A Heart With Quadruple Atria
Does it Exist?
A 72-year-old woman was admitted to our department for further investigation of a heart defect noted in transthoracic echocardiography performed in a district hospital. The patient complained of palpitations and moderately limited physical activity.
On ECG, atrial fibrillation was recorded with a well-controlled ventricular rate. The chest x-ray demonstrated an enlarged heart and signs of moderate pulmonary congestion.
The transthoracic echocardiography showed gross enlargement of the left atrium and its division into 2 compartments without flow obstruction and an excessively grown Eustachian valve within the right atrium (Figure 1). Furthermore, study revealed the paradoxical interventricular septum movement, reduced left ventricular ejection fraction (49%), mild mitral regurgitation resulting from mitral annular calcification, and mild tricuspid regurgitation. A central atrial septal defect causing an insignificant left-to-right shunt was also detected. Transesophageal echocardiography confirmed the presence of a bilateral atrial division (Figures 2–4 and Movies I–IV in the online-only Data Supplement) and enabled the estimation of left atrial membrane fenestration size (superior, 1.8 cm; inferior-posterior, 3.5 cm).
Computed tomography imaging was performed to demonstrate the anatomy of both atria and to exclude coronary artery anomalies. It revealed the fibrous diaphragm within the left atrium with pulmonary veins draining into the upper chamber (Figures 5 and 6).
Cor triatriatum sinister is a rare congenital heart defect that results from an abnormal fetal development of pulmonary veins.1 The prevalence of cor triatriatum dexter is exceedingly rare.2 The case we describe is unusual because of the coexistence of both left and right atrial divisions. Such a situation, although clinically benign, is worth mentioning.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.115.016814/-/DC1.
- © 2015 American Heart Association, Inc.