A rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis
https://doi.org/10.12737/article_5a3a0ef1814dc1.76335284
Abstract
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
About the Authors
O. S. Efremova
Irkutsk State Medical Academy of Postgraduate Education - Branch Campus of the Russian Medical Academy of Continuing Professional Education
Russian Federation
Russian Federation
L. V. Bregel
Irkutsk State Medical Academy of Postgraduate Education - Branch Campus of the Russian Medical Academy of Continuing Professional Education
Russian Federation
Russian Federation
V. M. Subbotin
Irkutsk State Medical Academy of Postgraduate Education - Branch Campus of the Russian Medical Academy of Continuing Professional Education
Russian Federation
Russian Federation
A. O. Barakin
Irkutsk State Regional Children’s Clinical Hospital
Russian Federation
Russian Federation
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Review
For citations:
Efremova O.S., Bregel L.V., Subbotin V.M., Barakin A.O. A rare combination of noncompact myocardium and coronary dilatation with type 1 neurofibromatosis. Acta Biomedica Scientifica. 2017;2(5(2)):159-162. (In Russ.) https://doi.org/10.12737/article_5a3a0ef1814dc1.76335284
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