Long-Term Outcomes And Predictors For Survival Following Fontan In Patients With Single Ventricle Anomalies And Heterotaxy Syndrome
*Bahaaldin Alsoufi1, Kristina Kuo2, J'Neka Claxton2, Jessica Knight2, Lazaros Kochilas2
1University of Louisville, Prospect, Kentucky, United States, 2Emory University, Atlanta, Georgia, United States
Objective: Heterotaxy syndrome is established mortality risk factor at all single ventricle palliation stages. We aim to examine long-term outcomes and predictors of survival following Fontan in patients with heterotaxy.
Methods: Patients who underwent Fontan from 1986 to 2003 were reviewed (n=1735). 195 patients with heterotaxy syndrome were identified. Analysis of patient characteristics, hemodynamic, anatomic and surgical details and their influence on transplant-free survival was performed.
Results: Median age at Fontan was 3.7 years. Patients had right isomerism (n=109), left isomerism (79), non-specified (n=7). Anatomic details included atrioventricular septal defect (AVSD, n=134), total anomalous pulmonary venous connection (TAPVC,n=46), bilateral SVC (n=37), interrupted IVC (n=69). The dominant ventricle was left (n=56), right (n=120), unknown (n=19). 170 (87%) had undergone Stage-I palliation (most commonly shunt in 129) and 162 (83%) had undergone prior Stage-II palliation (including 62 Kawashima). Hospital survival was 87% (94% in later era). Among 169 hospital survivors, 16 received heart transplant and 33 died (with 7 dying after transplant). Overall survival was 84%,78%,74%,67% at 1,5,10,20 years. In comparison with the non-heterotaxy Fontan cohort, patients with heterotaxy had higher operative mortality (13% vs. 6%) and transplant/attrition following discharge (25% vs. 12%). For the heterotaxy group, risk factors for low transplant-free survival included AVSD (HR=2.49,95%CI=1.33-4.65,p=0.004), and PA pressure >15mmHg (HR=1.1,95%CI=0.95-1.21,p=0.05). Age, right isomerism, dominant ventricle, Fontan type, fenestration, and TAPVC repair history were not significant factors for transplant-free survival.
Conclusions: Fontan procedure in patients with heterotaxy syndrome is associated with relatively high operative mortality and late attrition/transplant need risk. Mortality decreased in the later era. While many anatomic and surgical factors were not found to influence survival after Fontan, AVSD and elevated PA pressures were risk factors. Better long-term treatment strategies to address specific complications in this challenging group are necessary.
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