Never Too Old for Congenital Heart Disease: A Case of Cor Triatriatum Sinistrum and Mitral Valve Prolapse

Abstract

Cor triatriatum is a congenital atrial abnormality, which comprises a membrane that divides the atrium into two chambers. Symptoms can arise either due to the obstructive nature of this membrane or related to other associated anomalies, such as atrial septal defects or abnormal pulmonary venous return. The authors report the case of an adult male, in whom an incidental finding of cor triatriatum sinistrum was documented in association with mitral valve prolapse. However, both the late diagnosis and the association with mitral valve disease are uncommon. Multimodality imaging evaluation can prove very helpful in these cases to better define the anatomy of the left atrium and appropriately plan for intervention when indicated.

Keywords: 3d imaging; adult congenital heart disease (achd); cor triatriatum sinister; mitral valve prolapse; multimodality cardiac imaging.

Figure 1. Transthoracic ECG showing both the presence of severe mitral regurgitation (top row) and cor triatriatum sinistrum (lower row; red arrow).

A) Apical 4-chamber view; B) Parasternal short-axis view; C) Parasternal long-axis view; and D) Three-dimensional biplanar apical view. LA: Left atrium; LV: Left ventricle.

Figure 2. Cardiac magnetic resonance showing no myocardial fibrosis and confirming the presence of cor triatriatum sinistrum (red arrow).

No late gadolinium enhancement in both 4-chamber (A) and 2-chamber views (B); 5 chamber T1-imaging (C), showing a membrane dividing the left atrium.

Figure 3. Transesophageal echocardiography showing a severe mitral regurgitation, due to anterior leaflet prolapse at A2 and A3 scallops. The eccentric jet, directed laterally and posteriorly, reaches the left atrium's ceiling.

A) Three-dimensional imaging of the mitral valve, atrial view; B) mid-esophageal 4-chamber view (0º) with colour Doppler.

Figure 4. Transesophageal echocardiography characterizing the cor triatriatum sinistrum. The LA is partially divided by a nonrestrictive membrane, shaped like a diaphragm, with a single posterior wide opening (red arrow).

A) Biplanar mid-esophageal view; B) modified mid-esophageal view at 90º; C) Three-dimensional reconstruction of the septum, atrial view.  LA: Left atrium; LV: Left ventricle.