Congenital Heart Disease in the Adult: A Review with Internet-Accessible Transesophageal Echocardiographic Images

Abstract

The number of adults recognized with congenital heart disease (CHD) has increased dramatically over the past five decades because of significant advances in diagnosis and medical and surgical care. At the moment, the population of adults with CHD (ACHD) in the United States is estimated at approximately one million (1). For the first time, the number of adults with congenital cardiovascular malformations equals the number of children with these disorders. With additional refinements in surgical techniques and definitive repair at an earlier age, this patient group is likely to increase even further. Survival rates in CHD are influenced by many factors, including year of birth, age at diagnosis, complexity of the pathology, and whether the lesion(s) has been palliated or surgically corrected (Table 1) (1). As survival and life expectancy continue to improve, a growing number of unoperated, palliated, and “repaired” individuals require surgical interventions or other procedures related or unrelated to their heart disease. The care of these patients is becoming more frequent in all surgical settings, including tertiary care facilities, ambulatory centers, and labor and delivery suites.Table 1: Survival Rate from Year of Birth (1940–2000) by Complexity of Congenital Heart DiseaseAdults with CHD may come to the attention of anesthesiologists for various indications including: Cardiac surgery for the first time (for either palliation or definitive surgery) Cardiac reoperation for further palliation or definitive correction after palliative surgery Cardiac surgery for management of residua, complications of prior intervention, or conversion of a priori repair to a modern, potentially more favorable, strategy Noncardiac surgery or other nonsurgical procedures in the presence of uncorrected, palliated, or corrected lesions. Anesthesia and surgery may carry an increased risk for adverse events during emergent or elective procedures in these patients. This is particularly the case in those with cyanosis, pulmonary hypertension, rhythm disturbances, and significant hemodynamic abnormalities. Recommendations from organizations such as the American College of Cardiology (1) and the Canadian Cardiovascular Society (2–4) suggest that these patients should be cared for by cardiac anesthesiologists who have specialized training or extensive experience in the field. However, anesthesia care providers with such advanced expertise may not always be available. The challenge in caring for these patients is further magnified by the fact that there is a heterogeneous population. Individuals may present at any time with a bewildering array of structural variations, each with specific physiologic perturbations and hemodynamic consequences, and situations that require sophisticated perioperative care. The spectrum of CHD ranges widely from relatively mild defects seen in isolation to lesions of moderate to severe complexity typically characterized by several coexistent malformations. An important objective in caring for ACHD is to diminish cardiac-related morbidity and avoid adverse perioperative events. Of utmost importance in this mission is having a basic understanding of the native anatomy, physiology, surgical strategies, and late outcome of the defect under consideration. The primary goal of this article is to present a general overview of the most common congenital cardiovascular defects as applied to the adult age group, with a focus on anatomy, physiology, and long-term outcome (Table 2). To facilitate this review, representative images of the various congenital pathologies, as displayed by transesophageal echocardiography (TEE), accompany this contribution. The graphics are accessible as digital clips on the Web site of Anesthesia & Analgesia (www.anesthesia-analgesia.org), and we hope the clips will serve as reference material for those involved in the care of these patients. The images are labeled according to the American Society of Echocard