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Advances in surgical techniques have greatly altered the prognosis for children who have heart defects. Thirty or forty years ago, many children with such defects did not live to adulthood. Now, most undergo surgical repair during childhood and do reach adulthood. This study examined the 25-year mortality for persons who had surgical repair of a heart defect in childhood. Data were obtained for individuals who underwent reparative surgery for one of eight congenital heart defects before the age of 18 years between 1958 and 1989 in Oregon. A total of 2,701 subjects underwent such surgery during this time period; the results reported in this study are based on the follow-up of 94 percent of this group. Operative mortality occurred in 22 percent of patients who had simple transposition of the great arteries, in 11 percent who had tetralogy of Fallot, and in less than 7 percent who had other abnormalities. These rates were significantly lower for those operated on in the last five years, with an operative mortality of 7 percent for transposition of the great arteries and 3 percent or less for the other abnormalities. Late cardiac mortality (at 25 years after surgery) was 5 percent for those who were operated on for tetralogy of Fallot, isolated ventricular septal defect (VSD), or pulmonic stenosis. It was 17 percent for aortic stenosis and 10 percent for coarctation of the aorta. For transposition of the great arteries, two rates were calculated because of the different surgical procedures used to treat it. Late cardiac mortality at 15 years was 15 percent for those who underwent the Mustard operation; the rate was 2 percent at 10 years for those who underwent the Senning procedure. These results indicate significant improvement in mortality of children with certain congenital heart defects. However, predictions of future mortality can not be based on these data. Cardiac surgery is now performed in younger children who have suffered less cardiac disease, and surgical techniques continue to improve. Long-term studies, such as the current report, help to identify specific patient groups who need intense follow-up care and determine how to further decrease mortality. (Consumer Summary produced by Reliance Medical Information, Inc.)

Infants born with defects of the heart and associated blood vessels used to die soon after birth. However, since the 1950s and 1960s, when surgical techniques to repair these cardiac anomalies were introduced, the life span of affected children has increased measurably. Initially, survival in the period after surgery was the focus of study, but many patients have now lived for many years following reparative surgery, allowing analysis of long-term survival and causes of death. A report by Morris and Menashe, to be published in JAMA, the Journal of the American Medical Association, studied all children from the state of Oregon who underwent reparative surgery for congenital heart defects before the age of 18 years. Most were treated at one institution, under the leadership of one surgeon. During the last 30 years, the age at surgery has decreased and in-hospital mortality has fallen substantially. Lingering cardiac illness and mortality were observed in some patients, particularly those with aortic stenosis (constriction), coarctation (stricture or narrowing) of the aorta, and transposition of the great arteries. Heart arrhythmias, congestive heart failure, and obstructive disease of the pulmonary (lung) blood vessels with pulmonary hypertension were the main causes of late mortality. Several features could have been improved in the study, including assessment of the outcomes of nonoperated patients and a better statistical analysis. Improved understanding of fetal and neonatal physiology has been crucial to improved survival; advancements in neonatology have allowed the development of medications that are beneficial for newborns, improved anesthetic, surgical, and postoperative treatments, and have made it possible to treat very young infants. With increasing surgical experience, the occurrence of restenosis and persistent hypertension in children with coarctation of the aorta may be minimized; addressing progressive coronary artery disease in these patients is important. Long-term patient function and survival will be maximized with a combination of early detection and surgery, appropriate postsurgical treatment, and fastidious long-term follow-up care. (Consumer Summary produced by Reliance Medical Information, Inc.)