Correction of Tetralogy of Fallot and of Pulmonary Atresia with Ventricular Septal Defect in Adults

Background Early correction is regarded as the treatment of choice for patients with tetralogy of Fallot or pulmonary atresia with ventricular septal defect. Nevertheless, some of these patients reach adulthood without early correction. This study sought to assess risk factors for operative mortality and determine the benefit of total correction in adolescent and adult patients. Methods A retrospective analysis of 52 patients (>16 years at the time of corrective surgery) for tetralogy of Fallot (n = 42) or pulmonary atresia with ventricular septal defect (n = 10) between 1974 and 2003 was performed. Results Age at correction was 28.9 ± 9.9 (16 to 57 years). There were 8 early deaths (15.4%). Preoperative hemoglobin concentration (p = 0.002) and reconstruction of the right ventricular outflow tract with a patch (p = 0.002) were correlated with a significantly higher early mortality. Mean follow-up time was 12.3 ± 10.4 years. Late deaths (n = 6; 11.5%) were cardiac-related in 2 of 6 cases. At follow-up, 28 patients (87.5%) were assigned to the New York Heart Association functional class I. Twenty-four patients led a normal life with full-time work, 6 patients were able to do part-time work, and only 2 patients experienced noticeable limitation on activities. Conclusions Repair of tetralogy of Fallot and of pulmonary atresia with ventricular septal defect in this patient group is associated with a high early mortality. Preoperative chronic cyanosis, expressed by elevated hemoglobin concentration, is predictive for early mortality. Because cyanosis has been shown to lead to multiorgan dysfunction, we conclude that preoperative multiorgan dysfunction may be the intrinsic risk factor for perioperative mortality. Surgical correction in this patient group should still be recommended because the functional status considerably improves.