Cardiac transplantation after the Fontan or Glenn procedure.

OBJECTIVES: The purpose of this study was to review the clinical course and outcome of cardiac transplantation after a failed Glenn or Fontan procedure. BACKGROUND: Late complications of the Glenn or Fontan procedure, including ventricular failure, cyanosis, protein-losing enteropathy, thromboembolism, and dysrhythmias often lead to significant morbidity and mortality. If other therapies are ineffective, cardiac transplantation is the only therapeutic recourse. Transplantation in this unique population presents significant challenges in the operative and perioperative periods. METHODS: The anatomic diagnoses, previous operations, clinical status, and indications for transplantation were characterized in patients transplanted after a Glenn or Fontan procedure. Outcomes after transplantation, including postoperative complications and mortality, were reviewed. Comparisons were made between survivors and nonsurvivors. RESULTS: Primary orthotopic cardiac transplantation was performed in 35 patients (mean age 15.7 +/- 8.5 years) with a mean follow-up of 54 +/- 46 months. A total of 11 patients had undergone a Glenn shunt and 24 patients a Fontan procedure. Indications for transplantation were a combination of causes including ventricular dysfunction, failed Fontan physiology, and/or cyanosis. Ten patients died

Cardiac transplantation in twins with autosomal dominant Emery-Dreifuss muscular dystrophy.

Emery-Dreifuss muscular dystrophy (EDMD) is a neuromuscular disorder resulting in progressive muscle weakness, contractures, and cardiomyopathy. We report successful cardiac transplantation in identical twin brothers with autosomal dominant EDMD, complicated by ventricular arrhythmias and end-stage cardiomyopathy. Early recognition of progressive cardiac disease and subsequent cardiac transplantation are lifesaving in children with EDMD.

Children are not small adults: some differences between pediatric and adult cardiac transplantation.

Cardiac transplantation is a viable therapy for end-stage heart disease in both adults and children. Overall posttransplant survival in the pediatric age group (birth up to 18 years of age) is excellent (greater than 65% at 5 years for all age groups), comparable with the overall survival in the adult transplant recipients. Important differences exist regarding indications, evaluation, surgical technique, and posttransplant management. Indications for transplant in pediatric patients include metabolic and genetic forms of cardiomyopathy and structural congenital heart disease. Evaluation should include a metabolic workup, because potential etiologic factors include mitochondrial disorders, and genetic studies if indicated by phenotypic appearance or family pedigree. Children referred for transplantation with congenital heart disease have often had multiple attempts at palliative surgery, which increase peritransplant surgical risks. Key pediatric issues after transplantation include psychosocial support for the patient and family with regard to school, growth, development, and future expectations.

Lepirudin anticoagulation for heparin-induced thrombocytopenia.

Lepirudin is indicated for anticoagulation in patients with heparin-induced thrombocytopenia (HIT). We describe 2 cases of HIT and thrombosis in children with heart disease, including one that required extracorporeal membrane oxygenation. Lepirudin, dosed in the recommended adult weight--based fashion, was an effective antithrombotic agent in pediatric patients with HIT.