Complex congenital heart disease is the cause for 1.5%-2.4% of heart transplantations in adults. Indications for heart transplantation are ventricular dysfunction, failed Fontan physiology, and cyanosis. We present a 43-year-old man, 20 years after a Fontan operation by an univentricular heart, with a tricuspid atresia, L-Transposition of the Great Arteries, and high-grade subpulmonary stenosis. Persistent bleeding after successful transplantation necessitated major blood transfusions and reoperation. We reconstructed superior vena cava and vena brachiocephalica using a pericardial patch, and because of postoperative low output syndrome, he was connected to extracorporeal circulation through cannulation of femoral artery and explanted after hemodynamic stability. Heart transplantation can be successful in patients with end-stage congenital heart diseases after Fontan procedure, because of improved surgical and medical management. Challenges include limited ability to assess hemodynamics, complex anatomy, increased risk of bleeding caused by multiple prior procedures, and unique underlying pathologic states. Careful patient selection and surgical timing are essential to increase long-term survival.

The purpose of this study was to review the outcome of pediatric heart recipients with uncorrectable congenital heart disease, failed corrective procedures, or intractable acquired cardiomyopathy. Between 1988 and 2005, cardiac transplantations were performed in 128 pediatric patients (59 girls and 69 boys) at the Heart-Center North-Rhine-Westphalia. Their ages varied between 1 week and 18 years (mean, 7.6 +/- 6.2 years). Underlying diseases were dilative cardiomyopathy in 93 cases and uncorrectable congenital heart disease in 35 cases. We diagnosed and observed 130 episodes of acute rejection in 85 patients (65.3% of patients had an acute rejection during the observation period); 71 patients were treated efficiently with steroid-pulse therapy. Monoclonal antibody OKT3 was administrated in 14 patients because of steroid-resistant acute donor organ rejection. Five of those 14 patients survived. The cumulative survival was 88% after 1 year and 68% after 10 years for all patients. Orthotopic heart transplantation is the ultimate treatment option for children with end-stage heart disease and shows an acceptable perioperative mortality rate as well as good long-term results.

Supravalvular aortic stenosis is a rare congenital cardiac anomaly occurring mainly as a part of Williams-Beuren syndrome. Aortic narrowing above the level of the aortic valve causes obstruction of the left ventricular outflow tract, and a pressure gradient between the left ventricle and the aorta causes left ventricle hypertrophy. We report here a case of a 22-year-old man who underwent extended patch aortoplasty because of supravalvular aortic stenosis accompanying Williams-Beuren syndrome. He was in New York Heart Association functional class III with localized hourglass type supravalvular aortic stenosis. Related to arterial hypertension he was in a cardiac decompensation. Mean pressure gradient was 73 mm Hg and maximum gradient 104 mm Hg. Electrocardiography indicated left ventricle hypertrophy, which was also seen in x-ray, as heart enlargement. We successfully treated this patient with extended patch aortoplasty and immediate postoperative echocardiography showed reduction of gradient. Good surgical outcome of congenital supravalvular aortic stenosis in adults can be achieved with this treatment. This technique provides symmetric reconstruction of the aorta with good postoperative results and no gradient across aortic valve and aortic valve insufficiency remains, providing excellent long-term relief of localized supravalvular gradients and preservation of aortic valve competence.

Heart transplantation (HTx) is an ultimate treatment for children with end-stage heart failure or inoperable congenital heart disease. The supply of hearts is inadequate; therefore, different mechanical support systems must be used as bridge to HTx in pediatric patients with postoperative low output. The use of ventricular assist devices (VADs) as bridge to HTx in children is limited because of size differences. The purpose of this study was to evaluate the overall long-term outcome of pediatric circulatory support before pediatric HTx. From 1989 through 2004, 91 pediatric patients underwent isolated HTx. Seven of them required mechanical support before transplantation. We reviewed retrospectively the course of 91 children (mean age 14.7 years) who underwent HTx. Group A consisted of elective HTx patients who were treated as outpatients before HTx, whereas group B was the VAD-HTx bridging group (n=7; mean age 12.31 +/- 2.8 years). Mean duration of VAD support was 108 +/- 98 days (minimum 1 day, maximum 258 days). Overall survival rate after HTx was 80% at 1 year without significant differences between groups. Five of seven patients survived and could be discharged after successful HTx, for a survival rate of 77%. The mean follow-up period was 16.76 +/- 10.6 months. No differences in posttransplantation long-term survival and rejection episodes occurred between patients transplanted with or without VAD. VAD therapy can keep pediatric patients with end-stage heart failure alive until successful HTx, and bridge to HTx is a safe procedure in pediatric patients. After HTx, survival rates of these children are similar to those of patients awaiting elective HTx.