Comparison of morphologic profile of congenital heart defects associated with right isomerism and left isomerism in Western Indian population

Context: Heterotaxy syndrome is a disorder that involves abnormal lateralization of the abdominal viscera, thoracic organs, and cardiac atria. The objective was to compare the frequency of morphologic profile of congenital heart defects associated with right isomerism and left isomerism in the Western Indian population.Materials and Methods: This cross-sectional observational study was conducted by the Department of Pediatric Cardiology, U N Mehta Institute of Cardiology and Research Centre, Ahmedabad, Gujarat, India, from January 2016 to April 2018. All children presenting to the hospital with suspected congenital heart defect were included in the study. Echocardiography-based sequential segmental analysis was used; various cardiac defects were noted for patients with right isomerism (RAI) and left isomerism (LAI).Results: Occurrence of heterotaxy in our screening population is 0.12%. A total of 93 children had atrial heterotaxy (M: F; 1.06:1), with 65 (69.89%) having RAI and 28 (30.11%) having LAI. Most common lesions associated with RAI included complete atrioventricular septal defect (n = 43, 66.2%), and pulmonary outflow tract obstruction (n = 35, 53.84%). LAI was associated with atrial septal defect (n = 24, 85.7%) ventricular septal defect (n = 23, 82.1%).Conclusion: Heterotaxy encompasses a wide spectrum of congenital cardiac defects. The frequency of various defects associated with RAI and LAI in Western India is compared here to those as reported in the Western world.