Isolated Left Ventricular Noncompaction Cardiomyopathy Accompanied by Severe Mitral Regurgitation

Abstract

Isolated left ventricular noncompaction cardiomyopathy (IVNC) is a cardiomyopathy thought to be caused by arrest of normal embryogenesis of the endocardium and myocardium. This abnormality is often associated with other congenital cardiac defects. A 21-year-old man presented to the emergency department with worsening exertional dyspnea during the previous 2 months. Two-dimensional and Doppler echocardiography revealed an enlarged left atrium (LA) and a markedly dilated left ventricle (LV) with preserved LV systolic function, severe mitral valve regurgitation, and prolapse due to chordae rupture. The myocardium of the LV and right ventricle (RV) had excessively prominent trabeculations and deep intertrabecular recesses. He is the first patient in Korea who has undergone mitral valve replacement surgery because of severe mitral valve regurgitation and prolapse due to chordae rupture accompanied by IVNC.

Long-term myocardial recovery after mitral valve replacement in noncompaction cardiomyopathy

“…Clinical presentation is variable and can be any combination of heart failure, arrhythmias, embolic events and conduction disorders [ 1 ]. Severe mitral regurgitation associated with ILVNC has been also been documented recently [ 2 , 4 , 5 ]. Long-term outcome of patients with ILVNC is not clear, but a recent small study showed worse prognosis than in the general population, but similar to dilated cardiomyopathy patients [ 6 ].…”

“…Management of patients with ILVNC is same as that of other cardiomyopathies that require treatment for heart failure, and appropriate prevention and management of complications that include arrhythmias, conduction abnormalities, systemic emboli and valvular dysfunction like severe mitral valve regurgitation [ 3 ]. There have been few cases reported of ILVNC associated with severe mitral regurgitation [ 2 , 4 , 5 ]. But due to limited data, appropriate management and their long-term outcome is not clear.…”

Surgical Restoration of Left Ventricular Diastolic Function: Possible Treatment for Noncompaction Cardiomyopathy

“…Due to secondary enlargement of the annulus or possible subvalvular abnormality, VNC of the LV could be accompanied by moderate‐to‐severe mitral regurgitation. Several studies have addressed the clinical findings in this situation and surgical treatment . However, the surgeries mainly focused on valve problems.…”

“…Several studies have addressed the clinical findings in this situation and surgical treatment. [7][8][9] However, the surgeries mainly focused on valve problems. One case partially excluded the trabeculated ventricular cavity, 8 and none performed any surgical treatment to the abnormal myocardium.…”

Noncompaction Cardiomyopathy

“…Неком-пактный миокард левого желудочка часто сочета-ется с митральной региргутацией при сохраненной функции левого желудочка [26,27]. При такой соче-танной патологии наблюдаются склероз и кальци-фикация створок митрального клапана [28], и как следствие, ретракция створок [29].…”