Cor triatriatum dexter is a rare congenital heart anomaly in which a membrane divides the right atrium into 2 chambers. We report the case of a 43-year-old C or triatriatum dexter (CTD) is a congenital anomaly in which the right atrium is divided into 2 parts by a membrane or fibromuscular band. This condition is extremely rare: in high-volume echocardiographic laboratories, the prevalence of CTD is less than 0.01%.1 Cor triatriatum dexter is often associated with other congenital anomalies, such as pulmonary artery stenosis or atresia, hypoplastic right ventricle, Ebstein anomaly, and atrial septal defect (ASD).2,3 Symptomatic patients with CTD and ASD are typically candidates for surgery, although successful percutaneous procedures have been reported. 4,5 We report our experience with a CTD patient when we attempted percutaneous treatment of the anomaly.
Case ReportIn January 2013, a 43-year-old woman was examined at a regional hospital because of dyspnea on exertion (New York Heart Association [NYHA] functional class II-III). She had no signs of chronic heart failure. A continuous murmur was heard. A transthoracic echocardiogram (TTE) showed a 2.5 × 3.5-cm ASD (pulmonary-to-systemic flow ratio, 3:1) and a membrane that incompletely divided the right atrium into 2 chambers. Cor triatriatum dexter or a giant Eustachian valve was suspected, and the patient was referred to our institution.We used transesophageal echocardiography (TEE) and catheterization in an attempt to correct the anomaly percutaneously. The TEE showed that a membrane divided the right atrium into upper and lower chambers, thus conf irming a diagnosis of CTD (Fig. 1). Percutaneous balloon dilation disrupted the membrane and enlarged the communication between the chambers. This exposed the complete absence of an inferior rim on the patient's ASD: no tissue was available for anchoring an Amplatzer Septal Occluder (St. Jude Medical, Inc.; St. Paul, Minn). The risks of device malpositioning, device embolization, and residual shunting were high, so the patient was scheduled for surgery.Median sternotomy was performed and cardiopulmonary bypass was established. Access to the anomaly was gained through a right atriotomy. The large, fibrous congenital membrane, partially disrupted by the earlier percutaneous dilation, ran diagonally through the right atrium, reaching the interatrial septum on one end and the orifice of the inferior vena cava (IVC) on the other (Fig. 2A). A free margin and multiple small fenestrations enabled communication between the 2 atrial compartments. The ASD had a well-defined rim except in its inferior portion, where there was none. We resected the membrane, preserved its basal section for reconstructing the interatrial septum (Fig. 2B), and closed the residual ASD with use of a 3 × 2.5-cm pericardial