Children with congenital heart disease (CHD) undergoing cardiac surgery during infancy are at high risk for a variety of neurodevelopmental delays affecting behavioural and cognitive abilities. Our knowledge about this problem has increased significantly over the past decade, and the paper by Goldberg et al (10.1542/peds.2019-1010, commentary: 10.1542/peds.2019-1664) recently released in Pediatricsfurther enhances our understanding of the potential neurodevelopmental complications of children with CHD undergoing cardiac surgery.
Goldbert et al were able to collect behavioral, functional, and quality of life data from a cohort of 250 patients enrolled in the “Single Ventricle Reconstruction” (SVR) trial using parent response survey tools. We know from prior studies that complex cardiac anatomy and a complicated clinical course have been found to be associated with worse neurodevelopmental delays in children with CHD.1,2 Hence, by studying the patients in the SVR trial, the group was able to look at a more homogenous cohort of children, all with an underlying diagnosis of hypoplastic left heart syndrome and single ventricle physiology who underwent Stage I palliation with a Norwood and either a modified Blalock-Taussig-Thomas (mBTT) shunt or a Sano shunt. The study found that these patients had lower motor skills scores compared to normative values and additionally, a higher proportion of patients had abnormal functional and behavioural scores. The scores on the quality of life questionnaires administered to parents were also lower in the study cohort compared to a normative sample, with survey responses indicating worse physical functioning, emotional functioning, and self-esteem for patients as well as greater emotional impact on parents of these children with congenital heart disease. On multivariate analysis, a variety of non-modifiable demographic and clinical comorbidity factors (including living at a lower socioeconomic status, being born with a genetic syndrome, being supported with ECMO, having seizures prior to 6years of age, undergoing cardiac catheterization after the Stage II (Glenn) palliation, suffering from a protein losing enteropathy, undergoing G-tube placement, and experiencing clinical complications) were associated with worse functional and quality of life scores compared to those who did not have those factors. The study further highlights the challenges faced by these patients and their families as they live longer and enter school age years. The authors offer some great suggestions in the discussion section regarding how their findings can benefit the primary care clinicians who care for these children with congenital heart disease. The authors of this study also remind all of us about the need to continue to perform ongoing developmental and psychological screening and surveillance as these patients get older.
Looking to the future, this study adds to the growing body of evidence about neurodevelopmental and psychological problems faced by children with congenital heart disease as they continue to survive longer and better through palliative and therapeutic surgical and interventional catheterization procedures. As we plan for resource allocation to developmental paediatrics, and neurodevelopmental and psychological therapy, this study is a reminder that the population needing these specialized resources will only increase. This may help programs better anticipate the need for clinics and resources for this growing population, potentially allowing better preparedness and shorter wait times for developmental clinic appointments. Further, the increasing awareness about the neurodevelopmental problems faced by children with congenital heart disease may help improve comfort of therapists with this complex patient population, as they are seen more routinely in their clinics and the long abbreviations that often define their diagnoses become more commonly encountered and, hopefully, more easily understood. It is exciting to read this study and we look forward to further longer term follow-up data from this cohort of patients as these children grow older.
- International Cardiac Collaborative on Neurodevelopment (ICCON) Investigators. Impact of Operative and Postoperative Factors on Neurodevelopmental Outcomes After Cardiac Operations. Ann Thorac Surg. 2016 Sep;102(3):843-849.
- Gaynor JW, Stopp C, Wypij D et al. International Cardiac Collaborative on Neurodevelopment (ICCON) Investigators. Neurodevelopmental outcomes after cardiac surgery in infancy. Pediatrics. 2015 May;135(5):816-25.