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Objectives To measure the health-related quality of life (HRQOL) and functional status of children with cardiomyopathy and to determine whether they are correlated with sociodemographics, cardiac status, and clinical outcomes. Study design Parents of children in the Pediatric Cardiomyopathy Registry completed the Child Health Questionnaire (CHQ; age ≥5 years) and Functional Status II (Revised) (age ≤18 years) instruments. Linear and Cox regressions were used to examine hypothesized associations with HRQOL. Results The 355 children evaluated at ≥5 years (median 8.6 years) had lower functioning (CHQ Physical and Psychosocial Summary Scores 41.7 ± 14.4 and 47.8 ± 10.7) than that of healthy historical controls. The most extreme CHQ domain score, Parental Impact-Emotional, was one SD below normal. Younger age at diagnosis and smaller left ventricular end-diastolic dimension z score were associated independently with better physical functioning in children with dilated cardiomyopathy. Greater income/education correlated with better psychosocial functioning in children with hypertrophic and mixed/other types of cardiomyopathy. In the age ≥5 year cohort, lower scores on both instruments predicted earlier death/transplant and listing for transplant in children with dilated and mixed/other types of cardiomyopathy (P < .001). Across all ages (n = 565), the Functional Status II (Revised) total score was 87.1 ± 16.4, and a lower score was associated with earlier death/transplant for all cardiomyopathies. Conclusions HRQOL and functional status in children with cardiomyopathy is on average impaired relative to healthy children. These impairments are associated with older age at diagnosis, lower socioeconomic status, left ventricular size, and increased risk for death and transplant. Identification of families at risk for functional impairment allows for provision of specialized services early in the course of disease. Trial registration ClinicalTrials.gov: NCT00005391.

Background The use of ventricular assist devices (VADs) in children with heart failure may be of particular benefit to those with accompanying renal failure, as improved renal function is seen in some, but not all recipients. We hypothesized that persistent renal dysfunction at 7 days and/or 1 month after VAD implantation would predict chronic kidney disease (CKD) 1 year after heart transplantation (HT). Methods Linkage analysis of all VAD patients enrolled in both the PEDIMACS and PHTS registries between 2012 and 2016. Persistent acute kidney injury (P‐AKI), defined as a serum creatinine ≥1.5× baseline, was assessed at post‐implant day 7. Estimated glomerular filtration rate (eGFR) was determined at implant, 30 days thereafter, and 12 months post‐HT. Pre‐implant eGFR, eGFR normalization (to ≥90 mL/min/1.73 m2), and P‐AKI were used to predict post‐HT CKD (eGFR <90 mL/min/1.73 m2). Results The mean implant eGFR was 85.4 ± 46.5 mL/min/1.73 m2. P‐AKI was present in 19/188 (10%). Mean eGFR at 1 month post‐VAD implant was 131.1 ± 62.1 mL/min/1.73 m2, significantly increased above baseline (P < 0.001). At 1 year post‐HT (n = 133), 60 (45%) had CKD. Lower pre‐implant eGFR was associated with post‐HT CKD (OR 0.99, CI: 0.97‐0.99, P = 0.005); P‐AKI was not (OR 0.96, CI: 0.3‐3.0, P = 0.9). Failure to normalize renal function 30 days after implant was highly associated with CKD at 1 year post‐transplant (OR 12.5, CI 2.8‐55, P = 0.003). Conclusions Renal function improves after VAD implantation. Lower pre‐implant eGFR and failure to normalize renal function during the support period are risk factors for CKD development after HT.

Heart transplantation (HTx) is a treatment option for end‐stage heart failure in children. HTx is limited by the availability and acceptability of donor hearts. Refusal of donor hearts has been reported to be common with reasons for refusal including preexisting donor characteristics. This review will focus on the impact of donor characteristics and comorbidities on outcomes following pediatric HTx. A literature review was performed to identify articles on donor characteristics and comorbidities and pediatric HTx outcomes. There are many donor characteristics to consider when accepting a donor heart. Weight‐based matching is the most common form of matching in pediatric HTx with a donor‐recipient weight ratio between 0.7 and 3 having limited impact on outcomes. From an age perspective, donors <50 years can be carefully considered, but the impact of ischemic time needs to be understood. To increase the donor pool, with minimal impact on outcomes, ABO‐incompatible donors should be considered in patients that are eligible. Other factors to be considered when accepting an organ is donor comorbidities. Little is known about donor comorbidities in pediatric HTx, with most of the data available focusing on infections. Being aware of the potential infections in the donor, understanding the testing available and risks of transmission, and treatment options for the recipient is essential. There are a number of donor characteristics that potentially impact outcomes following pediatric HTx, but these need to be taken into consideration along with their interactions with recipient factors when interpreting the outcomes following HTx.

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