Abstract
Cor triatriatum is a rare congenital heart defect characterized by the presence of a fibromuscular membrane dividing the left atrium into two distinct chambers. Lipomatous hypertrophy of the atrial septum is an infrequently observed benign abnormality caused by large fatty tissue deposits in the interatrial septum. An increased incidence of atrial arrhythmias is described in both pathologies, while a significant obstruction of blood flow mimicking mitral stenosis is typically manifested in cor triatriatum. We report the case of a 75-year-old woman with a previously undescribed association of the above stated abnormalities detected by both transthoracic and transeosophageal echocardiography. Diagnosis was confirmed by means of computed tomography. The singular physiologic and anatomic factors underlying survival until such a late age are described. The diagnostic, therapeutic and surgical management is discussed and a short review of the literature performed.
Highlights
Cor triatriatum (CT), first described by Church in 1868, is a rare congenital heart disease. It is characterized by the presence of a fibromuscular diaphragm which divides the left atrium into two chambers: a posterosuperior chamber receiving blood flow from the pulmonary veins and an anteroinferior chamber communicating with the mitral valve and the left atrial appendage
The postoperative course was uncomplicated and the patient was discharged from hospital one week after surgery with a saturation of 98%. In conclusion, this previously unreported association of CT and Lipomatous hypertrophy (LH) of the atrial septum contributed to the development of atrial fibrillation and subsequent heart failure
Subsequent fibrosis and calcification of the orifice manifested on ageing, and the development of both mitral stenosis and atrial fibrillation may account for the late conversion to a symptomatic state
Summary
Cor triatriatum (CT), first described by Church in 1868, is a rare congenital heart disease (presenting approx. 0.4% of all congenital cardiac anomalies). In our patient no significant obstruction of the superior caval vein was detected, chronic fibrillation was observed. No tendency towards a rapid increase has been observed for LH of the interatrial septum To this regard complete excision of the tumour is not strictly necessary. Transthoracic echocardiography detected a membrane subdividing the dilated left atrium into two chambers and hypertrophy of the interatrial septum (Figure 2). Transeosophageal echocardiography revealed the presence of the above stated diaphragm in the left atrium as well as echogenic hypertrophy of the interatrial septum (about 2 cm), sparing the foramen ovale. As a consequence of the identification of the pulmonary veins, an excision was made into the intra-atrial membrane (radial incision from the orifice of the diaphragm to the atrial septum). The postoperative course was uncomplicated and the patient was discharged from hospital one week after surgery with a saturation of 98%
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