Cor Triatriatum Dexter With a Sinus Venosus Atrial Septal Defect in a 50-Year-Old Woman: A Case Report

The diagnosis of atrial septal defect (ASD) may be delayed until adulthood or even later in life as it is a well-tolerated congenital heart disease. If patients are not examined and investigated well in childhood, the diagnosis may be delayed until later in adulthood when patients present with palpitations and sometimes dyspnea due to the right chambers dilatation from right ventricular volume overload. In this report, we present a case of a 50-year-old female patient with symptoms of heart failure and atrial fibrillation who was found to have dilated right cardiac chambers, dilated pulmonary artery, severe tricuspid regurgitation, pulmonary hypertension, and a pulmonary-to-systemic flow ratio (Qp/Qs) of more than 1.5 by transthoracic echocardiography and Doppler, indicating left to right shunt at the atrial level. However, transthoracic echocardiography could not visualize the defect, and two-dimensional (2D) transesophageal echocardiography was done in this patient and documented the presence of a sinus venosus ASD with an incomplete cor triatriatum dexter membrane; all four pulmonary veins were identified going to the left atrium. Since the presence of an incomplete cor triatriatum dexter membrane (despite causing no symptoms) makes the percutaneous closure of the sinus venosus ASD and the percutaneous repair of tricuspid regurgitation very difficult, we decided to advise surgical ASD closure and tricuspid valve repair for the patient.