Heart failure in congenital heart disease: A review of clinical status and meta-analysis of diagnostic value of serum natriuretic peptides, and medical and device therapies

Traditional description of heart failure (HF) has been within the context of ischemic, hypertensive, valvular and myopathic disease. This description has contributed to the under-appreciation of HF in adult patients with congenital heart disease (CHD), who also manifest all the pathophysiological criteria constituting the clinical syndrome of HF. In adult CHD patients, heterogeneity of residual substrates predisposes them to an early development of HF, presenting a unique challenge in both categorization and protocol management of HF. As such, current diagnosis and treatment using consensus guidelines developed for HF in general remains unclear if they can serve as a meaningful framework for these patients. In this systematic review and meta-analysis, we review current evidence of this rare but life threatening form of HF affecting a large and growing number of adults. The discussion centers on clinical definition, epidemiology, pathophysiology, diagnosis and management with the view of advancing knowledge of the clinical status of HF in CHD.