Surgical correction of congenital heart disease in the adult: experience with 139 patients.

Patients over 18 years of age who have undergone a surgical correction of a congenital cardiac malformation during the period 1968 through 1977 have been reviewed. Those patients with calcific aortic stenosis which was thought, but not proved, to have arisen in a congenitally malformed aortic valve, were excluded from the review. Patients with cystic medial necrosis of the aorta were similarly excluded. There were 139 patients in the series (age range from 18 years to 67 years). The most common defects were those involving the intra-atrial septum and the related great veins, 50%. Abnormalities involving the great arteries including patent ductus arteriosus and coarctation of the aorta accounted for 19%. Common defects of conal development including ventricular septal defects and Tetralogy of Fallot malformations accounted for 15%. Valvular abnormalities including pulmonic stenosis, aortic valve abnormalities and Ebstein's malformation of tricuspid valve accounted for 11.5%. Complex congenital malformations were relatively uncommon, 4%. There were two patients with a combination of acquired and congenital heart disease. There were two operative deaths in the series, both occurring in patients with complex forms of congenital heart disease (multiple ventricular septal defects, double outlet right ventricle). There were two additional postoperative hospital deaths, one occurring following repair of an atrial septal defect from massive pulmonary embolus, and another occurring six weeks following a Fontan procedure performed for tricuspid atresia. Thus, the hospital mortality for the series was 2.9%. This reviewed series reveals the incidence of operable congenital heart defects appearing in an adult cardiac surgical practice and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.