Abstract 18813: Outcomes of Patients with Congenital Heart Disease following Heart Transplantation: the Impact of Disease Type, Prior Thoracic Surgeries and End-Organ Dysfunction
Introduction: Adults with congenital heart disease (ACHD) are at increased risk for early adverse outcomes following heart transplantation (Htx). Despite the need for improved risk stratification, small cohorts have constrained identification of patient-specific factors associated with poor prognosis. We hypothesized that type of CHD, number of sternotomies and prior end-organ dysfunction would be associated with an increased risk for mortality post-HTx.
Methods: We performed a retrospective, observational cohort study of all patients with ACHD who underwent HTx at our institution from 1/1997 to 1/2014. The primary endpoint was death. Exposures of interest included type of CHD, number of sternotomies and hepatic dysfunction secondary to passive congestion, measured as high Models for End-Stage Liver Disease Excluding INR (MELD-XI) score.
Results: 48 ACHD patients were followed (mean age at HTx: 36±1.7 years). Diagnoses included: Tetralogy of Fallot (TOF)/pulmonary atresia/double outlet right ventricle in 15 (31%), D-transposition of the great arteries in 10 (21 %), tricuspid atresia/double inlet left ventricle in 9 (19%), VSD/ASD in 4 (8%), heterotaxy in 3 (6%), congenitally corrected transposition of the great arteries in 2 (4%), and 5 (10%) with other diagnoses. Pre-transplant cardiac surgeries included: Fontan operation in 12 (25%), Mustard/Senning repair in 9 (19%), TOF repair in 5 (11%), Rastelli repair in 5 (11%), VSD/ASD closures in 3 (6%), other another congenital heart surgery in 8 (17%) and no prior cardiac surgery in 5 (11%).
Over a median follow-up time of 3.9 years, 14 patients died (29%) and 10 (71%) deaths occurred within 30 days of HTx. Survival at 1 and 5 years was 77%. Deaths within 30 days of surgery were secondary to hemorrhage in 4 (40%), graft failure in 3 (30%) and multi-system organ failure in 3 (30%). By multivariate analysis, ≥3 sternotomies (HR 8.5, p=0.02), MELD-XI score >18 (HR 6.2, p=0.009) and heterotaxy (HR 9.8, p=0.01), were significant predictors of mortality.
Conclusion: In our cohort of patients with CHD undergoing Htx, ≥3 sternotomies, MELD-XI score >18 and heterotaxy syndrome were significantly associated with death. These findings may be important in patient selection and timing of Htx in this population.
Author Disclosures: M.J. Lewis: None. J. Ginns: None. P.C. Schulze: None. M. Lippel: None. P. Chai: None. E. Bacha: None. D. Mancini: None. M. Rosenbaum: None. M. Farr: None.
- © 2014 by American Heart Association, Inc.