Report from the Japanese Society of Pediatric Cardiology and Cardiac Surgery Research Committee on Cardio-Vascular Function in Adult Patients with Congenital Heart Disease: Treatment Strategy for Hypoplastic Left Heart Syndrome Based on the Cardiovascular Pathophysiology

Abstract

Hypoplastic left heart syndrome has been considered to be one of the most challenging congenital heart diseases with a high mortality. Early postoperative mortality after the first stage intervention has dramatically improved due to advances in perioperative management and surgical technique. Nonetheless, mid- and long-term mortality and morbidity are still suboptimal, and improvement of quality of life including neurodevelopmental outcome remains to be accomplished. This syndrome has both intrinsic and acquired cardiovascular functional abnormalities, constructing a complex pathophysiology that needs to be understood. In this review, we summarize and discuss cardiovascular pathophysiological characteristics inherent to this unique syndrome.