With complex anatomy, extensive surgical history, and brittle hemodynamics, the adult congenital heart disease (ACHD) patient poses unique challenges in the modern Cardiac Intensive Care Unit (CICU). Due to advancements in surgical technique and pediatric care, 90% of these patients are surviving into adulthood;1 as these patients age, they begin to accrue the negative effects wrought by years of cardiomyopathy and hypoxemic circulation. Generally speaking, roughly one-third of ACHD patients will develop heart failure at some point in their lives, and one-half will develop arrhythmia.2 Additionally, ACHD patients often face unique social and psychological challenges that stem from spending significant portions of their young lives hospitalized, inability to work as adults, and under-insurance. Taken together, these factors lead to significant obstacles for the critically ill ACHD patient to overcome.
Dr. Joel Hardin (Director of the Emory Adult Congenital Heart Center) recently discussed the incoming burden of congenital heart patients in the adult CICU at the 2023 Houston Shock Symposium, citing the approaching “tsunami” of ACHD patients who will be entering adult practice. While each ACHD patient’s unique anatomy calls for a personalized approach, one patient population Dr. Hardin called attention to is those with Tetralogy of Fallot.
Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease, and accordingly patients with ToF are among the most commonly hospitalized ACHD patients. ToF is characterized by anterior malalignment of the outlet aspect of the interventricular septum (leading to the classic constellation of ventricular septal defect, RV outflow obstruction, over-riding aorta, and RV hypertrophy); even after complete repair, these patients are at risk of developing pulmonary valve regurgitation and subsequent right heart failure.3 Additionally, these patients tend to develop arrhythmias in their 20s and 30s (either atrial or ventricular), often in the presence of concurrent structural disease. Thankfully, the advent of transcatheter pulmonary valve replacement is an emerging therapy to address structural pulmonary valve disease in these patients without an additional sternotomy.4
While congenital heart disease in the adult CICU can be intimidating, it is vital to remember that no one is alone when caring for these patients. A team-based approach involving ACHD consultants, cardiothoracic surgery, interventional cardiology, electrophysiology, heart failure, and critical care is essential in caring for these patients, not to mention the support of nursing, social work, physical therapy, and other auxiliary services. Increasingly, adult cardiologists will have the responsibility of helping these patients with congenital heart disease write their history, and the privilege to ensure it is written well.
Dr. Thomas Das is a cardiology fellow at Cleveland Clinic and served as a CardioNerds Conference Scholar for the 2023 Houston Shock Symposium.
- Liu A, Diller GP, Moons P, Daniels CJ, Jenkins KJ, Marelli A. Changing epidemiology of congenital heart disease: effect on outcomes and quality of care in adults. Nat Rev Cardiol. 2023;20(2):126-137. doi:10.1038/s41569-022-00749-y
- Moore JP, Marelli A, Burchill LJ, et al. Management of Heart Failure With Arrhythmia in Adults With Congenital Heart Disease. J Am Coll Cardiol. 2022;80(23):2224-2238. doi:10.1016/j.jacc.2022.09.038
- Lee MGY, Yao JV, Binny S, et al. Long-term outcome of adult survivors of tetralogy of Fallot. Int J Cardiol Congenit Heart Dis. 2021;4:100147. doi:10.1016/j.ijcchd.2021.100147
- Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019;139(14). doi:10.1161/CIR.0000000000000603