Heart Failure in adult patients with congenital heart disease

摘要

In Western societies, the most common cause of heart failure (HF) in adults is "acquired", and generally arises secondary to coronary artery disease and/or hypertension. The incidence of HF in adults with congenital heart disease is 1.2/1000 patient years, and is increasing in this young population. The cause and pathophysiology of HF in adults with congenital heart disease is extremely complex and can affect both systolic and diastolic function, the left and/or right ventricle or a single ventricle. The complex anatomy makes the pathophysiology non superimposable on that of acquired HF. The definition of HF in ACHD by itself is not obvious and does not follow the same rules as those of acquired HF, making its diagnosis and long-term prognostic evaluation difficult. Similarly, the proven efficiency of treatments in acquired HF is no longer evident in adults with congenital heart disease; this is especially true for preventive treatment in chronic HF. A multidisciplinary approach is essential, involving HF specialists, cardiologists specializing in congenital heart disease, cardiac surgeons, anaesthetists, specialists in transplantation, and psychologists. In this review, we address the types of patients with ACHD who develop HF, the pathophysiological mechanisms involved, the diagnostic methods used, and the therapeutic options available.