Chronic Heart Failure in Congenital Heart Disease

770 Part I: General Considerations Introduction The past 60 years have brought remarkable advancements in the diagnosis and treatment of congenital heart disease (CHD). Early diagnosis and improvements in cardiac surgery and interventional cardiology have resulted in unprecedented survival of patients with CHD, even those with the most complex lesions. Despite remarkable success in treatments, many interventions are palliative rather than curative, and patients often develop cardiac complications, including heart failure (HF). HF management in the setting of CHD is challenged by the wide range of ages at which HF occurs, the heterogeneity of the underlying anatomy and surgical repairs, the wide spectrum of HF causes, the lack of validated biomarkers for disease progression, the lack of reliable risk predictors or surrogate end points, and the paucity of evidence demonstrating treatment efficacy. The purposes of this statement are to review the literature pertaining to chronic HF in CHD and to elucidate important gaps in our knowledge, emphasizing the need for specific studies of HF mechanisms and improving outcomes for those with HF. In this document, the definition of CHD severity is the definition common in CHD documents, including the American College of Cardiology (ACC)/American Heart Association (AHA) guidelines for the management of adults with CHD (Table 1). The definition of HF corresponds to that found in the multiple guidelines on diagnosis and management of HF. Although nuances and specific details may be controversial, the broad definition from the Heart Failure Society of America guidelines states the following: “In physiologic terms, HF is a syndrome characterized by either or both pulmonary and systemic venous congestion and/or inadequate peripheral oxygen delivery, at rest or during stress, caused by cardiac dysfunction.” The definition of chronic HF in this document concurs with that of the European Society of Cardiology guidelines, which emphasize chronic HF (whether stable, progressively worsening, or decompensated) rather than acute HF. Although specific definitions of acute and chronic HF are not universally accepted, we focus here on chronic HF as a persistent syndrome that requires consideration of therapy to prevent progression, decompensation, or death. This document focuses on the mechanisms and treatment of myocardial dysfunction while recognizing that HF symptoms may be attributable to underlying hemodynamic abnormalities such as valve dysfunction, outflow obstruction, coronary abnormalities, or residual shunting. Therefore, all patients with CHD with HF symptoms should undergo a detailed hemodynamic assessment by CHD-experienced cardiologists for any reversible hemodynamic abnormalities and receive appropriately targeted interventions if possible. Treatment recommendations for HF caused by valve