Heart Failure in Single Right Ventricle Congenital Heart Disease: Physiologic and Molecular Considerations.

Heart Failure in Single Right Ventricle Congenital Heart Disease: Physiologic and Molecular Considerations. Am J Physiol Heart Circ Physiol. 2020 Feb 28;: Authors: Garcia AM, Beatty JT, Nakano SJ Abstract Due to remarkable surgical and medical advances over the past several decades, there are growing numbers of infants and children living with single ventricle congenital heart disease (SV), where there is only one functional cardiac pumping chamber. Nevertheless, cardiac dysfunction and ultimately heart failure is a common complication in the SV population and pharmacologic heart failure therapies have largely been ineffective in mitigating the need for heart transplantation. Given that there are several inherent risk factors for ventricular dysfunction in the setting of SV in addition to probable differences in molecular adaptations to heart failure between children and adults, it is perhaps not surprising that extrapolated adult heart failure medications have had limited benefit in children with SV heart failure. Further investigations into the molecular mechanisms involved in pediatric SV heart failure may assist with risk stratification as well as development of targeted, efficacious therapies specific to this patient population. In this review, we present a brief overview of SV anatomy and physiology, with a focus on patients with a single morphologic right ventricle requiring staged surgical palliation. Additionally, we discuss...
Source: American Journal of Physiology. Heart and Circulatory Physiology - Category: Physiology Authors: Tags: Am J Physiol Heart Circ Physiol Source Type: research