Dilated cardiomyopathy in children

Dilated cardiomyopathy is a disease of the myocardium, characterized by a sharp expansion of the heart cavities, a decrease in the contractile function of the myocardium, the development of congestive heart failure, often refractory to the treatment and poor prognosis.

ICD-10 code

142.0 Dilated cardiomyopathy.

Epidemiology

Dilated cardiomyopathy in children is one of the most common clinical forms of cardio-myopathies, it is met in most countries of the world and at any age. The true frequency of dilated cardiomyopathy in children is unknown in connection with the lack of unified diagnostic criteria for the disease. According to various authors, the incidence among children is 5-10 cases per 100 000 population. Almost all studies note a predominance of male patients (62-88%).

Causes and pathogenesis of dilated cardiomyopathy

Various hypotheses of the origin of dilated cardiomyopathy have been put forward, nevertheless, in recent years, the opinion about the multifactorial genesis of the disease has been increasingly expressed.

At the heart of the development of dilated cardiomyopathy is the disruption of the systolic and diastolic functions of the myocardium, followed by dilatation of the heart cavities, caused by damage to cardiomyocytes and the formation of replacement fibrosis under the influence of various factors (toxic substances, pathogenic viruses, inflammatory cells, autoantibodies, etc.).

Causes and pathogenesis of dilated cardiomyopathy

Symptoms of dilated cardiomyopathy

The clinical picture of dilated cardiomyopathy is variable and depends mainly on the severity of circulatory insufficiency. In the early stages of the disease, the disease is small or asymptomatic, subjective manifestations are often absent, children do not complain. Cardiomegaly, changes on the ECG are often detected accidentally during preventive examinations or when contacting a doctor for another reason. This explains the later detection of pathology.

Symptoms of dilated cardiomyopathy

Diagnosis of dilated cardiomyopathy

Diagnosis of dilated cardiomyopathy is difficult, since the disease does not have specific criteria. The final diagnosis of dilated cardiomyopathy is established by the elimination of all diseases that can lead to increased heart cavities and circulatory insufficiency. The most important element of the clinical picture in patients with dilated cardiomyopathy is episodes of embolism, which often lead patients to death.

The survey plan is as follows.

• Collection of anamnesis of life, family history, anamnesis of the disease.
• Clinical examination.
• Laboratory research.
• Instrumental studies (Echocardiography, ECG, Holter monitoring, chest radiography, ultrasound [ultrasound] of the abdominal organs and kidneys).

Diagnosis of dilated cardiomyopathy

Treatment of dilated cardiomyopathy

Along with innovations in the pathogenesis of dilated cardiomyopathy, the last decade has been marked by the appearance of new views on her therapy, but until now the treatment remains mostly symptomatic. Therapy is based on the correction and prevention of the main clinical manifestations of the disease and its complications: chronic heart failure, heart rhythm disorders and thromboembolism.

Treatment of dilated cardiomyopathy

Prognosis for dilated cardiomyopathy

The prognosis of the disease is very serious, although there are some reports of a significant improvement in the clinical state of patients on the background of conventional therapy.

The criteria for the prognosis include the duration of the disease after diagnosis, clinical symptoms and severity of heart failure, the presence of low-voltage type of electrocardiogram. Ventricular arrhythmias of high grades, the degree of reduction of the contractile and pumping functions of the heart. The average life expectancy of patients with dilated cardiomyopathy is 3.5-5 years. The opinions of different authors differ when studying the outcome of dilated cardiomyopathy in children. The highest survival rate was observed in young children.

[1], [2], [3], [4], [5], [6], [7], [8]