Hospital pediatrics. Cardiomyopathies in children. Clinic, diagnosis, treatment (lecture notes)

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Hospital pediatrics. Cardiomyopathies in children. Clinic, diagnosis, treatment (lecture notes)

Lecture notes, cheat sheets

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LECTURE No. 3. Cardiomyopathies in children. Clinic, diagnosis, treatment

Classification of cardiomyopathies:

1) dilated (DCMP);

2) hypertrophic (HCMP);

3) restrictive (RCMP);

4) arrhythmogenic right ventricle (AKMP).

1. Dilated cardiomyopathy

Dilated cardiomyopathy is a sharp expansion of the ventricular cavity, especially the left one.

Clinical manifestations. At any age, in any gender (more often in men), signs of heart failure (up to total), decreased blood pressure, expansion of the boundaries of the heart, cardiomegaly. Auscultation: deafness of the first tone at the apex, bifurcation, gallop rhythm. Respiratory organs: tympanitis or dullness on the right, fine bubble moist rales on the left.

Diagnostics. ECG-tachycardia, arrhythmia, appearance of the R wave and ("-") T wave; FCG - I tone weakened, systolic, protodiastolic murmur; EchoCG - dilatation of all parts of the heart, EchoCG - dilatation of the left ventricular cavity (LV EDP = 56 mm), decreased myocardial contractility (ejection factor 0,34), EchoCG - symmetrical myocardial hypertrophy Tzsp = 28, mitral regurgitation.

Treatment.

Principles of treatment.

I. Conservative.

1. ACE inhibitors (capoten, enalapril, renitek).

2. Angiotensin-2 receptor blockers (Cozaan, Diovan).

3. Diuretics.

4. β-blockers (carvediol).

5. Antiaggregants, anticoagulants. II. Surgical.

2. Hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy is expressed by hypertrophy of the left ventricle and interventricular septum.

Clinical manifestations. More often in boys, there are familial forms, shortness of breath, pain in the heart, expansion of the borders of the heart, weakening of the first tone at the apex, emphasis of the second tone over the pulmonary artery, variable systolic-diastolic murmur along the left edge of the sternum, development of cardiovascular failure of the left ventricular type.

Diagnostics. ECG - signs of hypertrophy of the left atrium and left ventricle; the Q wave is changed in the hole, V4, EchoCG - thickening of the interventricular septum, decreased volume of the left ventricle. X-ray examination of the chest organs - depends on the size of the heart, forward displacement of the mitral valve.

Carnitine CMP.

Ventricular hypertrophy: EDP = 65 mm, atriomegaly, EF = 0,2, mitral regurgitation of 2-3 degrees. Treatment. Surgical - septal myectomy.

1. Valve prosthetics - two-chamber constant stimulation.

2. Conservative:

1) limitation of physical activity;

2) diet with restriction of salt and water;

3) β-blockers;

4) Ca-channel blockers;

5) antiarrhythmics of other groups;

6) ACE inhibitors.

Cardiac glycosides are contraindicated in HCM.

3. Restrictive cardiomyopathy

Restrictive cardiomyopathy is associated with a decrease in the distensibility of the ventricular walls with the manifestation of signs of hypodiastolia and symptoms of stagnation in the systemic and pulmonary circulation.

Primary myocardial (isolated myocardial damage, similar to DCM).

Endomyocardial (thickening of the endocardium and infiltrative necrotic and infiltrative changes in the myocardium).

1. Lefler's hypereosinophilic parietal fibroplastic endocarditis.

2. Endomyocardial fibrosis (Davis' disease). Stages.

I. Necrotic.

II. thrombotic.

III. Fibrotic: on the ECG - a decrease in the voltage of the teeth, a violation of the processes of conduction and excitation, a change in the final part of the ventricular complex.

Ultrasound reveals dilatation of the heart cavities and a decrease in myocardial contractility. An x-ray shows the enlarged size of the heart or its parts.

Arrhythmogenic right ventricular cardiomyopathy. Clinic: ventricular extrasystoles, paroxysmal tachycardia.

Cardiomyopathy in mitochondrial pathology. These include the following:

1) Kearns-Sayre syndrome;

2) MELAS syndrome;

3) MERRF syndrome;

4) Barth's syndrome;

5) carnitine CMP;

6) histiocytic cardiomyopathy;

7) CMP with a deficiency of the P-complex of the chain of respiratory enzymes.

Criteria for cardiomyopathy in mitochondrial pathology.

1. Extracardiac:

1) infantile somatotype (3-5 centile);

2) muscle weakness;

3) decreased tolerance to physical activity;

4) visual impairment (ptosis), hearing;

5) stroke-like episodes;

6) periodic neutropenia;

7) persistent enlargement of the liver;

8) high levels of lactate and pyruvate;

9) increased excretion of organic acids;

10) decrease in blood carnitine content - acidosis.

2. Cardiac:

1) disorders of the conduction system of the heart are malignant, ventricular arrhythmias are typical for young children;

2) a combination of HCM, DCM, fibroelastosis;

3) detection of HCM at an early age;

4) family nature of the disease;

5) giant T waves on the ECG in the left chest leads.

Kearns-Sayre syndrome. Debut by 20-30 years. Symptoms: CMP with the development of complete atrioventricular block, possible formation of HCPM and DCM, ophthalmoplegia with ptosis, retinopathy, delayed physical and sexual development, valgus deviation of the foot, cerebellar ataxia.

MELAS syndrome (mitochondrial myopathy-encephalopathy-lactic acidosis, stroke-like episodes). Debut between 6 and 10 years. Symptoms: convulsions, headache, vomiting, anorexia, dementia.

MERRF syndrome (myoclonus epilepsy and cerebral infarction, RRF fibers). Debut - from 3 to 63 years. Symptoms: myoclonus epilepsy, ataxia, dementia (due to multiple cerebral infarctions), hearing loss, muscle weakness, HCM.

Barth's syndrome. Debut at 5-7 months of life. Symptoms: weight and height at 3-5 centiles, growth retardation; bone age delay by 1-2 years; skeletal myopathy; neutropenia; HCM, DCM.

Carnitine CMP. Debut at 3-5 months. Often sudden death due to metabolic stress. Symptoms: myocardial hypertrophy with dilatation of the left ventricular cavity, endocardial fibroelastosis, ECG giant T waves (above R) in the left precordial leads.

Histiocytic CMP (cytochrome-B deficiency). Debut - at 3 weeks - 1 year. More often in girls. Despite treatment, they die. Symptoms: tachyarrhythmias, ventricular fibrillation, myocardial hypertrophy with dilatation of the left ventricular cavity, cardiac fibroelastosis.

CMP with deficiency of the P-complex of the respiratory enzyme chain. Debut - after 9 years. Symptoms: ophthalmoplegia, encephalomyopathy, ataxia, myoclonic jerks, secondary carnitine deficiency, lactic acidosis, HCM, DCM, ACM.

Author: Pavlova N.V.

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