Transplantation for complex congenital heart disease in adults: a subanalysis of the Spanish Heart Transplant Registry.

Background: Congenital heart diseases (CHDs) have high infant mortality in their severe forms. When adulthood is reached, a heart transplant (HTx) may be required. Spanish adult population transplanted for CHD was analyzed and compared with the most frequent causes of HTx and between different subgroups of CHD.

Materials And Methods: A total of 6048 patients (HTx 1984-2009) were included. Pediatric transplants (<15 yr), combined transplants, reHTx, and HTx for heart diseases other than idiopathic dilated cardiomyopathy (IDCM) and ischemic heart disease (IHD) were excluded. Total patients included: 3166 (IHD = 1888; IDCM = 1223; CHD = 55). Subgroups were studied as follows: (1) single ventricle with pulmonary stenosis (n = 18), (2) single ventricle with tricuspid atresia and Glenn/Fontan surgery (n = 10), (3) congenitally corrected transposition of the great vessels (TGV) or with switch atrial surgery (n = 10), and (4) CHD with right ventricle overload (n = 17).

Results: Survival probability was different between groups (p = 0.0001). Post hoc analysis showed some differences between groups (CHD vs. IHD, p = 0.05; CHD vs. IDCM, p = 0.5; IHD vs. IDCM, p = 0.0001). Early mortality was different between CHD subgroups (group 1 = 19%, group 2 = 40%, group 3 = 0%, group 4 = 29%; p < 0.001); however, overall mortality did not show differences between subgroups (p = 0.5).

Conclusions: The percentage of Spanish adult HTx patients for CHD is low (1%). The survival curve is better than for other HTx causes (IHD). Nevertheless, early mortality was higher, particularly in some subgroups (Fontan).