Left Ventricle Non Compaction: Could it be Reversed Back?

Left Ventricle Non Compaction: Could it be Reversed Back?

 

S.P. Mahaley¹, Irnizarifka², H. Arifianto²

¹General Practitioner, ² Cardiologist at UNS Hospital, Department of Cardiology and Vascular Medicine, Sebelas Maret University

  

Introduction

Left Ventricular non-compaction (LVNC), also known as ‘spongy myocardium is a rare abnormality of the left ventricular (LV). It is a pathophysiological process involving the arrest of the normal compaction of the embryonic sponge-like meshwork of interwoven myocardial fibers. Heart failure remains the most common presentation besides arrhythmias, and propensity for thromboembolism (21-24%). Impaired filling and abnormal relaxation leading to diastolic heart failure could also result from prominent trabeculations. With its under-developed myocardium, its reversibility is in question.

The diagnosis could be established primarily based on echocardiography with contrast ventriculography, CT and MRI as additional examination. LVNC on echocardiogram typically characterized by two-layered myocardial structure with thin compacted outer (epicardial) band, a much thicker non-compacted inner (endomyocardial) layer and deep myocardial trabeculae, particularly in the apex and free wall of the LV.

One reported that beta blocker may have benefits for LV function, and this may improves prognosis.  At that study, LVEF increased from 30% to 57% using beta blocker treatment, particularly carvedilol. Left ventricular end diastolic volume, end systolic volume, and end diastolic pressure decreased strikingly after treatment. After adequate therapy, non-compacted myocardium occupied a smaller percentage of the total ventricular wall area than before treatment.

Case

This is a case report of 56 years old woman who came to outpatient clinic of UNS hospital with chief complain of chest discomfort. She was recently diagnosed with HF due to ischemic cardiomyopathy, but has worsening symptoms despite optimal ischemic medications. Her metabolic profile and kidney function were within normal limit.

Echocardiography showed dilated all chamber, global hypokinetic, 15% LVEF with moderate mitral regurgitation. Echocardiography also proved a two-layered structure with a thin, compacted layer (C) and a thickened noncompacted layer (NC) at end-systole with ratio of NC/C >2, which ensures diagnosis of LVNC. Coronary CT angiography confirmed normal coronary arteries, which made us decide to stop standard ischemic therapies given to improve her compliance.

Furosemide, ramipril, and bisoprolol were initiated and uptitrated according to guidelines. Education regarding her disease and the importance of her compliance was routinely done every month. After 3 months, her functional class improved dramatically under 10 mg ramipril, 5 mg bisoprolol, and on demand 40 mg furosemide. Echocardiography evaluation evinced increase in LVEF (23.5%) and reduction of NC area.

Summary

LVNC is rare disease that has been increasingly diagnosed in clinical practice. There is no specific therapy for LVNC, thus adequate education should follows optimal guidelines directed medical therapy (GDMT) in order to produce better outcome. We have managed 56 years old woman with LVNC and prove its early reversibility through adequate education and simpler GDMT.

 

Keywords : LV Non Compaction, Echocardiography, Education, GDMT, Reversibility