Gradually Progressive Dyspnea and Exercise Intolerance in an Otherwise Active Middle-Aged Woman: Why Was the Presentation of Congenital Heart Disease Delayed?

Patients with congenital heart disease (CHD) increasingly are surviving into adulthood. In the United States alone, there are more than one million adult patients living with CHD with the number increasing about 5% each year. With more than 85% of infants with CHD surviving into adulthood with their disease, encounters with these patients in the operating room for cardiac and noncardiac operative procedures is becoming more commonplace. Most of these patients receive corrective surgery early in life, although some may live with uncorrected CHD with no-to-relatively mild symptoms and present at a later time in life with symptoms of heart failure or pulmonary hypertension. The authors present an adult patient with uncorrected CHD presenting with late onset of heart failure symptoms. The authors also review the patient's complex congenital heart lesion, transesophageal echocardiography findings, and intraoperative management.