Atrioventricular Septal Defect with Cor Triatriatum: Case Report and Review of the Literature

Atrioventricular septal defect with cor triatriatum is a rare combination, with only nine cases reported so far. Cor triatriatum, a potentially correctable cause of pulmonary arterial hypertension in atrioventricular septal defect patients, was missed in a few earlier case reports, leading to death and subsequent diagnosis at autopsy. We report the case of 3-year-old female child with this combination (partial atrioventricular septal defect with cor triatriatum) wherein the final diagnosis was made at cardiac catheterization. Successful surgical correction was performed.