Heart failure in adult congenital heart disease: Emerging concepts with a focus on tetralogy of Fallot

Abstract Emerging heart failure (HF) concepts in the growing population of adults with congenital heart disease (ACHD) are reviewed in the following article with a focus on individuals with tetralogy of Fallot (TOF), the largest group of adults with repaired cyanotic congenital heart disease (CHD). In the first section, the changing epidemiology of CHD and HF in ACHD patients is described. We demonstrate the challenges health care providers face when caring for this unique population. Emphasis is placed on the importance and difficulty of identifying patients at risk for HF, of which TOF patients comprise a substantial subset, underscoring the benefits of specialized cardiac care. In the second portion of the article, we review underlying mechanisms of HF in adults with TOF. We elaborate on the wide-ranging etiologies of HF that reflect a confluence of factors related to native anatomic substrate, history of surgical intervention(s), and superimposed hemodynamic and/or ischemic burden to the right and left heart. We describe state-of-the-art imaging concepts as they apply to qualifying and quantifying acquired myocardial and valvular dysfunction in adults with repaired TOF. In the final part of the article, we review the current literature pertaining to the management of adults with repaired TOF. Specifically, we explore medical and surgical issues related to pulmonary valve replacement, arrhythmia management, and transplantation. Finally, we highlight current knowledge gaps and propose future directions of much-needed research that will improve the quality of care for this growing population.