Late follow-up of patients who underwent palliation for complex congenital heart disease in childhood.

Late follow-up of patients who underwent palliation for complex congenital heart disease in childhood.
Congenital heart disease, Mar 2008, vol. 3, no. 2, p. 155-8
Hoffmann-Andreas, Wyler-Felix, Guenthard-Joelle, Graedel-Erich.
Abstract
We report on a series of 12 patients with complex congenital heart disease who had reached adult life after surgical palliation in early childhood and who were systematically followed by clinical visits. Patients were born between 1953 and 1979 and were followed up into 2007. All patients had complex lesions with single ventricle physiology and were palliated either by banding of the pulmonary artery or by creation of systemic-pulmonary shunts. Single operations were performed in 4 cases, and 8 patients had 1 or more further palliative interventions. Late corrective surgery and heart transplantation were performed in 1 patient each. Eight survivors reached a mean age of 36 years at follow-up (range 28-48), whereas 4 patients died at a mean age of 32 years (range 22-53). All patients were in New York Heart Association classes II and III. Complications during follow-up were bacterial endocarditis (3), cerebrovascular accidents (3), arrhythmias (3), need for pacemakers (2). Deaths occurred perioperatively at transplant (1), or were caused by sudden death (2), and hypoxia related to fall in blood pressure (1). Successful pregnancies occurred in 3 patients with healthy (small) babies delivered by cesarean section in 2 of them, and vaginal delivery in 1. The course of life in these patients shows not only a wide spectrum with good quality of life in some of them but also a large number of potentially severe complications. Survival beyond 50 years is rare.