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.
