Cor Triatriatum Dextrum in a Pregnant Woman Combined With an Atrial Septal Defect and Severe Pulmonary Hypertension: A Rare Case Report

Cor triatriatum, first described by Church in 1868, is an extremely rare congenital cardiac defect and accounts for between 0.1% and 0.4% of all congenital cardiac anomalies. Isolated cor triatriatum is rare and is usually associated with other cardiac anomalies, most commonly the atrial septal defect (ASD). Although the clinical presentations of cor triatriatum depend on the size of the fenestration, almost all cases are diagnosed in childhood and very few cases remain asymptomatic until adulthood. Several techniques such as transthoracic echocardiography, transesophageal echocardiography, computed tomography, and magnetic resonance imaging are used for the diagnosis of cor triatriatum; nevertheless, the definitive diagnosis and the identification of the associated anomalies can be easily made by echocardiography. The use of computed tomography is associated with the risk of radiation, and transesophageal echocardiography has the discomfort of scope intubation. 5
We herein describe a 35-year-old pregnant woman with no previously described cardiac disorder who was diagnosed with cor triatriatum dextrum presenting with shortness of breath, tachycardia, and palpitation of 1 week’s duration. She had a history of 5 uncomplicated vaginal deliveries. (Iranian Heart Journal 2020; 21(4): 131-134)