Successful outpatient cardiac rehabilitation in an adult patient post-surgical repair for tricuspid valve atresia and hypoplastic right ventricle: a case study.

CLINICAL CASE: This case report documents outpatient cardiac rehabilitation (CR) in a 28-year-old woman born with transposition of the great vessels, tricuspid atresia, hypoplastic right ventricle, and an atrial septal defect. Surgical procedures were performed during childhood to correct these defects. In 2006, she underwent the following procedures: Fontan revision with a graft to an extracardiac total cavopulmonary connection; ASD creation; right atrial reduction; bidirectional Glenn shunt; right atrial and modified left atrial Maze procedures; and placement of an epicardial dual-chamber anti-tachycardia pacemaker. The patient was referred to CR because of postoperative complaints of fatigue, dyspnea on exertion, and low exercise tolerance. At intake, she underwent a cardiopulmonary stress test, measurement of percentage body fat, and completed the Medical Outcomes Survey 36-Item Short-Form Health Survey, Diet Intake Survey, and Center for Epidemiologic Studies Depression Scale. After completing 36 sessions, all outcomes demonstrated improvement with the exception of percentage dietary fat intake.

DISCUSSION: Despite increasing numbers of patients with congenital heart disease (CHD) surviving into adulthood, exercise prescription in this population remains poorly delineated. In this case, possible physiologic limitations to exercise included diminished cardiac output secondary to low pressures and flow rates in the pulmonary arteries and veins because of the absence of a functioning right ventricle, limited chronotropic response, and severe deconditioning.

SUMMARY: This case presents an adult patient who underwent surgical procedures to avoid heart transplantation. Despite severe CHD with many surgical procedures, and what is effectively a 2-chambered heart, she was able to successfully complete CR, graduate to home exercise, return to independent home living, and pursue her master's degree.