Researchers found numerous inconsistencies concerning the neurodevelopmental assessment of tetralogy of Fallot (TOF) survivors, including those associated with Alagille syndrome (ALGS), as published in Children.

The studies revealed poor outcomes after focusing on isolated neurodevelopmental features, making conclusions for at least 1 of the following aspects: executive function, cognition, adaptive function, speech-language, and motor function.

Kordopati-Zilou and colleagues performed a systematic review that yielded 14 studies that met the eligibility criteria. Among the different published studies retrieved, the population’s selection criteria were not concordant as some included patients with syndromic or genetic features such as ALGS while others excluded them. For example, Gaynor et al assessed preschool TOF survivors and other children with congenital heart defects (CHDs) after surgery and excluded those with recognized genetic syndromes.

Continue Reading

Read more about ALGS comorbidities

All participants achieved mean scores within the average range for all the neurodevelopmental domains tested. No significant differences emerged when comparing the CHDs groups, which was most probably a consequence of eliminating patients with ALGS and other syndromes, therefore underlining the negative impact of genetic factors on neurοdevelopment.

TOF is a CHD that accounts for 7%-10% of all CHD with a prevalence of about 4 per 10,000 live births, with 16% of them being associated with ALGS and other genetic syndromes. Within the present surgical era, infants with TOF undergo complete intracardiac repair early in their lives, with high long-term survival rates, approximately >90% after 25 years of the surgery.

Existing studies for TOF survivors focus on different developmental aspects, using varying evaluation methods and thus making conclusions for either 1 of the 4 aspects of neurodevelopmental. The poor outcomes of these isolated results indicate the need for future research as well as for continuous neuropsychological assessment and close monitoring of children and adolescents with TOF.

“Our recommendation along with [American Heart Association]/[American Academy of Pediatrics] guidelines, is the application of enhanced neurodevelopmental surveillance for children with TOF and the creation of tailored-to-each-patient intervention, aiming to help them reach their fullest potential,” the authors concluded.

“Furthermore, our results suggest that extended research is required for the identification of the exact risk factors and patterns of neurodevelopmental impairments in TOF patients and, therefore, the establishment of prevention and early intervention protocol.”


Kordopati-Zilou K, Sergentanis T, Pervanidou P et al. Neurodevelopmental outcomes in tetralogy of Fallot: a systematic review. Children. Published online February 15, 2022. doi:10.3390/children9020264