Improved Surgical Management of Complex Neonates With Heterotaxy Syndrome.

Background: Neonatal management of congenital heart defects (CHD) among heterotaxy patients remains challenging due to significant heterogeneity in cardiac and visceral phenotypes. This study evaluated contemporary surgical outcomes and identified high-risk anatomic substrates. Methods: A total of 41 heterotaxy CHD patients who underwent neonatal surgical repair and/or palliation over a 10-year period were evaluated at a single institution. Heterotaxy anatomy was characterized according to right atrial isomerism (RAI) or left atrial isomerism (LAI), and other cardiac defects. Multivariate Cox regression and Kaplan-Meier analyses evaluated operative and intermediate-term outcomes. Results: Median age at initial operation was 7.0 days, and median operative weight was 3.1 kg. Median follow-up was 2.3 years. Of the total 41 patients, 27 (66%) had RAI, and 14 (34%) had LAI. Functional single ventricle anatomy was present in 30/41 patients (73%); 28/30 (93.3%) patients achieved stage II bidirectional Glenn and 14/30 (46.7%) achieved stage III Fontan completion; 1/30 (2%) patient underwent biventricular conversion. Operative mortality was 9.8% (4/41) after the initial operation. Permanent pacemaker placement was more common in patients with LAI. Interstage death rate was 10%, with no deaths after Fontan. Obstructed total anomalous pulmonary venous connection (TAPVC) was a risk factor for overall mortality (hazard ratio [6.0]; 95% confidence interval, 1.2-31.2; P = .033). Kaplan-Meier survival analysis demonstrated decreased five-year survival in RAI patients, LAI patients with single ventricle physiology, and RAI patients with obstructed TAPVC. Conclusions: Cardiac surgical outcomes for neonates with heterotaxy syndrome have significantly improved. Right atrial isomerism anatomy, single ventricle LAI patients, and RAI patients with obstructed TAPVC are associated with increased operative risk.