Cor triatriatum dexter in children: Literature review and case report

Abstract

Cor triatriatum dexter (CTD) is one of the rarest congenital cardiac malformations in which there is an abnormal septation of the right atrium (RA) due to the failure of regression of the embryonic right-sided sinus venosus valve.1 We present our experience regarding a young patient with CTD associated with severe tricuspid regurgitation and review the 13 pediatric cases of CTD previously reported in the literature who underwent surgery (Table 1).1-12 Informed consent was received for our patient. A female child was initially seen at our institution at the age of 5 years of age because of frequent episodes of palpitation on exertion. Her electrocardiogram exhibited a normal sinus rhythm with a heart rate of 100 beats/min. Peripheral arterial oxygen saturation was normal (99%). Transthoracic echocardiography (TTE) detected the presence of a wide unrestrictive membrane in an enlarged RA and revealed mild-to-moderate tricuspid regurgitation with no evidence of Ebstein disease or any intracardiac shunting. At regular clinical examinations, the patient began demonstrating progressive dyspnea, palpitations, and fatigue on moderate exertion. Her latest TTE control, at the age of 9 years, confirmed the presence of this wide membrane in the RA, which was protruding toward the tricuspid valve (TV) during diastole and also revealed severe central regurgitation during systole (Video 1). At surgery, the RA was opened and a nonobstructive membrane extending across the RA—which was dividing it into 2 chambers, with both caval veins and the coronary sinus on one side and the TV and RA appendage on the other—was exposed (Figure 1). This membrane, which was completely covering the TV leaflets, was carefully resected starting from the upper and lower parts of its anterior unobstructed sinoatrial orifice. After resection, the TV was exposed and its analysis confirmed the suspicion of annular dilatation as well as the presence of a retracted fibrotic septal leaflet with limited mobility. Tricuspid annuloplasty with a Carpentier Edwards tricuspid ring (Edwards Lifesciences, Irvine, Calif) size 28, and a stitch closure of the anteroseptal commissure were performed. Postrepair transesophageal echocardiography confirmed the satisfactory surgical correction with no residual leak. The postoperative course was uneventful.