Noncompaction of Ventricular Myocardium in a Patient with Congenitally Corrected Transposition of the Great Arteries Treated Surgically: Case Report

Abstract

Noncompaction of the ventricular myocardium is a rare disorder that represents numerous prominent trabeculations and intratrabecular recesses in the ventricles. It is believed to represent not only an arrest in endomyocardial morphogenesis but also an unclassified cardiomyopathy. The pathology has been almost invariably associated with other congenital cardiac malformations. A female patient with noncompaction of the myocardium of both ventricles and congenitally corrected transposition of the great arteries (cTGA), situs inversus totalis, and atrial and ventricular septal defects is described. When she was 7 days old a permanent pacemaker was implanted because of complete heart block. Prazosin (Minipress), an alpha-receptor blocker, was administered, and the cardiac ejection fraction showed a striking increase from 20% to 42%. Despite careful and regular follow-up evaluations, the general condition of the patient slowly worsened. Five months after surgery she died of hepatorenal failure and low cardiac output. This case report is thought to be the first description of congenital complete heart block, cTGA, and situs inversus totalis with noncompaction of the myocardium of both ventricles.

Left ventricular noncompaction with a single coronary artery of anomalous origin

“…The disease is a form of congenital cardiomyopathy that affects the left or right ventricle. It frequently presents as an isolated anomaly involving the ventricular myocardium; although it can be associated with other congenital abnormalities such as valvular heart disease, congenital heart block and great vessel malformations [1,2]. Here we report a case of NVM combined with a coronary abnormality.…”

A possible diagnosis of isolated right ventricular hypertrabeculation /noncompaction in an elderly man by three-dimensional echocardiography

“…Some cases were associated with noncompaction of the left ventricular myocardium [11], while others were not [12]. The cases are extremely rare to be reported in which only the right ventricle was involved without evidence of left ventricular involvement in an adult subject.…”

“…Although there is no established criteria for HVM/NVM in the right ventricle, right ventricle HVM/NVM has been reported in some cases [6][7][8][9][10][11][12] in whom the diagnosis of right ventricle HVM/NVM were pretty dependent on 2DE. The morphological assessment of the right ventricle is often difficult in adult patients by 2DE and 2DE image quality is operator dependent.…”

An isolated right ventricular hypertrabeculation and dyskinesia in an elderly man: A possible diagnosis of isolated right ventricular noncompaction?

“…Some cases were associated with noncompaction of the left ventricular myocardium [7], while others were not [8]. The cases are extremely rare to be reported in which only the right ventricle was involved without evidence of left ventricular involvement in an adult subject [9].…”