Long-term outcomes after heart transplantation in adult patients with cardiac congenital heart disease: impact of initial defect and repair: a collaboratve study on 97 patients

Abstract

Purpose: An increasing number of children with complex congenital heart disease reach adulthood because of the advances in pediatric cardiology and surgery. Residual abnormalities in cardiac structure and function predispose adults with congenital heart disease (ACHD) to late-onset heart failure and its complications. Heart transplantation (HTx) often remains the only option. But ACHD represent a heterogeneous population. We thought to compare outcomes in different defects and in different physiopathological models of repair i.e. univentricular (1V) or biventricular (2V) status. Methods: Out of a multi-institutional (3 centers) series of 2257 HTx from 1988 and 2012, 100 (4.4%) were performed in 97 ACHD aged ≥16 years at the time of operation (65 men). We evaluated clinical data, etiology, surgical history, perioperative issues and outcomes. Results: The mean age at the time of HTx was 29.8±10.5 years (range 16-58). Forty-three patients (44%) had 1V physiology including 16.5% Fontan-type circulation and 54 (56%) had 2V physiology including 26% with a systemic right ventricle. Primary diagnosis included single ventricle (27%), D-transposition of the great arteries (20%), tricuspid atresia (13%), tetralogy of Fallot (12%), congenital valvular heart diseases (11%), corrected transposition of the great arteries (7%), and other anomalies (9%). Eighty-seven patients (90%) had undergone ≥1 prior cardiac surgery before HTx.An additional surgical procedure during the HTx, like pulmonary artery reconstruction, was performed in 22% patients. Twenty-four patients (25%) required reintervention for post-operative hemorrhage. In-hospital mortality was high (34%). Multivariable factors associated with increased in-hospital mortality included female gender of the donor and tricuspid atresia. The 1V patients were more often younger (26.9 vs 32.1 years, p=0.014), had undergone more surgeries (2.9 vs 1.9 prior surgeries, p=0.002), had a lower requirement for ventricular assist device implantation before HTx (4.7% vs 20.4%, p=0.02), and had longer ischemic (229 vs 182min, p<0.001) and cardiopulmonary bypass times (240 vs 163min, p<0.001). Long-term survival was significantly higher in 2V patients (p=0.023). Conclusion: HTx of ACHD poses several surgical challenges due to complex anatomy, specific physiology and prior surgical interventions. 1V physiology is associated with higher in-hospital and long-term mortality, in comparison to 2V physiology. Further studies are warranted to identify whether earlier HTx of these patients may improve these outcomes.