Rescue cardiac transplantation for failing staged palliation in patients with hypoplastic left heart syndrome

Abstract

Cardiac transplantation can offer children with failing staged palliation their only chance of survival. Transplantation, however, carries a high risk in this subgroup, especially in the setting of elevated panel reactive antibody.

Transplantation and Mechanical Circulatory Support in Congenital Heart Disease

“…Akin to other single-ventricle patients for whom palliation fails in the short term, patients with HLHS undergoing rescue transplantation after a failed Norwood procedure experience significantly higher mortality than those undergoing transplantation as a primary treatment strategy (n=8 [50%] versus n=23 [13%], respectively; P=0.053 45 ). A Pediatric Heart Transplant Study registry study supported these findings, showing a 52% postoperative mortality rate in those cases that crossed over from primary transplantation listing to an interim repair, some of which were presumably delayed Norwood repairs.…”

Changing Indications for Pediatric Heart Transplantation

“…These include renal failure, the need for extracorporeal membrane oxygenator support, mechanical ventilation, the presence of anti-HLA antibodies, and infection within 2 weeks of transplantation. 17,19,[78][79][80][81][82] The use of extracorporeal membrane oxygenation before transplantation had a hazard ratio of 2.65, and 1-year posttransplantation survival was 64%. 19 Mortality on the wait list for sensitized patients who express anti-HLA antibodies is reported to range between 19% and 22% because a negative prospective HLA cross-match is often a prerequisite to accepting the donor heart.…”

“…17,18,90 Although results in infants transplanted for unrepaired complex congenital heart disease are similar to those for transplanted infants with dilated cardiomyopathy, 18,91 outcomes of transplantation in young infants as a rescue therapy for failed hypoplastic left heart syndrome are worse compared with those for patients with cardiomyopathy (1-year survival, 70% versus 89%, respectively). 81,91 Older children and young adults with congenital heart disease have additional risk factors affecting their outcome after listing and transplantation compared with children with cardiomyopathy. Patients who were listed for transplantation within 6 months of the Fontan procedure were more likely to die while waiting for a heart than patients who were listed >6 months after the procedure (33% versus 11%, respectively).…”

Chronic Heart Failure in Congenital Heart Disease

“…285 Patients with HLHS transplanted after a failed Norwood procedure may have a higher mortality than recipients who were not palliated. [286][287][288] However, overall survival from listing to posttransplantation was similar to the overall Norwood outcome Use of homografts Requirement for a prospective crossmatch or presence of PRA >25% associated with wait-list time and increased mortality 271,273 Strategies to address sensitization, including need for negative crossmatch, delaying time to transplantation; desensitization strategies may increase risk 263 Previous blood transfusions Presence of donor-specific antibodies increases risk of antibody-mediated rejection and allograft vascular disease [274][275][276][277][278] Pulmonary hypertension 37,270 High left atrial filling pressures, cyanosis, volume overload, high shear force, and abnormal development of the vasculature and lungs of 54% at 5 years reported in a cohort from a similar era. 289 With improved Norwood outcomes, a standard-risk patient with HLHS should undergo SV surgical palliation rather than primary listing for transplantation.…”