Joseph A. Dearani, M.D., of the Department of Cardiovascular Surgery at Mayo Clinic discusses results of a study of patients 40 years or older treated for tetralogy of Fallot, one of the most common cyanotic congenital heart lesions. The paper was published in the December 1, 2010 issue of Mayo Clinic Proceedings.
To report the outcomes of patients with tetralogy of Fallot (TOF) undergoing surgical repair at age 40 years or older.
Patients and Methods
We reviewed records of patients (age, ≥40 years) who underwent TOF repair from January 1, 1970, through December 31, 2007. Symptoms, palliative procedures, surgical reports, and long-term outcomes were analyzed.
Fifty-two patients (30 men [58%]) had surgery at a mean ± SD age of 50±8 years; 27 (52%) had prior palliative surgery at a mean ± SD age of 17±11 years. Procedures for TOF repair included pulmonary valve replacement (n=10), transannular patch (n=10), and native pulmonary valve preservation (n=32). The 30-day mortality rate was 6% (stroke, n=2; ventricular fibrillation, n=1). A mean ± SD follow-up of 14.9±9.3 years was feasible in 48 of 49 survivors; improvement in functional class was observed in 42 patients. Reoperation was performed in 7 patients (4 for pulmonary regurgitation). Twenty-nine patients died (mean ± SD age, 65±12 years); causes of death were cardiac (n=7), noncardiac (n=4), and unknown (n=18). Mean ± SD age at death was younger in patients with previous palliation (59±11 years vs 70±12 years; P=.03). The 10-year survival rate was lower than expected compared with an age- and sex-matched population (73% vs 91%; P<.001).
Complete repair of TOF in patients 40 years or older is feasible but carries increased operative risk. Surgical survivors have improvement in functional class; however, survival remains lower than expected. Reduced survival and need for reoperation emphasize the importance of pulmonary valve replacement at the time of initial repair and long-term follow-up.