Purpose: to study clinical variants, follow-up and outcomes of the left ventricular noncompaction (LVN) syndrome by adult patients.
Methods: The diagnosis of LVN was established in 45 adults (21 females, 18-76 years, on the average 42.7?15.3) on the basis of visual criteria, in 9 patients using three methods (Echo-CG, MRI, CT), in 28 patients using any two methods. Were also performed endomyocardial/intraoperative myocardial biopsy and autopsy (n=11) with viral DNA investigation (real-time PCR), anti-heart antibody investigation. The mean follow-up was 12 [5.0; 28.5] months, from 3 month to 12 years.
Results: there was a high frequency of the association of LVN syndrome and other heart disease by adults: 6 patients (13%) had congenital heart disease (atrial and ventricular septal defects, pulmonary artery stenosis, bicuspid aortic valve, vascular malformations), 3 (6%) – hypertrophic cardiomyopathy, 2 (4%) – arrhythmogenic right ventricular cardiomyopathy, 1 (2%) – genetic myodystrophy with cardiac involvement, 1 (2%) – restrictive cardiomyopathy, 1 (2%) – Danon disease. Only one patient with LVN syndrome had no symptoms (but dilated cardiomyopathy, DCM, by Echo-CG). In 10 patients (23%) LVN had “idiopathic” arrhythmias mask, in 15 patients (35%) – mask of DCM, in 4 patients (9%) LVN was first identified at the same time with acute myocarditis. In 5 cases (11%) LVN was associated with ischemic heart disease, but in 2 patients (11%) the initial diagnosis “ischemic heart disease” was false. The associated myocarditis was in 26 patients (58%) detected, incl. 11 patients by morphologic study of myocardium. The virus was found in 6 patients in the myocardium (parvovirus B19 in 5, human herpes virus type 6 in 2, herpes simplex virus type 1, 2 in 1 and Ebstein-Barr virus in 1) and in 11 patients in the blood. Heart failure was in 36 patients (80%) and angina pectoris in 10 patients (22%) diagnosed; 6 patients had a typical signs of the myocardium infarct due to coronary artery atherosclerosis, necrosis in myocarditis, embolism from left ventricle. The incidence of atrial fibrillation was 24% (11 patients), stable and unstable ventricular tachycardia – 62% (28 patients), left bundle branch block – 16% (7 patients). The average LV diastolic size was 6.1±0.9 cm, LV ejection fraction 35.4±15.0%. Eleven patients (24%) without anticoagulants had intracardiac thrombi; 10 of them had LV ejection fraction less than 40%. Three patients (7%) had embolism (renal, pulmonary, myocardium infarct, stroke). The devises was in 18% patients (40%) implanted: CRT in 1 (2%), ICD in 11 (24%), pacemakers in 3 (7%). In two patients was cardiac transplantation performed. The mortality was 11.1% (5 patients).
Conclusions: LVN syndrome in adults is vary polymorphic and often associated with other cardiomyopathy (18%) and myocarditis (58%). The frequency of complications (incl. stable ventricular tachycardia, embolism) is high, that requires development of differential approaches to the treatment.