Congenital heart disease is a common indication for pediatric heart transplantation, Dr. Jacqueline M. Lamour of Children’s Hospital at Montefiore in Bronx, New York told, but it presently (accounts for) a very small percentage of adults undergoing heart transplant.
This percentage, she added, is likely to increase because, as she and her co-authors note, congenital heart surgery is improving and patients are surviving longer. However, the researchers point out, patients who undergo the palliative Fontan procedure have a known propensity for late cardiac failure after the operation.
As congenital heart surgery improves, Dr. Lamour commented, the group that is most likely to come to transplant are the patients with single ventricle physiology.
To support the findings Dr. Lamour and her team studied 488 patients who received heart transplants for congenital heart disease. Of all, 93% had undergone at least 1 previous major operation. The median age was 12.4 years (range, 6 months to 62 years) at transplant, and 29 patients were older than 40 years.
The largest group was single-ventricle patients (36%), followed by patients with transposition of the great arteries (12%), and right ventricular outflow tract lesions, usually tetralogy of Fallot (10%).
Actuarial posttransplant survival was 71% and 60% at 1 and 5 years, respectively, in Fontan patients versus 83% and 74%, respectively, in other patients. Having a previous Fontan procedure increased the relative risk of death to 8.6, the researchers said.
While these data show transplant for congenital heart disease has results similar to the rest of the adult and pediatric transplant population if one survives the early post-operative period, Dr. Lamour said, the Fontan patient is at increased risk for poor survival.
Although cause of death was not analyzed in this study, one can speculate that this group of patients is at increased risk for preoperative malnutrition, operative bleeding and postoperative infection, she added. Research in developing strategies to minimize these risks will be important for improved survival in this group of patients.
