Surgery for Adults with Congenital Heart Disease (ACHD)

Stephanie M. Fuller, MD, Joseph A. Dearani, MD, Constantine D Mavroudis, MD, MSc, MTR, Tracy R. Geoffrion, MD, MPH, Elizabeth H. Stephens, MD, PhD, Andrew Goldstone, MD, PhD, Katsuhide Maeda, MD, PhD, Arjun K. Mahendran, MD, Dipankar Gupta, MD, Frederick J. Fricker, MD, Mark S. Bleiweis, MD, Jeffrey P Jacobs, MD

Key Points

The surgical management of adults with congenital heart disease (ACHD) is inherently complex, often involving reoperations and unique anatomic/physiologic challenges. A dedicated multidisciplinary team, including congenital cardiac surgeons, adult congenital cardiologists, adult cardiac specialists, interventionalists, anesthesiologists, and intensivists, can optimize outcomes. Thorough preoperative evaluation is critical. This includes multimodal imaging to delineate anatomy and physiology and assessment of comorbidities that may increase surgical risk. Most ACHD patients present for subsequent multiple reoperations. Surgeons should plan for peripheral cannulation, have blood products readily available, and be prepared for emergent situations such as catastrophic injury upon sternal re-entry. ACHD patients can develop acquired cardiac conditions (coronary artery disease, valve degeneration, and aortic pathology) similar to the general population. Established adult guidelines should inform management, but with special attention to the unique anatomy and prior interventions in ACHD patients. Some patients with ACHD may require heart or multi-organ transplantation, which should be performed in experienced centers.

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