Abstract
Children, adolescents and adults living with Fontan circulation face numerous neurological and developmental challenges. As the population with complex CHD increases thanks to outstanding improvement in medical and surgical care, the long-term developmental and mental health sequelae have become a public health priority in pediatric and congenital cardiology. Many patients with a Fontan circulation experience difficulty in areas of cognition related to attention and executive functioning, visual spatial reasoning and psychosocial development. They are also at high risk for mental health morbidities, particularly anxiety disorders and depression. Several hemodynamic risk factors, beginning during the fetal period, may influence outcomes and yield to abnormal brain growth and development. Brain injury such as white matter lesions, stroke or hemorrhage can occur before, during, or after surgery. Other sociodemographic and surgical risk factors such as multiple catheterizations and surgeries and prolonged hospital stay play a detrimental role in patients' neurodevelopmental prognosis. Prevention and intervention to optimize long-term outcomes are critical in the care of this vulnerable population with complex CHD.
Highlights
Remarkable advances in pediatric cardiac surgical and medical care in the last few decades have substantially improved survival rates of patients with the most complex forms of congenital heart disease (CHD)
Children in this cohort have lower scores on developmental assessments at 14 months and at three years of age. [5, 14] Interestingly, patient specific factors and measures of perioperative morbidities, but not treatment strategies, were found to be predictive of lower developmental scores. [5, 13, 14] Long-term follow-up of the cohort enrolled in the Single Ventricle Reconstruction Trial will likely provide further insights on factors associated with impaired neurodevelopmental outcomes for this high-risk patient group
Patients with single ventricle are at high risk for neurologic, developmental, and psychosocial sequelae
Summary
Remarkable advances in pediatric cardiac surgical and medical care in the last few decades have substantially improved survival rates of patients with the most complex forms of congenital heart disease (CHD). Along with excellent survival and improved short-term outcomes has come recognition of long-term neurodevelopmental and psychosocial morbidities [1–4]. Individuals with complex CHD, including those with a Fontan circulation, have an elevated risk for structural brain abnormalities and neuropsychological and mental health disorders, starting early in life and continuing throughout adulthood [5, 6]. The etiologies of neurological vulnerability and injury are multifactorial, additive, and interactive. For complex CHD, such as single ventricle anatomies, genetic factors influencing heart formation may affect brain development. Whole exome sequencing of CHD parentoffspring trios revealed substantial overlap between damaging de novo mutations in children with
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