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In this large paediatric cohort of SCN5A mutation-positive subjects, cardiac conduction disorders were the most prevalent phenotype; CEs occurred in about one-third of genotype-positive children, and several independent risk factors were identified, including age ≤1 year at diagnosis, compound mutation, and mutation with both gain- and loss-of-function.

Purpose of review To provide an international perspective and current review of pediatric heart transplantation (PHTx). Recent findings Waitlist survival and long-term outcomes in PHTx continue to improve. Strategies to maximize donor pool utilization include ABO incompatible listing for infants and expanded donor-to-recipient weight ranges. However, there is a high degree of practice variation internationally, from listing strategies and donor acceptance practices to chronic immunosuppression regimens, long-term graft surveillance, and consideration for retransplantation. Summary Common indications for PHTx include end-stage congenital heart disease and cardiomyopathy. Current median graft survival among PHTx recipients ranges from 13 to 22 years. Common morbidities include infection, rejection, renal dysfunction, coronary allograft vasculopathy, and posttransplant lymphoproliferative disease. International registry data, collaborative initiatives to standardize management, and multicenter studies continue to improve knowledge and advancement of the field.

There is considerable variability in donor acceptance practices among adult heart transplant providers; however, pediatric data are lacking. The aim of this study was to assess donor acceptance practices among pediatric heart transplant professionals. The authors generated a survey to investigate clinicians’ donor acceptance practices. This survey was distributed to all members of the ISHLT Pediatric Council in April 2018. A total of 130 providers responded from 17 different countries. There was a wide range of acceptable criteria for potential donors. These included optimal donor‐to‐recipient weight ratio (lower limit: 50%‐150%, upper limit: 120%‐350%), maximum donor age (25‐75 years), and minimum acceptable left ventricular EF (30%‐60%). Non‐US centers demonstrated less restrictive donor selection criteria and were willing to accept older donors (50 vs 35 years, P < 0.001), greater size discrepancy (upper limit weight ratio 250% vs 200%, P = 0.009), and donors with a lower EF (45% vs 50%, P < 0.001). Recipient factors were most influential in the decision to accept marginal donors including recipients requiring ECMO support, ventilator support, and highly sensitized patients with a negative XM. However, programmatic factors impacted the decision to decline marginal donors including recent programmatic mortalities and concerns for programmatic restrictions from regulatory bodies. There is significant variation in donor acceptance practices among pediatric heart transplant professionals. Standardization of donor acceptance practices through the development of a consensus statement may help to improve donor utilization and reduce waitlist mortality.