Ultrasound diagnostics of left ventricular noncompaction

The clinical cases of rare, genetically heterogeneous cardiomyopathy are described - the left ventricular noncompaction (LVNC) cardiomyopathy for four children (three boys and one girl) in age from two months to 6 years. Description of echocardiography signs is presented in accordance with modern di...

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Autores principales:	A. O. Barakin, L. V. Bregel, V. M. Subbotin, O. S. Efremova
Formato:	article
Lenguaje:	RU
Publicado:	Scientific Сentre for Family Health and Human Reproduction Problems 2017
Materias:	noncompaction of the left ventricular myocardium ultrasound diagnosis echocardiography cardiomyopathy children Science Q
Acceso en línea:	https://doaj.org/article/f299cc8f4d88448e99b1ce74073de78e
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spelling	oai:doaj.org-article:f299cc8f4d88448e99b1ce74073de78e2021-11-23T06:14:38ZUltrasound diagnostics of left ventricular noncompaction2541-94202587-959610.12737/article_59e8bc42c0bf14.47394265https://doaj.org/article/f299cc8f4d88448e99b1ce74073de78e2017-09-01T00:00:00Zhttps://www.actabiomedica.ru/jour/article/view/454https://doaj.org/toc/2541-9420https://doaj.org/toc/2587-9596The clinical cases of rare, genetically heterogeneous cardiomyopathy are described - the left ventricular noncompaction (LVNC) cardiomyopathy for four children (three boys and one girl) in age from two months to 6 years. Description of echocardiography signs is presented in accordance with modern diagnostic criteria. Noncompaction of the left ventricle is recognized in three patients, of the left and right ventricle - in one. In three patients out of four, LVNC was a separate disease, and yet in one it was associated with neurofibromatosis type 1, in combination with dilatation of coronary arteries and pericardial effusion. Three of four patients were admitted with congestive heart failure (CHF) of New York Heart Association (NYHA) functional class 3-4 (FC), and one - with CHF 2 FC. The echocardiograms are brought with description of characteristic features of LVNC (hypertrabecularity, spongy myocardium on the apex of the left ventricle, typical correlation of incompact and compact layer from 2.0 to 2.6). All four patients received treatment for CHF (diuretics, beta-blockers, captopril, in one case - digoxin); two out of four got antithrombotic therapy (aspirin, warfarin). Results of follow-up observation of the patients are described with the estimation of echocardiography signs, including dilatation of coronary arteries. Left ventricular ejection fraction - both by Simpson and Teicholz - increased in two patients, with therapy of chronic heart failure at a follow-up visits over > 5 years simultaneously with the decline of severity of CHF. Another two patients have been examined from 2 months to 5 years and have had CHF 3 FC; their left ventricular ejection fraction increased too, but cardiomegaly and right ventricular heart failure preserved.A. O. BarakinL. V. BregelV. M. SubbotinO. S. EfremovaScientific Сentre for Family Health and Human Reproduction Problemsarticlenoncompaction of the left ventricular myocardiumultrasound diagnosisechocardiographycardiomyopathychildrennoncompaction of the left ventricular myocardiumultrasound diagnosisechocardiographycardiomyopathychildrenScienceQRUActa Biomedica Scientifica, Vol 2, Iss 5(1), Pp 186-192 (2017)
institution	DOAJ
collection	DOAJ
language	RU
topic	noncompaction of the left ventricular myocardium ultrasound diagnosis echocardiography cardiomyopathy children noncompaction of the left ventricular myocardium ultrasound diagnosis echocardiography cardiomyopathy children Science Q
spellingShingle	noncompaction of the left ventricular myocardium ultrasound diagnosis echocardiography cardiomyopathy children noncompaction of the left ventricular myocardium ultrasound diagnosis echocardiography cardiomyopathy children Science Q A. O. Barakin L. V. Bregel V. M. Subbotin O. S. Efremova Ultrasound diagnostics of left ventricular noncompaction
description	The clinical cases of rare, genetically heterogeneous cardiomyopathy are described - the left ventricular noncompaction (LVNC) cardiomyopathy for four children (three boys and one girl) in age from two months to 6 years. Description of echocardiography signs is presented in accordance with modern diagnostic criteria. Noncompaction of the left ventricle is recognized in three patients, of the left and right ventricle - in one. In three patients out of four, LVNC was a separate disease, and yet in one it was associated with neurofibromatosis type 1, in combination with dilatation of coronary arteries and pericardial effusion. Three of four patients were admitted with congestive heart failure (CHF) of New York Heart Association (NYHA) functional class 3-4 (FC), and one - with CHF 2 FC. The echocardiograms are brought with description of characteristic features of LVNC (hypertrabecularity, spongy myocardium on the apex of the left ventricle, typical correlation of incompact and compact layer from 2.0 to 2.6). All four patients received treatment for CHF (diuretics, beta-blockers, captopril, in one case - digoxin); two out of four got antithrombotic therapy (aspirin, warfarin). Results of follow-up observation of the patients are described with the estimation of echocardiography signs, including dilatation of coronary arteries. Left ventricular ejection fraction - both by Simpson and Teicholz - increased in two patients, with therapy of chronic heart failure at a follow-up visits over > 5 years simultaneously with the decline of severity of CHF. Another two patients have been examined from 2 months to 5 years and have had CHF 3 FC; their left ventricular ejection fraction increased too, but cardiomegaly and right ventricular heart failure preserved.
format	article
author	A. O. Barakin L. V. Bregel V. M. Subbotin O. S. Efremova
author_facet	A. O. Barakin L. V. Bregel V. M. Subbotin O. S. Efremova
author_sort	A. O. Barakin
title	Ultrasound diagnostics of left ventricular noncompaction
title_short	Ultrasound diagnostics of left ventricular noncompaction
title_full	Ultrasound diagnostics of left ventricular noncompaction
title_fullStr	Ultrasound diagnostics of left ventricular noncompaction
title_full_unstemmed	Ultrasound diagnostics of left ventricular noncompaction
title_sort	ultrasound diagnostics of left ventricular noncompaction
publisher	Scientific Сentre for Family Health and Human Reproduction Problems
publishDate	2017
url	https://doaj.org/article/f299cc8f4d88448e99b1ce74073de78e
work_keys_str_mv	AT aobarakin ultrasounddiagnosticsofleftventricularnoncompaction AT lvbregel ultrasounddiagnosticsofleftventricularnoncompaction AT vmsubbotin ultrasounddiagnosticsofleftventricularnoncompaction AT osefremova ultrasounddiagnosticsofleftventricularnoncompaction
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Ultrasound diagnostics of left ventricular noncompaction

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