title: [Noncompaction myocardium as a primary phenomenon or consequence of myocardial dysfunction: clinical masks of the syndrome].
abstract: Noncompaction myocardium (NCM) is a genetic heterogeneous primary cardiomyopathy which affects both children and adults and can be either isolated or combined with other congenital heart disorders. It has common pathogenesis of symptoms but is distinguished by pronounced clinical polymorphism. We have observed 25 adult patients (15 men, 10 women aged from 20 to 62 years, mean age 42.9+/-13.3 years) with NCM syndrome. Heart failure have been found in 96% of patients (functional class [FC] I in 7, II - in 6, III in 7, and IV - in 4 patients). Ninety two percent of patients have ventricular extrasystoles, 32% - atrial fibrillation, 28% - FC I-III angina. Mean end diastolic left ventricular dimension is 6.5+/-0.8cm, ejection fraction 29.7+/-13.0%, mean pulmonary artery pressure - 42.6+/-13.5 mm Hg. Intracardiac thrombosis have been found in 24% of patients. In 7 patients morphological study of myocardium has been performed. NCM syndrome was diagnosed at initial investigation just in 1 case. We distinguished the following clinical masks (variants of diagnosis) of NCM: 1) clinically not manifest, is revealed at accidental examination (4%); 2) exists under mask of "idiopathic" rhythm disturbances (8%); 3) has a mask of ischemic heart disease; 4) is revealed in patients with acute or subacute myocarditis (12%); 5) has a mask of dilated cardiomyopathy (52%); 6) NCM in patients with other primary cardiomyopathies (hypertrophic, restrictive, genetic myopathy, arrhythmogenic right ventricular dysplasia). Combination of NCM with congenital heart defects has been found in 20% of patients. In 56% of cases myocarditis was diagnosed  (it was viral in no less than 44%). Only in 32% of patients it is possible to consider presence of isolated NCM syndrome. This paper contains discussion of problems of diagnostics (including morphological) and treatment in the presented group of patients, significance of myocarditis for development of decompensation, role of NCM in patients with other primary cardiomyopathies, possibility of compensatory (secondary) character of NCM in severe systolic dysfunction.
authors.forename: O V
authors.initials: OV
authors.lastname: Blagova
authors.forename: A V
authors.initials: AV
authors.lastname: Nedostup
authors.forename: V P
authors.initials: VP
authors.lastname: Sedov
authors.forename: N V
authors.initials: NV
authors.lastname: Gagarina
authors.forename: E A
authors.initials: EA
authors.lastname: Kogan
authors.forename: V A
authors.initials: VA
authors.lastname: Sulimov
authors.forename: Iu V
authors.initials: IuV
authors.lastname: Frolova
authors.forename: S L
authors.initials: SL
authors.lastname: Dzemeshkevich
authors.forename: E V
authors.initials: EV
authors.lastname: Zakliaz'minskaia
authors.forename: E A
authors.initials: EA
authors.lastname: Mershina
authors.forename: V E
authors.initials: VE
authors.lastname: Sinitsyn
authors.forename: A G
authors.initials: AG
authors.lastname: Kupriianova
authors.forename: V A
authors.initials: VA
authors.lastname: Zaĭdenov
authors.forename: A E
authors.initials: AE
authors.lastname: Donnikov
mesh_terms: D000328:Adult; D001145:Arrhythmias, Cardiac / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D001706:Biopsy; D009202:Cardiomyopathies / Q000145:classification / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D002317:Cardiovascular Agents / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D019468:Disease Management; D004562:Electrocardiography; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000175:diagnosis; D006333:Heart Failure / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D006334:Heart Function Tests / Q000379:methods*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D009205:Myocarditis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D009206:Myocardium / Q000473:pathology*; D011379:Prognosis; D013577:Syndrome; D036542:Tomography, Spiral Computed / Q000379:methods*; D016896:Treatment Outcome
chemical_list: D002317:Cardiovascular Agents