Non-compaction of the left ventricular myocardium with Cor-triatriatum and associated anomalies

Abstract

Non-compaction of ventricular myocardium (NVM) is a rare clinical entity. It has been reported more frequently in recent years because of continuous improvements in imaging techniques and resolution. Although apical region of the left ventricle is the most commonly involved site, biventricular involvement has also been reported in the published literature. This abnormality is often associated with other congenital cardiac and extracardiac anomalies. We describe such a case, incidentally detected and documented by the combination of echocardiography and multidetector CT coronary angiography. In our case, NVM was associated with Cor-triatriutum, ventricular septal defect, persistent left superior vena cava and an anomalous extracardiac vessel. Synchronous association of all these anomalies in a child has never been reported in the literature.

Left Ventricular Noncompaction Is More Prevalent in Ventricular Septal Defect Than Other Congenital Heart Defects: A Morphological Study

“…Many authors reported cases of patients presenting concomitant CHD and LVNC, including Ebstein [ 16 , 18 , 20 , 22 ], congenital atresia of the left main coronary artery [ 19 ], VSD [ 24 ], double-chambered left ventricle [ 23 ], patent ductus arteriosus [ 21 ], and also a complex congenital defect formed by cor-triatriatum , VSD, persistent left superior caval vein and an anomalous extracardiac vessel [ 17 ]. To date, however, only four studies sought to establish epidemiological associations between the two entities.…”

“…Understanding this association could guide both clinical practice, as well as anatomy, embryology and genetic studies aiming to understand the development of LVNC. Although many authors reported cases of patients presenting concomitant CHD and LVNC [ 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 ], to date, only four studies sought to establish epidemiological associations between the two entities. All of them, however, selected first patients known to have LVNC and looked for the prevalence of CHD among them.…”