Abstract 17363: Has Survival of Patients With Heterotaxy Syndrome Improved in the Current Era?
Introduction: Most reports on outcomes in heterotaxy syndrome (HS) have focused on early mortality with little information on long-term survival. Some reports suggest improved survival in more recent years, but others have failed to show similar results.
Hypothesis: Mid- to long-term outcomes for HS have improved over time.
Methods: Patients with HS born between 1985 and 2007 were included. Kaplan-Meier analysis and log-rank test were used to estimate and compare the survival distributions of three birth cohorts (E1=1985-1991, E2=1992-1998, E3=1999-2007). Risk factors for mortality were identified using Cox proportional hazards regression and a stepwise multivariable model constructed.
Results: The 160 patients were 53% female, 53% diagnosed prenatally, 89% with ≥1 cardiac surgery; 80 (50%) died. Anatomic diagnoses, age at first surgery, and rates of single versus biventricular circulation were similar across cohorts. Estimated 15-year survival was 56%, with no birth era difference (E1=51%, E2=58%, E3=60%, p=0.3). Rates of prenatal diagnosis were higher in E3 (E1=36%, E2=51%, E3=77%, p<0.001) but prenatal diagnosis was not associated with mortality (p=0.3). Among those who underwent surgery (n=140), independent risk factors for mortality (all p<0.001) were: totally anomalous pulmonary venous connection (TAPVC; HR=3.2, 95% CI 1.9-5.5), single ventricle palliation (HR=2.3, 95% CI 1.1-4.8) or other non-biventricular surgeries (HR=8.1, 95% CI 2.7-24.6), and ≥mild atrioventricular valve regurgitation (AVVR) at presentation (HR=2.2, 95% CI 1.3-3.7). We found no risk factors that depended on birth era.
Conclusions: Patients with HS have poor long-term survival with no significant improvement in mortality rates over time. Prenatal diagnosis is not associated with lower mortality. TAPVC, single ventricle or other non-biventricular surgery, and ≥mild AVVR at presentation are independently associated with poor outcomes.
Author Disclosures: P. Banka: None. B. Schaetzle: None. A. Adar: None. L. Sleeper: None. T. Geva: None.
- © 2016 by American Heart Association, Inc.