Редкое сочетание некомпактного миокарда и коронарной дилатации с нейрофиброматозом 1-го типа

Acta Biomedica Scientifica

А. О. Баракин¹  В. М. Субботин²  Л. В. Брегель³  О. С. Ефремова³ 

[1] ГБУЗ «Иркутская государственная областная детская клиническая больница»;Иркутская государственная медицинская академия последипломного образования - филиал ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования»;Иркутская государственная медицинская академия последипломного образования - филиал ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава России

关键词: некомпактный миокард левого желудочка;    кардиомиопатия;    нейрофиброматоз 1-го типа;    коронарная дилатация;   

DOI  :  10.12737/article_5a3a0ef1814dc1.76335284

学科分类：自然科学（综合）

来源: Scientific entre for Family Health and Human Reproduction Problems

【 摘 要 】

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.

【 授权许可】

CC BY