Abstract
Introduction. Left ventricular non-compaction (LVNC) is a rare form of cardiomyopathy, congenital defect of the intrauterine process of ventricular embryogenesis. The main feature of this pathology is an existence of prominent trabeculations in the left ventricle and deep intertrabecular recesses. The pathological structure of the myocardium leads to its dilatation and dysfunction, heart failure symptoms, embolic episodes and arrhythmia. The aim of the manuscript is to present a spectrum of clinical manifestations of left ventricular non-compaction (LVNC). Materials and methods Patients were diagnosed and treated in The Lviv Centre of Cardiology. Two of them were admitted for heart failure symptoms, in one patient with dysrhythmia and left bundle brunch block (LBBB); and one patient had non-specific chest pain. Cardiac imaging with echocardiography and MRI were performed. Results. Echocardiography revealed criteria of LVNC in all patient, in two of them the diagnosis was confirmed by cardiac MRI. Patients with heart failure symptoms were managed with optimal medical therapy, CRT-D has been implanted in patient with LBBB. Asymptomatic patient is under follow-up. Conclusions. LVNC is a cardiomyopathy with distinct echocardiographic and MRI-features and wide spectrum of clinical presentations. The prognosis in case of LVNC is serious due to systolic LV dysfunction, cardiac arrhythmia and embolic events. Echocardiography is a diagnostic tool to identify LVNC, cardiac MRI confirms the diagnosis of LVNC if echocardiography data is insufficient.
Highlights
Left ventricular non-compaction (LVNC) is a rare form of congenital cardiomyopathy due to a defect of the intrauterine process of ventricular embryogenesis [4]
The abnormal structure of the myocardium leads to its dilatation and dysfunction, heart failure symptoms, embolic episodes and arrhythmia
We present a series of cases of LVNC, illustrated with images from echocardiography and cardiac MRI
Summary
Left ventricular non-compaction (LVNC) is a rare form of congenital cardiomyopathy due to a defect of the intrauterine process of ventricular embryogenesis [4]. The exact frequency of LVNC remains unknown, because of uncertainty of diagnostic criteria, but multiple authors report, that this diagnosis is made in 0,014% - 0,26% of patients, who underwent echocardiographic examination [9, 13, 23]. The pathological LV structure leads to dilatation and dysfunction of the myocardium, and to heart failure symptoms, which remain the most frequent symptoms in patients with LVNC [9, 13, 14, 15, 17]. Echocardiographic assessment can lead both to underestimation and to overestimation of LVNC syndrome [22] The latter occurs if numerous left ventricular trabeculations close to the apex, which do not meet the criteria but are considered as LVNC symptoms. The aim of the manuscript is to present a spectrum of clinical manifestations of left ventricular non-compaction (LVNC)
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