The care of children with single ventricle heart disease has undergone substantial evolution over the past 2 decades, with improved surgical techniques and hemodynamic outcomes. Concurrently, attention is moving beyond immediate postoperative survival, to a greater emphasis on long term neuropsychological functioning, physiologic integrity, and quality of life. Wolfe et al in this volume of The Journal add to our understanding with a report on a large cohort of children seen, on average, about 6 years after Fontan procedure. With a Fontan procedure, there is one ventricle supporting systemic perfusion, while pulmonary blood flow is non pulsatile based upon passive flow from cavae directly to pulmonary arteries. As the authors note, Fontan physiology carries a risk of substantial long-term morbidity, both cardiac and extra-cardiac, including ventricular dysfunction, dysrhythmias, portal hypertension, protein-losing enteropathy, plastic bronchitis, sleep-disordered breathing, and neuropsychological impairments. The authors found that patients scored 0.5-1 standard deviation below population means for neuropsychological testing, with greater impact on specific domains depending on physiological functioning. Sleep-disordered breathing was associated with poorer visual-motor integration, lower arterial oxygen content with poorer executive function, and regurgitation of the atrial-ventricular valve with lower parent-rated adaptive functioning. Findings held when controlling for stroke, SES, and time since the Fontan procedure. Taken together, these findings have several implications. Acknowledging these risk factors, mitigating physiologic derangements to the extent possible, or revising targets such as tolerable oxygen saturation, may be associated with improved outcomes. In addition, early neurodevelopmental intervention may promote better long-term accommodation so that children with single ventricle physiology have the best opportunity to realize their fullest potential. Article page 239 ▸ Relationships between Physiologic and Neuropsychologic Functioning after FontanThe Journal of PediatricsVol. 227PreviewTo investigate potential relationships between neuropsychologic functioning and cardiac, gastroenterologic/hepatologic, and pulmonary complications in the single ventricle heart disease (SVHD) post-Fontan population. Full-Text PDF