A Rare Case of Left Ventricular Dysfunction-Left Ventricle Noncompaction–Misdiagnosed Entity

Case Report

Austin J Clin Cardiolog. 2020; 6(1): 1066.

A Rare Case of Left Ventricular Dysfunction-Left Ventricle Noncompaction–Misdiagnosed Entity

Ali A*, Vijaykumar JR and Manjunath CN

Sri Jayadeva Institute of Cardiovascular Sciences Bangalore, India

*Corresponding author: Ahmad Ali, Sri Jayadeva Institute of Cardiovascular Sciences Bangalore, India

Received: December 23, 2019; Accepted: January 21, 2020; Published: January 28, 2020


Isolated ventricular noncompaction is an extremely rare cardiomyopathy; with prevalence of 0.014% in a group of patients referred to an echocardiography laboratory for abnormal findings or congestive heart failure not fully clarified. It is characterized by persistent embryonic myocardium morphology without associated cardiac abnormalities. Men appear to be more frequently affected; accounting for 56 to 82% of reported cases. Doppler echocardiogram is considered the reference method for diagnosis. Diagnosis remains difficult since there are similarities with other cardiac defects, clinical manifestations are nonspecific and echocardiographic criteria are not universally accepted. Treatment is directed towards important clinical manifestations (heart failure, arrhythmias and embolic events). We present a clinical case of severe congestive cardiac failure in previously healthy and asymptomatic 50 years old female, which was the initial presentation of an isolated ventricular noncompaction.

Learning points: Isolated ventricular noncompaction is a rare and recently described congenital cardiomyopathy. Its impact on morbidity and mortality is gaining importance as it is recognized with increasing frequency. An early, reliable diagnosis is crucial and echocardiography represents a prominent role. Treatment is directed towards its most important clinical implications, which include heart failure, arrhythmias and systemic embolic events. Heart transplantation is the only definitive treatment.

Keywords: LV noncompaction; LV dysfunction; Trabecular recesses; Hypertension


Isolated ventricular noncompaction (IVNC) is a rare cardiomyopathy, until now, not fully clarified. It is thought to result from an arrest of the compaction of loose myocardial meshwork during fetal development. It is generally associated with other congenital abnormalities such as obstruction of ventricular outflow tracts. IVNC is characterized by persistent embryonic myocardium morphology in the absence of other cardiac abnormalities. Engberding first described it in 1984 referring to a 33-year old woman with persistent “sinusoids” in the left ventricle as an isolated abnormality [1]. Most of the literature is based on a few case reports. Due to lack of pathophysiological characterization, IVNC has been unspecifically assigned to a heterogeneous group of “unclassified cardiomyopathy”. As a consequence, diagnosis is frequently missed, with important negative prognostic implications for these patients.

Case Report

Fifty years old, female, diagnosed outside as hypertension since 2 years on treatment, presented tothe emergency department with history of swelling of lower limbs and progressive dyspnea. ECG showed Left ventricular hypertrophy with nonspecific ST-T wave changes. She was diagnosed as hypertensive heart disease with moderate LV dysfunction with possibility of acute coronary syndrome. She was started on angiotensin-converting enzyme (ACE) inhibitors. Routine investigations including cardiac enzymes were normal. Chest x-ray demonstrated increased heart-thorax index with pulmonary venous hypertension. A new echocardiogram (Figure 1) demonstrated exuberant thickening and trabeculation of the lateral and posterior walls of the apical half of the left ventricle with two distinct myocardial layers: a normal compact (C) epicardium and a thickened non-compact (NC) endocardium. The ratio between NC endocardium and C epicardium =2.2 (measured at end systole in parasternal short axis view). Color flow imaging demonstrated flow within these spongiform recesses giving a “Swiss cheese like” appearance (Figure 2). Left ventricle cavity was dilated and presented diffuse hypo kinesis and an ejection fraction of 40%. No additional abnormalities were found. These findings were consistent with the diagnosis of IVNC. Contrast echocardiogram revealed multiple sinusoidal cavities within the spongy myocardium confirming noncompaction of LV (Figure 3).