Indications for Heart Transplantation in Pediatric Heart Disease

Abstract

Background— Since the initial utilization of heart transplantation as therapy for end-stage pediatric heart disease, improvements have occurred in outcomes with heart transplantation and surgical therapies for congenital heart disease along with the application of medical therapies to pediatric heart failure that have improved outcomes in adults. These events justify a reevaluation of the indications for heart transplantation in congenital heart disease and other causes of pediatric heart failure. Methods and Results— A working group was commissioned to review accumulated experience with pediatric heart transplantation and its use in patients with unrepaired and/or previously repaired or palliated congenital heart disease (children and adults), in patients with pediatric cardiomyopathies, and in pediatric patients with prior heart transplantation. Evidence-based guidelines for the indications for heart transplantation or retransplantation for these conditions were developed. Conclusions— This evaluation has led to the development and refinement of indications for heart transplantation for patients with congenital heart disease and pediatric cardiomyopathies in addition to indications for pediatric heart retransplantation.

Elevated pre-transplant pulmonary vascular resistance is not associated with mortality in children without congenital heart disease: A multicenter study

“…It does not appear that the larger volume centers, which may care for larger numbers of patients with elevated PVRI, are the only centers willing to provide transplant for these historically high-risk patients. This suggests that the pediatric heart transplant community at large may not perceive elevated PVRI as a risk factor in the same way that adult programs have; an idea supported in the 2007 AHA scientific statement on Pediatric Heart Transplantation (9).…”

Transplantation and Mechanical Circulatory Support in Congenital Heart Disease

“…24 They include chronic dependence on inotropic therapy, mechanical ventilation, or MCS; malignant arrhythmias unresponsive to therapy; severe HF symptoms despite optimal medical/surgical therapy; and growth failure or unacceptable quality of life attributable to heart disease. A patient with active HF who resides at home without the need for continuous support also meets the indication if over time the patient has not improved to the point of qualifying for additional palliative/definitive surgery that will improve the quality of life or offer a survival advantage that is sustainable.…”

Risk Stratification and Transplant Benefit in Children Listed for Heart Transplant in the United States

“…1,2 In theory, HT listing is offered when HT is expected to result in a meaningful survival advantage compared with conventional medical or surgical therapy. 3,4 However, an accepted method to quantify survival benefit from HT compared with not pursuing transplant that may also be used to compare listed candidates for this benefit has not been described. Although it may be assumed that the sicker HT candidate is more likely to benefit from transplant, the actual relationship between heart failure severity and transplant benefit is likely to be complex and is currently unknown.…”