Improved current era outcomes in patients with heterotaxy syndromes☆

Abstract

Patients with heterotaxy syndrome have a myriad of visceral and cardiac malformations historically resulting in significant morbidity and mortality. We sought to assess whether current era management strategies have improved outcomes in patients with visceral heterotaxy. A retrospective review (1994-2008) of our database identified 45 consecutive heterotaxy patients who underwent surgical palliation. There were 29 patients with right atrial isomerism (RAI) and 16 patients with left atrial isomerism (LAI). Functional single ventricle was present in 32 patients. Pulmonary outflow obstruction was present in 29 of the patients. Twenty patients had total anomalous pulmonary venous return (TAPVR), of which 9 were obstructed. An initial neonatal surgical approach was performed in 27 patients. Thirty patients had systemic to pulmonary artery shunt. Mean follow-up was 43.6+/-47 months in RAI and 41.0+/-40.8 months in LAI patients (p=0.4). There were three hospital deaths, all after the first operation, and four interstage deaths (six RAI; one LAI). There were no deaths after cavopulmonary shunt, Kawashima or Fontan operation. A multivariate Cox regression identified greater than moderate atrioventricular valve regurgitation (Hazard Ratio (HR) 17.5, p=0.017) and obstructed TAPVR (HR 17.5, p=0.007) as factors associated with increased RAI mortality. Due to the absence of late mortality in both groups, patient survival at 3 years were 79% in RAI and 94% in LAI patients and remained stable after that (p=0.22). All survivors but one are in NYHA class I or II, without significant cardiovascular related symptoms. LAI patients have a higher incidence of sinus node dysfunction than RAI patients (47% vs 12.5%, p=0.009). Surgical outcomes in heterotaxy patients are improving in the current era. The risk for operative mortality and attrition is highest between the first and second stage palliation procedures. Significant atrioventricular valve regurgitation and obstructed TAPVR remain risk factors for RAI mortality. Survivors are doing well with no activity restrictions, although LAI patients maintain a higher proclivity of sinus node dysfunction.