Abstract
Congenital heart disease is the most common congenital defect. During childhood, survival is generally good but, in adulthood, late complications are not uncommon. Abnormal autonomic control in children with congenital heart disease may contribute considerably to the pathophysiology of these long term sequelae. This narrative review of 34 studies aims to summarize current knowledge on function of the autonomic nervous system in children with a congenital heart defect. Large scale studies that measure both branches of the nervous system for prolonged periods of time in well-defined patient cohorts in various phases of childhood and adolescence are currently lacking. Pending such studies, there is not yet a good grasp on the extent and direction of sympathetic and parasympathetic autonomic function in pediatric congenital heart disease. Longitudinal studies in homogenous patient groups linking autonomic nervous system function and clinical outcome are warranted.
Highlights
Congenital heart defects are the most common congenital defects, affecting around 90 per 10,000 newborns [1]
Enhanced sympathetic activity plays a significant role in the progression of heart failure and it is very plausible that it plays a role in the long term sequelae in congenital heart disease, including eventual fibrosis
Significant sex differences were found for all three measures but not in all body postures
Summary
Congenital heart defects are the most common congenital defects, affecting around 90 per 10,000 newborns [1]. Altered autonomic function is found in various patient groups; for example, in cardiac failure [6] and patients after coronary artery bypass [7], decreased heart rate variability (HRV) is found Altered function of this system in cardiac patients usually entails increased sympathetic and decreased parasympathetic activity and is associated with an increased risk of cardiac events and sudden cardiac death in patients with known cardiovascular disease [8,9], and in persons without a history of cardiovascular disease [10]. We will briefly describe the main structure and function of the ANS, ways to measure ANS and healthy maturation of the ANS
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