Impact of gastrointestinal comorbidities in patients with right & left atrial isomerism

ABSTRACT Objective: Heterotaxy syndrome (HS), being right atrial isomerism (RAI) or left atrial isomerism (LAI) often presents with congenital heart disease (CHD). Intestinal abnormalities including malrotation are common. We aim to assess the impact of gut abnormalities on outcome in HS. Methods: We reviewed cardiology records of HS patients regarding presence of CHD, time for cardiac intervention, presence of gastrointestinal (GI) abnormalities and type/time of surgery. A questionnaire about GI status was sent to patients <18 years old. Kaplan-Meier curves were derived for survival data. Results: Data were available for 195 patients (49 RAI, 146 LAI) of 247 identified. Questionnaires were sent to 77 families, 47 replied. CHD was present in all RAI and 63.7% of LAI cases. Thirty-eight patients had abdominal surgery (19.5%), similar rate in RAI and LAI (20.4% vs 19.1%, p=0.92). Ladd procedure was performed in 17 (44.7%), non-Ladd in 12 (31.5%) and both procedures in nine patients (23.7%). Ten-year freedom from Ladd procedure for all was 86% (RAI=87%; LAI=85%, p=0.82). Freedom from any GI surgery at one year was 86% (RAI=86%; LAI=86%, p=0.98) and at ten years was 80% (RAI=77%; LAI=81%, p=0.65). Ten-year freedom from cardiac surgery was 34% (RAI=7%; LAI=42%, p<0.0001). Conclusions: In our cohort, one in five patients required abdominal surgery, mostly in their first year, similar in RAI and LAI. Between one and ten years of follow up the impact of GI abnormalities on outcome was minimal. Medium term survival was related to CHD.