The heterotaxy syndrome: associated congenital heart defects and management.

Heterotaxy syndrome (HS) constitutes a spectrum of anomalies arising from embryological errors that result in abnormalities of lateralization involving thoraco-abdominal viscera and culminate in loss of normal asymmetric arrangement of these organs. Besides the unique challenges involved in planning and execution of surgical procedures aimed at correction or palliation of these anomalies, they have the potential to cause profound physiological and immunological consequences in the individual patient due to their cardiac and extra-cardiac manifestations. This article aims to review the literature on this rare and extraordinary subset of developmental anomalies with the intention of familiarizing the reader on the modes of presentation, manifestations, and the variations thereof while dealing with this anomaly. In our institutional experience with HS, 75 consecutive patients were seen between January 2011 and September 2018. Of these, 48 (64%) were confirmed to have isomerism of right atrial appendages (IRAA) and the rest had isomerism of left atrial appendages (ILAA). The cardiac and extra-cardiac manifestations of these patients were listed out. Fifty-four patients (34 with IRAA and 20 with ILAA) underwent 83 surgical procedures. While 49 patients were palliated on the univentricular pathway, 5 underwent biventricular repair. The in-hospital mortality was 7 (13%) in both groups combined (5 for patients with IRAA and 2 for ILAA). In conclusion, the surgical management of HS is associated with satisfactory outcomes in current era. © Indian Association of Cardiovascular-Thoracic Surgeons 2020.