Cognitive Outcome and Health-Related Quality of Life in School-Aged Children and Young Adults after Heart Transplantation in Early Childhood

Introduction: Pediatric heart transplantation has become a common treatment strategy in the care of children with end-stage heart disease whether it is secondary to congenital heart disease or cardiomyopathy. Survival rate at our center is 80% at 10 and 75% at 15 years. The aims of this study were to examine comprehensively the neurocognitive and behavioral outcomes in long-term survivors.

Methods: Between 1988 and 2006, 93 children underwent heart transplantation in infancy or early childhood (< 4 years). Twenty patients (21.5%) died in between, 5 were lost in follow-up, and 68 patients were recruited for neurocognitive testing. The average age at re-examination was 11.5 years (range: 6–23 years). The re-evaluation included a detailed neurological examination, assessment of the intelligence coefficient (IQ) by means of the culture fair test and assessment of selective attention. Parental questionnaires (Short Form 36 survey and childhood behavior checklist 6–18) were used to measure health-related quality of life (HRQoL) and behavioral problems in this study group.

Results: The mean IQ was 88 (range: 54–115). Forty children (59%) achieve a test result within normal range (IQ ≥ 85). Seven children (10%) had significant neurological deficits (e.g., hemiparesis or tetraparesis). Fine motoric assessment was performed in the younger study group and showed significant lower scores on all motor domains. Attention test revealed clinical relevant results in 15 patients (22%); 35% had behavioral problems in internal and external domains. However, HRQoL was described well in majority of these patients.

Conclusion: Long-term survival and HRQoL are well in these children and young adults. However, neurocognitive deficits are common in this patient group. Multidisciplinary co-operation between health care professionals is mandatory to improve the outcome and the quality of life in these patients.