Correlation of Non-Compact Myocardial Layer Characteristics With the Risk of Death and Thrombotic Complications in Dilated Phenotype of Non-Compact Myocardium in Children: Cohort Study Results

The classifications of the World Health Organization, the European Society of Car-diology and the American Heart Association indicate the existence of several phenotypes of myocardial non-compaction (MnC) with specific structural and functional abnormalities. The MnC+dilated cardiomyopathy (DCM) phenotype is considered one of the most severe variants. Disputes continue about whether to regard MnC as an independent disease or as a consequence of DCM and heart failure. In other words, MnC remains one of the most mysterious heart diseases. As an illustration of MnC+DCM phenotype, the authors offer a case of a patient with cardiovascular disease from her youth, but maintained a satisfactory state of health and performance until her old age. Symptoms of arrhythmia and heart failure with massive pericardial effusion were first described in her at the age of 66, which is uncharacteristic for this MnC phenotype. Attention is drawn to the difficulties of differential diagnosis of MnC due to the non-specificity of clinical performance, the role of echocardiography in the recognition of the disease and predictors of its unfavorable outcome. The fact that the patient, even when typical signs of MnC were detected during echocardiography, initially had coronary artery disease as the main diagnosis, indicates the relevance of publishing another case report on this rare pathology in order to improve the awareness of cardiologists and general practitioners.

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Primary diagnosis of dilated cardiomyopathy in combination with myocardial non-compaction in an elderly patient: a case report

Vakhnenko

Bagdasaryan

Savchenko

2023

Russ J Cardiol

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The Frequency of Thrombotic Complications and Features of Genotypes of Polymorphic Markers of Hemostasis Genes in Children With Noncompact Cardiomyopathy

Басаргина

Umarova

Savostyanov

et al. 2019

jour

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Noncompaction cardiomyopathy (NCMP) is characterized by the anomalous myocardium structure and various types of cardiac remodeling, in some cases it is accompanied by thrombotic complications. Preconditions for thrombosis in the disease are unknown, as also there are differences in thrombosis rates between NCMP and other cardiomyopathies, similarly accompanied by the chronic heart failure and analogous remodeling phenotypes. Aim of study is to reveal the difference in the rate of thrombosis in NCMP and dilated cardiomyopathies (DCMP) in children, and to define differences in the frequency of different genotypes of polymorphic markers in an array of hemostasis genes in the two cardiomyopathies. Methods. There was executed a prospective-retrospective cohort study, included patients from the Cardiac Department of the National Scientific and Practical Center of Children's Health from October 2011 to May 2015. The presence of NCMP was established by echocardiography, alleles and genotypes of polymorphic markers of hemostasis and folate cycle genes were determined by polymerase chain reaction in real-time mode. Results. Thrombotic complications in NCMP children were observed more often than in DCMP cases. There were no differences between NCMP and DCMC patients in the frequency of the polymorphic markers c.1691G>A of the F5 gene (p=0.61) , c.20210G>A of the F2 gene (p=1.0) , c.1565T> C of the ITGB3 gene (p=0.32) , 5G(-675)4G of PLANH1 gene (p=0,52) , G(-455)A of FGB gene (p=0.82) , c.677C>T of MTHFR gene (p=0.11). Conclusion Thrombotic complications in NCMP children occur rather more often than in DCMP cases, studied polymorphic markers of the hemostasis and folate cycle genes do not cause this difference, and this requires continuation of the study.

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The Prevalence of Thrombotic Events in Children With Heart Failure on the Background of the Dilated Cardiomyopathy Phenotype

Derevnina

Басаргина

Savostyanov

et al. 2019

jour

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Thrombotic events seem to be one of the most common and severe complications having a direct impact on the course of the disease in patients with cardiomyopathy.There were examined 94 children with dilated cardiomyopathy (DCMP) phenotype [49 children with dilated cardiomyopathy (DCMP), including 45 patients with non-compaction cardiomyopathy (NCMP) and remodeling in dilated phenotype]. Thromboses were diagnosed in 9 patients, including 7 DCMP and 2 NCMP cases. In 4 DCMP children, the thrombus was localized in the cavity of the left ventricle, one in the left atrium, the right ventricle, and the inferior vena cava. In NCMP children, intracardiac thrombus formation was not determined, one patient was diagnosed with an acute ischemic disorder of the cerebral circulation; in the second one, the thrombus was detected in the superior vena cava. Thrombosis in DCMP patients was detected against a background of a severe systolic dysfunction of the left ventricle (LVEF of below 30%), and in NCMP children with a moderate dysfunction. Also, the greatest prevalence rate of thrombotic complications was noted in Functional Class III and IV heart failure cases. At the same time, there was no established any influence of polymorphic markers G1691A of gene F5, G20210A of gene F2, C677T of MTHFR gene on the prevalence of thrombotic events. The authors believe the formation of thrombi with the severe LV dysfunction in children with cMYP should be taken into account in the determination the tactics of the treatment of such patients, as it is necessary to make a decision about administering antithrombotic therapy.

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Correlation of Non-Compact Myocardial Layer Characteristics With the Risk of Death and Thrombotic Complications in Dilated Phenotype of Non-Compact Myocardium in Children: Cohort Study Results

Cited by 3 publications

References 24 publications

Primary diagnosis of dilated cardiomyopathy in combination with myocardial non-compaction in an elderly patient: a case report

Primary diagnosis of dilated cardiomyopathy in combination with myocardial non-compaction in an elderly patient: a case report

The Frequency of Thrombotic Complications and Features of Genotypes of Polymorphic Markers of Hemostasis Genes in Children With Noncompact Cardiomyopathy

The Prevalence of Thrombotic Events in Children With Heart Failure on the Background of the Dilated Cardiomyopathy Phenotype

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