Background Infants awaiting heart transplantation (HT) face the highest wait-list mortality among all children and adults listed for HT in the United States (US). We sought to determine the risk of death for infants <12 months old while awaiting HT in the current era, and to identify the principle risk factors associated with wait-list mortality. Methods We analyzed outcomes for all US infants listed for HT from January 1999 to July 2006 using data reported to the US Scientific Registry of Transplant Recipients. Results Of 1133 listed infants, 61% were <3 months of age, 80% were listed status 1A, 64% had a congenital heart disease (CHD) and 31% had cardiomyopathy. Of 724 infants with CHD, 25% were on prostaglandin (PG) and 27% had history of prior surgery. By 6 months after listing, 23% died on the wait-list and 54% were transplanted. Multivariable factors associated with wait-list mortality were weight <3 kg (HR 1.4, 95% CI 1.0–1.9), ECMO support (HR 5.6, CI 4.0–7.9), ventilator support (HR 2.1, CI 1.6–2.8), CHD with PG support (HR 2.8, CI 1.8–4.3), CHD without prior surgery (HR 2.8, CI 1.9–3.9) and nonwhite race/ethnicity (HR 1.8, CI 1.4–2.3). Conclusions One in four US infants listed for HT dies before a donor heart can be identified. Wait-list mortality is associated with weight under 3 kilograms, level of invasive support and CHD, but not listing status, which captures medical urgency poorly. Measures to expand infant organ donation, especially among neonates, are urgently needed.

Background— Cardiac rhythm devices are increasingly used in the pediatric population, although their impact on quality of life (QOL) is poorly understood. The purpose of this study was to compare (QOL) scores among pediatric device patients, healthy controls, and congenital heart disease (CHD) patients and determine the key drivers of QOL in pediatric device patients. Methods and Results— Multicenter, cross-sectional study at 8 pediatric centers of subjects aged 8 to 18 years with either a pacemaker or defibrillator was carried out. Patient–parent pairs completed the Pediatric Quality of Life Inventory and Pediatric Cardiac Quality of Life Inventory. QOL outcomes in device patients were compared with healthy controls and patients with various forms of CHD. Structural equation modeling was used to test for differences in Pediatric Cardiac Quality of Life Inventory scores among (1) device type, (2) presence of CHD, and (3) hypothesized key drivers of QOL. One hundred seventy-three patient–parent pairs (40 defibrillators/133 pacemakers) were included. Compared with healthy controls, patients with devices and their parents reported significantly lower Pediatric Quality of Life Inventory scoring. Similarly, compared with patients with mild forms of CHD, parents and patients with devices reported significantly lower Pediatric Cardiac Quality of Life Inventory scores and were similar to patients with more severe CHD. Key drivers of patient QOL were presence of implantable cardioverter-defibrillator and CHD. For patients, self-perception was a key driver of lower QOL, whereas for parents behavioral issues were associated with lower QOL. Conclusions— Patient QOL is significantly affected by the presence of cardiac rhythm devices. Whether these effects can be mitigated through the use of psychotherapy needs to be assessed.

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