The molecular genetics of inherited cardiomyopathies have been reviewed and the evidence to support routine genetic testing in all patients diagnosed with LVNC is not available [11,12].
There is much debate regarding the diagnostic criteria for LVNC and the predilection for overdiagnosis.
Management of isolated LVNC includes treatment for heart failure, arrhythmias, and thromboembolic events.
Long-term prophylaxis with oral anticoagulants seems beneficial in all cases with isolated LVNC and has been especially effective in cases where impaired left ventricular function, thrombi, or atrial fibrillation have been documented.
The inheritance of isolated LVNC may be sporadic or familial.
LVNC represents the persistence of multiple trabeculations in the ventricular myocardium with deep intratrabecular spaces due to arrested compaction of the wall.
The diagnosis of LVNC was established by 2-dimensional and color Doppler echocardiography.
The major clinical manifestations of LVNC are heart failure, cardiac arrhythmias, and cardioembolic events (2).
The clinical course of LVNC
is highly variable, ranging from the complete asymptomatic status to severe heart failure requiring heart transplantation.
This process is believed to be abruptly interrupted in LVNC
patients (3-4, 8).
is a rare congenital cardiomyopathy that appears to result from the intrauterine arrest of endomyocardial morphogenesis.
The test will identify a mutation in approximately 30% of patients with a high index of suspicion for LVNC