A RARE COMBINATION OF NONCOMPACT MYOCARDIUM AND CORONARY DILATATION WITH TYPE 1 NEUROFIBROMATOSIS
Rubrics: CASE REPORT
Authors:
1.
Irkutsk, Irkutsk region, Russian Federation
2.
Irkutsk State Medical Academy of Postgraduate Education
( Head of the Department of Pediatrics)
Irkutsk, Irkutsk region, Russian Federation
Irkutsk, Irkutsk region, Russian Federation
3.
Irkutsk State Medical Academy of Postgraduate Education
( Teaching Assistant at the Department of Pediatrics)
Irkutsk, Irkutsk region, Russian Federation
Irkutsk, Irkutsk region, Russian Federation
Type:
Article
Pages:
from 159
to 162
Status:
Published
Received:
20.12.2017
Accepted:
07.01.2023
Published:
18.01.2018
Subject area:
UDK 61 Медицина. Охрана здоровья
Language:
Russian, English
Keywords:
noncompact left ventricular myocardium, cardiomyopathy, type 1 neurofibromatosis, coronary dilatation
Abstract (English):
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 (NF 1). The first of these complications, undifferentiated cardio- myopathy, 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 neurofibro- matosis, 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
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 (NF 1). The first of these complications, undifferentiated cardio- myopathy, 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 neurofibro- matosis, 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
Keywords:
noncompact left ventricular myocardium, cardiomyopathy, type 1 neurofibromatosis, coronary dilatation
noncompact left ventricular myocardium, cardiomyopathy, type 1 neurofibromatosis, coronary dilatation
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