Heart Failure with Reduced and Preserved Ejection Fraction in Adult Congenital Heart Disease

Heart failure with reduced ejection fraction (HFrEF) is common in patients with adult congenital heart disease. Many of the most common congenital defects have a high prevalence of HFrEF, including left-sided obstructive lesions (aortic stenosis, coarctation of the aorta, Shone complex), tetralogy of Fallot, Ebstein anomaly, lesions in which there is a systemic right ventricle, and lesions palliated with a Fontan circulation. However, heart failure with preserved ejection fraction (HFpEF) is also prevalent in all these lesions. Comprehensive evaluation includes physical exam, biomarkers, echocardiography and advanced imaging, exercise stress testing, and, in some cases, invasive hemodynamics. Guideline-directed medical therapy for HFrEF can be applied to left-sided lesions and may be considered on an individual basis for systemic right ventricle and single-ventricle patients. Medical therapy is limited for HFpEF. However, in both HFrEF and HFpEF, ventricular dyssynchrony and arrhythmias play an important role, and medications for rhythm control, ablation, and cardiac resynchronization therapy should be considered. Finally, aggressive management of cardiovascular risk factors and comorbidities, including, but not limited to, hypertension, obesity, diabetes, dyslipidemia, and obstructive sleep apnea, cannot be overemphasized.