A rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis

The article describes the observation of a child with a very rare combination of noncompact left ventricular myocardium and coronary dilatation in type 1 neurofibromatosis (NF1). The first of these complications, undifferentiated cardiomyopathy, also known as noncompact myocardium, is not described...

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Autores principales:	O. S. Efremova, L. V. Bregel, V. M. Subbotin, A. O. Barakin
Formato:	article
Lenguaje:	RU
Publicado:	Scientific Сentre for Family Health and Human Reproduction Problems 2017
Materias:	noncompact left ventricular myocardium cardiomyopathy type 1 neurofibromatosis coronary dilatation Science Q
Acceso en línea:	https://doaj.org/article/019ec8c958554fe98503a8da51e2398e
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spelling	oai:doaj.org-article:019ec8c958554fe98503a8da51e2398e2021-11-23T06:14:39ZA rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis2541-94202587-959610.12737/article_5a3a0ef1814dc1.76335284https://doaj.org/article/019ec8c958554fe98503a8da51e2398e2017-09-01T00:00:00Zhttps://www.actabiomedica.ru/jour/article/view/527https://doaj.org/toc/2541-9420https://doaj.org/toc/2587-9596The article describes the observation of a child with a very rare combination of noncompact left ventricular myocardium and coronary dilatation in type 1 neurofibromatosis (NF1). The first of these complications, undifferentiated cardiomyopathy, also known as noncompact myocardium, is not described in combination with nF 1 in available literature, although cases of hypertrophic cardiomyopathy in patients with NF 1 have been previously reported. Dilatation of the coronary arteries also refers to infrequent manifestations of vascular pathology against the background of neurofibromatosis, and the combination of all three signs (NF 1, noncompact myocardium and coronary dilatation) has not yet been reported by other authors. When examining the patient, we found the characteristic signs of NF 1 (foci of "coffee" pigmentation on the skin, multiple nevuses, fibroids of the forearm, cognitive disorders), electrocardiogram features of left ventricular hypertrophy, negative T-tooth and ST-segment displacement 1 mm below the isoline in Leads V4-6, in echocardiography - pronounced trabecularity, thinning of the compact myocardium layer and a "spongy myocardium" in the region of the left ventricle apex, dilatation of the coronary arteries. With magnetic resonance imaging, signs of noncompact myocardium of the left ventricle were found, with selective coronary angiography - slowing of the coronary blood flow. The patient receives the permanent treatment for heart failure and aspirin, his condition remains stable for 4 years of follow-upO. S. EfremovaL. V. BregelV. M. SubbotinA. O. BarakinScientific Сentre for Family Health and Human Reproduction Problemsarticlenoncompact left ventricular myocardiumcardiomyopathytype 1 neurofibromatosiscoronary dilatationScienceQRUActa Biomedica Scientifica, Vol 2, Iss 5(2), Pp 159-162 (2017)
institution	DOAJ
collection	DOAJ
language	RU
topic	noncompact left ventricular myocardium cardiomyopathy type 1 neurofibromatosis coronary dilatation Science Q
spellingShingle	noncompact left ventricular myocardium cardiomyopathy type 1 neurofibromatosis coronary dilatation Science Q O. S. Efremova L. V. Bregel V. M. Subbotin A. O. Barakin A rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis
description	The article describes the observation of a child with a very rare combination of noncompact left ventricular myocardium and coronary dilatation in type 1 neurofibromatosis (NF1). The first of these complications, undifferentiated cardiomyopathy, also known as noncompact myocardium, is not described in combination with nF 1 in available literature, although cases of hypertrophic cardiomyopathy in patients with NF 1 have been previously reported. Dilatation of the coronary arteries also refers to infrequent manifestations of vascular pathology against the background of neurofibromatosis, and the combination of all three signs (NF 1, noncompact myocardium and coronary dilatation) has not yet been reported by other authors. When examining the patient, we found the characteristic signs of NF 1 (foci of "coffee" pigmentation on the skin, multiple nevuses, fibroids of the forearm, cognitive disorders), electrocardiogram features of left ventricular hypertrophy, negative T-tooth and ST-segment displacement 1 mm below the isoline in Leads V4-6, in echocardiography - pronounced trabecularity, thinning of the compact myocardium layer and a "spongy myocardium" in the region of the left ventricle apex, dilatation of the coronary arteries. With magnetic resonance imaging, signs of noncompact myocardium of the left ventricle were found, with selective coronary angiography - slowing of the coronary blood flow. The patient receives the permanent treatment for heart failure and aspirin, his condition remains stable for 4 years of follow-up
format	article
author	O. S. Efremova L. V. Bregel V. M. Subbotin A. O. Barakin
author_facet	O. S. Efremova L. V. Bregel V. M. Subbotin A. O. Barakin
author_sort	O. S. Efremova
title	A rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis
title_short	A rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis
title_full	A rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis
title_fullStr	A rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis
title_full_unstemmed	A rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis
title_sort	rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis
publisher	Scientific Сentre for Family Health and Human Reproduction Problems
publishDate	2017
url	https://doaj.org/article/019ec8c958554fe98503a8da51e2398e
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A rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis

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