Late Heart Transplantation in Hypoplastic Left Heart Syndrome With Intact Atrial Septum.

This case report describes 2 patients born with hypoplastic left heart syndrome and an intact atrial septum who underwent a strategy of immediate extracorporeal membrane oxygenation and left atrial decompression followed by hybrid Norwood palliation as a bridge to further palliation. Heart transplantation was ultimately performed in these 2 patients with persisting pulmonary vascular resistance abnormalities.

Current Expectations for Cardiac Transplantation in Patients With Congenital Heart Disease.

Congenital heart disease accounts for 40% of pediatric heart transplants and presents unique challenges to the transplant team. Suitability for transplantation is defined in part by degree of sensitization, pulmonary vascular resistance, and hepatic reserves. The incremental transplant risk for patients with congenital heart disease occurs within the first 3 months, after which survival is equivalent to transplantation for cardiomyopathy. Single ventricle with prior palliation, and especially the failing Fontan, carry the highest risk for transplantation and are least amenable to bridging with mechanical circulatory support. More effective bridging to transplant with mechanical circulatory support will require improvements in the adverse event profile of available pumps and the introduction of miniaturized continuous flow technology. The major barriers to routine long-term survival are chronic allograft failure and allograft vasculopathy. Despite these many challenges, continuing improvements in the care of pediatric heart transplant patients have pushed the median posttransplant survival past 15 years for children and to 20 years for infants.

Repair of early posttransplant endomyocardial biopsy-related tricuspid regurgitation in a child.

We describe surgical repair of symptomatic tricuspid valve regurgitation in the early posttransplant period in a small child. The tricuspid valve regurgitation was due to injury to the valve and chordal apparatus during surveillance endomyocardial biopsy. The described surgical technique produced durable improvement in valve function.

Single-center experience with extracorporeal photopheresis in pediatric heart transplantation.

BACKGROUND: The pediatric heart transplant literature contains little information regarding extracorporeal photopheresis (ECP), despite International Society for Heart and Lung Transplantation guidelines recommending it for recurrent/recalcitrant rejection. We report our experience with ECP in pediatric heart transplantation. METHODS: Data were obtained on heart transplant patients who were aged ≤ 18 years at the time of transplantation and received ECP between 1990 and 2012 at our institution. RESULTS: Twenty heart transplant patients underwent 22 courses of ECP. Median ages were 12.7 years (range, 0.3-18.5 years) at transplant and 15.3 years (range, 7.3-31 years) at initial ECP. Median time from transplant to ECP was 1.4 years (range, 0.1-12.6 years). The median ECP duration was 5.8 months (range, 1.9-16.1 months). Indications for ECP included rejection with hemodynamic compromise (HC) in 4 patients, rejection without HC in 12, and prophylaxis in 2. Eleven patients died at a median time of 3.1 years after the start of ECP. Survival after ECP was 84% at 1 year and 53% at 3 years. Eleven patients were considered non-compliant and had a trend toward lower survival of 75% at 1 year and 18% at 3 years (p = 0.06 compared with compliant patients). One patient developed Pneumocystis carinii pneumonia during ECP and post-transplant lymphoproliferative disease 21 months after finishing ECP. No other adverse effects or infectious complications associated with ECP were noted. CONCLUSIONS: This case series represents the largest reported experience with ECP in pediatric heart transplantation. ECP can be safely applied in this patient group. Despite EPC, non-compliant patients showed a trend toward lower survival than compliant patients.

Implantation of the HeartWare HVAD in a child after a recent thromboembolic stroke.

We report the implantation of a HeartWare left ventricular assist device in a child. A 9-year-old girl presented in cardiogenic shock complicated by an embolic stroke. Catheter-based thrombolysis partially reversed the neurologic deficit, but worsening cardiac failure prompted consideration of mechanical circulatory support (MCS). Limited available pericardial space, anticoagulation requirements, and thromboembolic potential favored the use of the HeartWare left ventricular assist device (HeartWare International, Inc, Framingham, MA). After device implantation and intensive physical rehabilitation, the patient underwent successful cardiac transplantation after 60 days of support.

Cardiac transplantation and mechanical support for functional single ventricle.

Cardiac transplantation has played a pivotal role in the therapeutic algorithm for anatomically uncorrectable congenital heart disease, particularly the failing single ventricle. The historical evolution from Kantrowitz to Bailey and beyond challenges the application of this scarce resource to complex cardiac malformations in the presence of physiologic and circulatory failure. While selection of cardiac transplantation as primary therapy for hypoplastic-left heart syndrome is currently rare, the failing single ventricle in various stages of the Fontan pathway is increasingly considered for this therapy. The results of transplantation in this complex situation have progressively improved and now approached the late outcomes for other conditions. Mechanical circulatory support for the failing single ventricle has recently carried infants and children to successful transplant. The development of miniaturized continuous flow pumps offers the hope of major new avenues of successful circulatory support for single ventricle patients.

Comparison of risk factors and outcomes for pediatric patients listed for heart transplantation after bidirectional Glenn and after Fontan: an analysis from the Pediatric Heart Transplant Study.

BACKGROUND: Patients listed for transplant after the bidirectional Glenn (BDG) may have better outcomes than patients listed after Fontan. This study examined and compared outcomes after listing for BDG and Fontan patients. METHODS: All patients listed for transplant after the BDG in the Pediatric Heart Transplant Study between January 1993 and December 2008 were evaluated. Comparisons were made with Fontan patients and with a matched cohort of congenital heart disease patients. Competing outcomes analysis and actuarial survival were evaluated for the study populations, including an examination of various risk factors. RESULTS: Competing outcomes analysis for BDG and Fontan patients after listing were similar. There was no difference in actuarial survival after listing or transplant among the 3 cohorts. Mechanical ventilation, United Network of Organ Sharing status, and age were risk factors for death after listing in BDG and Fontan patients, but ventilation at the time of transplant was significant only for the Fontan patients. Mortality was increased in Fontan patients listed < 6 months after surgery compared with patients listed > 6 months after surgery, but no difference was observed in BDG patients. There was a trend toward improved survival after listing for both populations across 3 eras of the study, but this did not reach statistical significance. CONCLUSION: Outcomes after listing for BDG and Fontan patients are similar. Mechanical ventilation at the time of transplant remains a significant risk factor for death in the Fontan population, as does listing for transplant soon after the Fontan, suggesting that some patients may benefit from transplant instead of Fontan completion.

Coronary artery fistula ligation in a child with use of the Starfish Heart Positioner.

The Starfish Heart Positioner aids off-pump coronary artery surgery in adult patients by providing posterior cardiac exposure without incurring hemodynamic instability. Herein, we describe its use in a 17-month-old girl who had a right coronary artery fistula that drained to the right ventricle. Use of the device enabled exposure that afforded closure of the fistula without cardiopulmonary bypass. The patient was discharged from the hospital 2 days postoperatively. Six weeks later, she was well, and echocardiography showed no residual fistulous flow.

Is the "perfect Fontan" operation routinely achievable in the modern era?

OBJECTIVE: In 1990, Fontan, Kirklin, and colleagues published equations for survival after the so-called "Perfect Fontan" operation. After 1988, we evolved a protocol using an internal or external polytetraflouroethylene tube of 16 to 19 millimetres diameter placed from the inferior caval vein to either the right or left pulmonary artery along with a bidirectional cava-pulmonary connection. The objective of this study was to test the hypothesis that a "perfect" outcome is routinely achievable in the current era when using a standardized surgical procedure. METHODS: Between 1 January, 1988, and 12 December, 2005, 112 patients underwent the Fontan procedure using an internal or external polytetraflouroethylene tube plus a bidirectional cava-pulmonary connection, the latter usually having been constructed as a previous procedure. This constituted 45% of our overall experience in constructing the Fontan circulation between 1988 and 1996, and 96% of the experience between 1996 and 2005. Among all surviving patients, the median follow-up was 7.3 years. We calculated the expected survival for an optimal candidate, given from the initial equations, and compared this to our entire experience in constructing the Fontan circulation. RESULTS: An internal tube was utilized in 61 patients, 97% of whom were operated prior to 1998, and an external tube in 51 patients, the latter accounting for 95% of all operations since 1999. At 1, 5, 10 and 15 years, survival of the entire cohort receiving polytetraflouroethylene tubes is superimposable on the curve calculated for a "perfect" outcome. Freedom from replacement or revision of the tube was 97% at 10 years. CONCLUSION: Using a standardized operative procedure, combining a bidirectional cavopulmonary connection with a polytetraflouroethylene tube placed from the inferior caval vein to the pulmonary arteries for nearly all patients with functionally univentricular hearts, early and late survival within the "perfect" outcome as predicted by the initial equations of Fontan and Kirklin is routinely achievable in the current era. The need for late revision or replacement of the tube is rare.