Managing Heart Failure in Complex Adult Congenital Heart Disease

Abstract

The management of complex congenital heart disease has advanced over the years. While certain complex congenital heart diseases are not compatible with life, palliative surgeries have developed to allow these children to survive into adulthood. Consequently, there are more children surviving into adulthood who have previously undergone complex palliative surgeries and have complex anatomy and physiology. There is a high prevalence of heart failure in adults with complex congenital heart disease due to its progressive nature. Often, anatomical and physiological sequelae of the disease itself or its surgical palliation lead to heart failure signs and symptoms over time. The nature of heart failure in this population is different than that of normal adults, and so management strategies must be adjusted based on the unique anatomy and physiology of these patients. While there are guidelines for monitoring and managing adults with complex congenital heart disease, there are limited data for medical therapies that improve symptom burden and mortality. Once these patients have worsening or decompensated heart failure, they often must be considered for advanced therapies, mechanical circulatory support, and transplant. There is still a need for further research and highly powered trials to elucidate how to optimally treat heart failure in adults with complex congenital heart disease.

Keywords: Fontan; advanced therapies; congenital heart disease; heart failure; heart transplant; single ventricle; systemic right ventricle.