndrome, and 3 required arch reconstruction with a biventricular repair. There was one death (7%). Abdominal aortic blood pressure was higher (12+/-3 mm Hg versus 0+/-0 mm Hg), and quadriceps blood volumes (5+/-24 versus -17+/-26) and oxygen saturations (57+/-25 versus 33+/-12) were greater during regional perfusion than during deep hypothermic circulatory arrest (p < 0.05). During rewarming, the arterial-gastric mucosal carbon dioxide tension difference was lower after circulatory arrest than after regional perfusion (-3.3+/-0.3 mm Hg versus 7.8+/-7.6 mm Hg, p < 0.05)., Conclusions: Regional low-flow perfusion provides somatic circulatory support during neonatal arch surgical procedures. Support of the subdiaphragmatic viscera should improve the ability of neonates to survive the postoperative period.

Background: Near-infrared spectroscopy is a potential tool for measuring adequacy of cerebral oxygenation during cardiac operations. The cerebral microcirculation is predominantly venous (by volume) and therefore regional cerebral oxygenation measured by near-infrared spectroscopy should reflect jugular bulb venous saturations., Methods: We compared simultaneous regional cerebral oxygenation and jugular bulb venous saturation measurements in 40 children (median age, 4.5 years; range 2 weeks to 14.5 years) in the cardiac catheter laboratory (n = 29) and during cardiac operations (n = 11)., Results: For all patients combined the correlation between regional cerebral oxygenation and jugular bulb venous saturation was 0.69 (p < 0.0001) and was similar for the two groups. For individual children undergoing cardiac operations excellent correlations were obtained (r = 0.78 to 0.96; median, 0.91). However, at low values of jugular bulb venous saturation, regional cerebral oxygenation tended to run high, whereas the converse was true for high values of jugular bulb venous saturation., Conclusions: These findings suggest that near-infrared spectroscopy may be a useful tool for assessing intravascular cerebral oxygenation during pediatric cardiac operations. Prospective studies of neurologic outcome will be required to establish the value of this technique for assessing the adequacy of cerebral protection.

The expected level of systemic arterial saturation may not be present after bidirectional superior cavopulmonary anastomosis in children with complex congenital cardiac anomalies. We present a case of persistent severe cyanosis in a patient with azygos continuation of the inferior vena cava after bidirectional superior cavopulmonary anastomosis (Kawashima procedure) due to an intrahepatic venovenous malformation. Subsequent transcatheter deployment of two Rashkind double umbrella devices into the malformation reduced the shunt and markedly improved systemic arterial saturation.

The early experience (February 1982 to June 1988) with transplantation for the treatment of congenital heart disease at the University of Pittsburgh was disappointing due to an excessively high perioperative mortality. From July 1988 to June 1992, a further 21 children with congenital heart disease underwent orthotopic transplantation. Thirteen had undergone multiple prior palliative procedures (mean, 2.8 per patient). In 12 of these patients, prior procedures involved the pulmonary arteries on one or more occasions. In contrast to our earlier experience, there were no deaths stemming from inadequate surgical reconstruction or pulmonary hypertension. The actuarial survival was 71% at both 1 and 3 years. This did not differ significantly from the survival among 18 patients who underwent transplantation for the management of cardiomyopathy over the same period (1-year and 3-year survival, 83%). The perioperative mortality and short-term survival are now similar for children undergoing transplantation for the treatment of either congenital heart disease or cardiomyopathy. These improved results probably reflect more careful patient selection and an increasing surgical experience with complex reconstructive procedures.

A staged surgical approach was developed for the management of hearts with univentricular atrioventricular connection (double-inlet left ventricle or tricuspid atresia) and discordant ventriculoarterial connection with anatomical substrate for the development of subaortic stenosis. This consisted of initial palliation with pulmonary artery banding, followed by early elective relief of subaortic obstruction using a proximal pulmonary artery to ascending aorta anastomosis in infancy. Pulmonary blood flow was maintained at this time by creating a bidirectional superior cavopulmonary anastomosis. Over an 18-month period, 5 children, including 4 seen in the first week of life with aortic arch obstruction, were palliated with this approach. All patients survived operation and are asymptomatic with transcutaneous oxygen saturations of 80% to 85%. Completion of cavopulmonary repair is planned at 2 years of age. Although some authors have considered pulmonary artery banding contraindicated in these infants, the current staged approach offers an attractive alternative to the construction of a pulmonary artery to aorta anastomosis in the neonatal period.