Noncompaction of ventricular myocardium, complete atrioventricular block and minor congenital heart abnormalities

Abstract

Ventricular noncompaction is a rare cardiomyopathy characterized by numerous, prominent ventricular trabeculations and deep intertrabecular recesses and is caused by a disorder of endomyocardial morphogenesis. It can occur in patients with other congenital anomalies, such as various ventricular obstructive lesions, or in isolated form in the absence of associated anomalies. Patients with this unique disease may present with heart failure, systemic emboli, and ventricular arrhythmias. In this case report, we present an unusual coexistence of ventricular noncompaction and atrial septal aneurysm and mitral valve cleft in a young patient who presented with complete AV-block.

Biventricular noncompaction presented with symptomatic complete heart block – Report of a case and review of literature

“…4 It is diagnosed primarily by echocardiography. 5 The most frequently applied echocardiographic definition of NCVM was established by Jenni et al, defined NVMC as the ratio of the end-systolic thickness of noncompacted/ compacted myocardium of greater than 2.0. 6 Another echocardiographic definition of NCVM which is also frequently applied was given by Stöllberger et al According to their criteria, NCVM is characterized by more than 4 coarse and prominent trabeculations, which move synchronously with the myocardium and have the same echogenicity and are surrounded by intertrabecular spaces.…”

Acquired Left Ventricular Hypertrabeculation/Noncompaction in Mitochondriopathy

“…In addition to echocardiography, LVHT can be visualized by cardiac MRI and ventriculography [6]. LVHT may occur without any other cardiac abnormality (lone LVHT) or may be associated with congenital cardiac malformations, thickening of the left ventricular (LV) myocardium, cardiac emboli, atrial septal aneurysm, valve abnormalities, LV dilatation, decreased LV systolic function or sudden cardiac death [7]. Lone LVHT does not require therapy, whereas LVHT associated with additional cardiac abnormalities needs appropriate treatment [7].…”

“…LVHT may occur without any other cardiac abnormality (lone LVHT) or may be associated with congenital cardiac malformations, thickening of the left ventricular (LV) myocardium, cardiac emboli, atrial septal aneurysm, valve abnormalities, LV dilatation, decreased LV systolic function or sudden cardiac death [7]. Lone LVHT does not require therapy, whereas LVHT associated with additional cardiac abnormalities needs appropriate treatment [7]. LVHT is associated with neuromuscular disorders, like Becker muscular dystrophy, Barth syndrome [2], myotonic dystrophy 1 [6], mitochondriopathy [1,4,8,9], myoadenylate deaminase deficiency, dystrobrevinopathy, cypher gene mutations, Pompe's disease, Friedreich ataxia and other rare genetic disorders, in four fifths of the cases [4].…”

Familial Left Ventricular Hypertrabeculation in Myotonic Dystrophy Type 1

“…Besides echocardiography, LVHT can be visualized by cardiac MRI and ventriculography [27][28][29]. LVHT may occur without any other cardiac abnormality (isolated or lone LVHT) or may be associated with other cardiac abnormalities, like congenital cardiac malformations, thickening of the left ventricular myocardium, systemic emboli, atrial septal aneurysm, valve abnormalities, left ventricular dilation, decreased left ventricular systolic function, or sudden cardiac death [30,31]. Lone LVHT does not require any cardiac therapy, whereas LVHT associated with other cardiac abnormalities needs adequate cardiac treatment [30,31].…”