Functional Health Status in Adult Survivors of Operative Repair of Tetralogy of Fallot

Study Questions:

What is the long-term functional health status of adults with repaired tetralogy of Fallot?


A single study of 840 patients was performed at a single center. A phone interview was performed, and chart review and review of echo reports was performed when possible. The Short Form-36 (SF-36) instrument was used to assess patient-reported functional status.


The median age at follow-up was 29.5 years (range 18-64.9). Telephone interview, review of echocardiograms, and SF-36 surveys were performed in 706,339, and 396 patients, respectively. Moderate or greater pulmonary valve insufficiency occurred in 54% of subjects, whereas right ventricular outflow tract stenosis was present in one third of patients. Cardiac symptoms were common, occurring in 45% of patients. Specifically, palpitations occurred in 27%, dyspnea in 21%, and chest pain in 17% of patients. Compared with age- and gender-matched normal controls, subjects with tetralogy of Fallot had significantly abnormal functional status in all physical functioning domains of the SF-36. Decreased physical functioning and echocardiographic abnormalities were more common in older patients (p < 0.0001).


The authors concluded that physical limitations are common in adults with repaired tetralogy of Fallot. Efforts to limit ventricular and outflow tract obstruction may result in improved late functional status.


This study investigated the functional status of a large cohort of patients with tetralogy of Fallot followed at a single institution. Although tetralogy of Fallot is associated with quite good long-term survival, patients are at risk for multiple long-term issues, including arrhythmias and right ventricular dilatation and dysfunction. The primary concern raised by this study is that functional status seems to worsen with age out of proportion to what is seen in the general population. Whether changes in the early surgical approach (single-stage surgery without initial systemic to pulmonary shunt and avoidance of right ventriculotomy) will lead to improved long-term outcomes in future patients is unclear. It is also not known as to whether a strategy of earlier pulmonary valve replacement may prevent this progressive decline in functional status.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Valvular Heart Disease, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Interventions and Imaging, Interventions and Structural Heart Disease, Echocardiography/Ultrasound

Keywords: Tetralogy of Fallot, Follow-Up Studies, Dilatation, Constriction, Pathologic, Pulmonary Valve, Dyspnea, Survivors, Chest Pain, Pulmonary Valve Insufficiency, Health Status, Cardiac Surgical Procedures, Heart Ventricles, Echocardiography

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