How are patients faring 30 years after radical tetralogy of Fallot repair?

Abstract

Out of 283 patients with tetralogy of Fallot (TOF) undergoing its radical repair in a Prague-based Pediatric Heart Center between 1978 and 1988, then with a mean age of 7 years and 7 months, we were successful of getting information about 161, i.e., two thirds of those alive. There are 80 patients lost to follow-up (28% of those operated). A total of 42 patients (14.8%) died during follow-up, with early postoperative mortality being 7.4% (21 patients) and 21 late deaths. Long-term survival rates at 26 years after radical TOF repair were 85% for all patients and 92% for pediatric patients discharged to receive home care after the procedure. Overall, 57 re-do surgery procedures were performed in 50 patients. Mortality in 26 late reoperations performed at the Prague-based Na Homolce Hospital in adult patients was zero. Reoperation has to be undertaken earlier and significantly more often (32%) in patients having pulmonary annulus incision (using a transannular patch or monocuspid heart valve) as compared with patients receiving a subpulmonary Dacron patch (p < 0.001). Among 105 patients, 52% had university or secondary school level of education. The number of Induviduals with full disability pensions was significantly higher in the group with elementary education only (47%) as compared with university graduates (0%; p= 0.004), secondary school graduates (3.4%; p= 0.0077) and those receiving apprenticeship training (10%; p= 0.0446). Congenital heart defects were present in 6.3% of offspring of our patients. Residual prognostically relevant findings are often made in TOF. All patients Including those with successful TOF repair in childhood require permanent follow-up by a cardiologist. Patients at risk of significant residual findings should be assessed in the Adult Center for Congestive Heart Defects regarding the need for and timing of reoperation. The outcome of early and correctly scheduled reoperation is favorable.