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OBJECTIVES Paediatric heart transplantation in children who fail multistage palliation for hypoplastic left heart syndrome is associated with challenges related to immune, clinical or anatomic risk factors. We review current outcomes and risk factors for survival following heart transplantation in this challenging patient population. METHODS The United Network for Organ Sharing transplantation database was merged with Paediatric Health Information System database to identify children who received heart transplantation following prior palliation for hypoplastic left heart syndrome. Multivariable Cox analysis of outcomes and factors affecting survival was performed. RESULTS Our cohort included 849 children between 2009 and 2021. The median age was 1044 days (interquartile range 108–3535), and the median weight was 13 kg (interquartile range 7–26). Overall survival at 10 years following heart transplantation was 71%, with most of the death being perioperative. On multivariable analysis, risk factors for survival included Black race (hazard ratio = 1.630, P  = 0.0253), blood type other than B (hazard ratio = 2.564, P  = 0.0052) and male donor gender (hazard ratio = 1.367, P  = 0.0483). Recipient age, the use of ventricular assist device or extracorporeal membrane oxygenation were not significantly associated with survival. Twenty-four patients underwent retransplantation, and 10-year freedom from retransplantation was 98%. Rejection before hospital discharge and within 1 year from transplantation was 20% and 24%, respectively, with infants having lower rejection rates. CONCLUSIONS Compared with existing literature, the number of children with prior hypoplastic left heart syndrome palliation who receive heart transplantation has increased in the current era. Survival following transplantation in this patient population is acceptable. Most of the death is perioperative. Efforts to properly support these patients before transplantation might decrease early mortality and improve overall survival. [ABSTRACT FROM AUTHOR]

Increased survival rates after corrective or palliative surgery for complex congenital heart disease (CHD) in infancy and childhood are now being coupled with increased numbers of patients who survive to adulthood with various residual lesions or sequelae. These patients are likely to deteriorate in cardiac function or end-organ function, eventually requiring lifesaving treatment including heart transplantation. Although early and late outcomes of heart transplantation have been improving for adult survivors of CHD, outcomes and pretransplant management could still be improved. Survivors of Fontan procedures are a vulnerable cohort, particularly when single ventricle physiology fails, mostly with protein-losing enteropathy and hepatic dysfunction. Therefore, we reviewed single-institution and larger database analyses of adults who underwent heart transplantation for CHD, to enable risk stratification by identifying the indications and outcomes. As the results, despite relatively high early mortality, long-term results were encouraging after heart transplantation. However, further investigations are needed to improve the indication criteria for complex CHD, especially for failed Fontan. In addition, the current system of status criteria and donor heart allocation system in heart transplantation should be arranged as suitable for adults with complex CHD. Furthermore, there is a strong need to develop ventricular assist devices as a bridge to transplantation or destination therapy, especially where right-sided circulatory support is needed. [ABSTRACT FROM AUTHOR]

The evolving demographics, outcomes, and anesthetic management of pediatric heart transplant recipients are reviewed. As survival continues to improve, an increasing number of these patients will present to our operating rooms and sedation suites. It is therefore important that all anesthesiologists, not only those specialized in cardiac anesthesia, have a basic understanding of the physiologic changes in the transplanted heart and the anesthetic implications thereof. [ABSTRACT FROM AUTHOR]