Abstract 15410: Post-operative Outcomes After Fontan Procedure in Patients With Heterotaxy and Other Situs Anomalies

Abstract

Background: Heterotaxy is a multisystem disorder of situs often associated with cardiac, immune, and gastrointestinal abnormalities. Heterotaxy and other situs abnormalities (H/SA) may share ciliary dysfunction as an embryologic origin. Extracardiac manifestations of H/SA may contribute to increased morbidity and mortality after total cavopulmonary connection (Fontan) procedure. We hypothesized that patients with H/SA have worse postoperative outcomes after Fontan than patients without H/SA. Methods: We queried the Pediatric Health Information System (PHIS) database for hospitalizations with ICD-9/10 codes for Fontan procedure in patients aged 1 to 12 years from 2004 to 2019. Those coded for dextrocardia, situs inversus, asplenia/polysplenia, and atrial isomerism were defined as H/SA. Outcomes were in-hospital all-cause mortality or heart transplantation, post-operative ECMO, dialysis, postsurgical length of stay (LOS), and mechanical ventilation >4 days. Outcomes were compared between H/SA and non-H/SA controls with chi-squared or rank-sum test (reported as median (IQR)), and statistically adjusted for surgical center, age, sex, race, genetic anomaly, and pre-operative support with multivariate logistic regression (reported as adjusted odds-ratio (95% CI)). Results: Of 7,897 patients who underwent Fontan at 50 PHIS centers, 1,366 (17%) met criteria for H/SA. Patients with H/SA versus non-H/SA were older (3.7 (2.9-4.8) vs 3.4 (2.7-4.2) years; p<0.001), more female (44 vs 40%, p=0.005), and Asian or Hispanic (35% vs 24%, p<0.001). H/SA had higher rates of discharge death/transplantation (1.9 vs 1.1%, aOR 1.76 (1.02-3.04); p=0.042), post-operative ECMO (3.7 vs 2.3%, aOR 1.73 (1.28-2.33); p<0.001), dialysis (2.1 vs 1.2%, aOR 1.66 (1.06-2.59); p=0.026), prolonged intubation (13.2% vs 7.6%, aOR 1.86 (1.53-2.25); p<0.001), and longer LOS (11 (8-17) vs 9 (7-13) days; p<0.001). Conclusions: We observed increased mortality and postoperative complications in this largest report to date on patients with H/SA after Fontan. We postulate that ciliary dysfunction may contribute to increased lung and renal morbidity, and should be explored further. Adjustment for anatomic complexity and hemodynamics are required for further study .