Pediatric restrictive cardiomyopathy

Introduction to restrictive cardiomyopathy in children Restrictive cardiomyopathy (RCM), also known as occlusive cardiomyopathy, is rare. Due to endocardial and/or cardiomyopathy, ventricular dilatation is reduced, diastolic filling is impaired, and heart failure occurs. Restrictive cardiomyopathy is characterized by endocardial and subendocardial fibrosis and hyperplasia, wall thrombosis, narrowing or occlusion of the heart chamber, decreased ventricular filling disorder and compliance, severely impaired diastolic function and normal systolic function or Cardiomyopathy characterized by mild damage. basic knowledge The proportion of illness: the probability of illness in infants and young children is 0.004% Susceptible people: children Mode of infection: non-infectious Complications: heart failure, pulmonary hypertension, ascites, pericardial effusion, arrhythmia

Etiology of restrictive cardiomyopathy in children

Causes:

The etiology of this disease is unknown, can be secondary to systemic amyloidosis, sarcoma-like disease, mucopolysaccharidosis, pigmentation, etc., causing myocardial invasive lesions, as well as endocardial myocardial fibrosis, eosinophilic syndrome, Carcinoid syndrome, cancer metastasis, radiation damage, etc., cause endocardial and myocardial lesions, some of the primary RCM are family-onset, autosomal dominant, RCM with atrioventricular block, and involving skeletal muscle In Europe, amyloidosis RCM is more common. In Africa, South America and parts of Asia, endocardial myocardial fibrosis is more common. RCM heart size is mostly normal or slightly enlarged, and early contraction function is normal due to endocardium. Myocardial lesions, stiff wall, affecting diastolic ventricular filling, left and right ventricular involvement, most of the right ventricle is heavier, so right and left heart failure occurs. This section discusses endocardial myocardial fibrosis.

Pathogenesis:

Currently limited cardiomyopathy can be divided into two categories:

1. Myocardial type: Myocardial type includes idiopathic restricted cardiomyopathy, myocardial hemochromatosis, elastin pseudo-yellow tumor, Fabry disease, and the like.

2. Endocardial myocardium: Endocardial myocardium includes endocardial myocardial fibrosis, eosinophilic endocardial myocardial syndrome (Löffler disease), etc., mainly in endocardial myocardium in childhood, see also There is a myocardial type, the etiology of endocardial myocardium is unknown, may be due to inflammation caused by viral or parasitic infection of endocardial and subendocardial myocardium, and then fibrosis, which in recent years is considered to be degranulation Due to the immune response of acid cells, the pathology of endocardial myocardial fibrosis is diffuse fibrosis of the intraventricular membrane, and can also invade the inner lining of the myocardium. The lesion may involve the bilateral ventricle or only the left or right ventricle. Right ventricular involvement is more common, papillary muscles, chordae and atrioventricular valve thickening, shortening, can cause atrioventricular valve insufficiency, endocardial myocardial fibrosis narrows the heart chamber, may have a wall thrombus, eosinophilic The pathology of endomyocardial syndrome is acute inflammatory reaction of eosinophil infiltration in the early endocardium, late endocardial collagen fibrosis, and most of the lesions are right heart. The pathophysiology of restrictive cardiomyopathy is mainly thickening. Endocardium and heart Myocardial fibrosis, ventricular diastolic filling the lower membrane barriers and decreased ventricular compliance, restricted Rhodobryum, reduced cardiac output, cause hemodynamic changes similar constrictive pericarditis.

Pediatric restricted cardiomyopathy prevention

At present, the etiology and pathogenesis of this disease are still unclear, and infections (viruses and parasites), malnutrition, etc. should be actively prevented to prevent excessive intake of foods rich in serotonin. The three principles that should be followed in scientific exercise are comprehensive exercise, gradual progress, and perseverance. Comprehensive exercise means that every part of the body and each system is exercised as much as possible; the second is to broaden the practice items and forms as much as possible in order to develop various physical qualities.

Pediatric restricted cardiomyopathy complications Complications, heart failure, pulmonary hypertension, ascites, pericardial effusion, arrhythmia

Concurrent heart failure, pulmonary hypertension, ascites, embolism, pericardial effusion, arrhythmia, etc.

1. Heart failure is a group of syndromes caused by various cardiac structural or functional diseases leading to ventricular filling and/or impaired ejection ability.

2. Pulmonary hypertension: a group of clinical pathophysiological syndromes with a mean pulmonary artery pressure of 25 mmHg measured by right heart catheter at rest in various causes. Pulmonary hypertension can exist independently as a disease, and more common is the pathophysiological manifestations of many diseases progressing to a certain stage.

Symptoms of restrictive cardiomyopathy in children Common symptoms Syringacic murmurs, pulsation, dyspnea, pericardial effusion, pulse pressure, small hair, right ventricular hypertrophy, heart failure, pulmonary congestion, mobile dullness

The disease is common in children and adolescents, the onset is mild, manifested as unexplained heart failure, clinical findings vary with the ventricle and the extent of the lesion, right heart lesions are mainly characterized by elevated venous pressure, jugular vein engorgement, Liver, ascites and lower extremity edema, similar to constrictive pericarditis, left heart disease often have difficulty breathing, cough, hemoptysis, chest pain, sometimes accompanied by pulmonary hypertension, much like rheumatic mitral valve damage, physical examination showed cheeks Dark red or bun, jugular vein engorgement, anterior bulging area, enlarged heart, weak apex beat, strong heart sound, fast heart rate, may have gallop, most no murmur or mild systolic murmur, low blood pressure, Pulse pressure is small, pulse is weak, there may be strange veins, abdominal swelling, mobile dullness, liver and hard, lower limbs can be concave edema, embolism, X-ray examination of the heart to severely increase, right Cardiac lesions cause multiple degrees of heart enlargement, spherical or flask-like, increased right atrial height, decreased pulmonary blood, partial cases of right ventricular outflow tract dilatation, mild bulging under the left upper lobe pulmonary artery, even visible to the right ventricle Endometrium Linear calcification shadow, shadow shape change of heart left heart disease and atrioventricular increased resembling rheumatic mitral valve disease, heart mild to moderate increase in left atrial enlargement, pulmonary congestion or have varying degrees of pulmonary hypertension performance.

Double-chamber lesions are the above comprehensive changes, often seen in right ventricular lesions, electrocardiogram most common atrial hypertrophy, atrial premature contraction, atrial fibrillation, ST-T changes, PR interval prolongation and low voltage, a few can be expressed as Right ventricular hypertrophy, echocardiography is helpful for diagnosis. It can be seen that the left and right atrium are obviously enlarged, the left and right ventricular chambers are small, the atrioventricular valve, the chordae, the papillary muscle and the apex of the apex are thickened, and there are often tricuspid valves and two. The cusp is incomplete, and there is a small amount of pericardial effusion. The Doppler flow spectrum shows an increase in the early filling rate of mitral diastolic, while the atrial filling rate is reduced, E/A 2, and the hepatic venous diastolic blood flow is reversed during inhalation. Increased, cardiac catheterization showed increased vena cava and atrial pressure, increased ventricular end-diastolic pressure, early diastolic pressure curve, late plateau wave, pulmonary artery pressure and pulmonary artery resistance, decreased stroke volume, cardiovascular angiography, right heart disease The right ventricular apex is occluded, the inflow tract is contracted and deformed, the outflow tract is dilated, the tricuspid regurgitation is incomplete, the right atrium is huge, and the left heart lesion shows a slight increase or small in the left ventricle, but there is deformation, and the mitral valve is closed. Incomplete, the left atrium is moderately enlarged.

Examination of restrictive cardiomyopathy in children

1. Blood and bone marrow examination: Eosinophilic endomyocardial syndrome shows early blood and bone marrow eosinophilia.

2. Endomyocardial biopsy: Endocardial myocardial biopsy can often confirm the diagnosis. The histological features are mainly endocardial and subendocardial myocardial fibrosis, which may have myocardial cell degeneration.

3. Chest X-ray examination: X-ray showed mild to moderate increase of heart shadow, with two atrial enlargement or right atrium and right ventricle enlargement, and occasionally right ventricular endocardial linear calcification shadow.

4. Electrocardiogram examination: ECG is common in atrial hypertrophy, and there may be QRS complex low voltage, arrhythmia (atrial arrhythmia, atrioventricular block or bundle branch block), ST-T changes.

5. Echocardiography: Echocardiography can show left and right atrium significantly enlarged, left and right ventricular cavity size is normal, ventricular wall hypertrophy, apical ventricular occlusion, myocardial endocardial structure ultrasound echo density abnormalities, ventricular wall activity amplitude Decreased, there are signs of intracardiac thrombosis and atrioventricular valve regurgitation, normal left ventricular systolic function (ejection fraction), Doppler ultrasound mitral flow spectrum: E peak increased, DT decreased, E / A ratio increased .

6. Cardiac catheterization: the ventricular end diastolic pressure is increased, the pressure curve is inclined downward, and then suddenly rises, and remains platform-like, showing a "square root" sign.

Diagnostic identification of limited cardiomyopathy in children

diagnosis

Pediatric RCM diagnosis is more difficult, should be based on a comprehensive analysis of medical history, physical signs and auxiliary examination, if necessary, myocardial endocardial biopsy to confirm the diagnosis, the clinical manifestations of this disease are mainly slow-developing right heart failure, including liver, ascites, lower extremity edema The signs are more prominent, and restrictive cardiomyopathy has the following characteristics:

1 no history of related infections.

2 often can touch the apex of the pulsation, there is galloping, the atrioventricular valve is not fully murmur.

3 ECG often has atrial hypertrophy, atrioventricular block, bundle branch block.

4X-ray examination, CT and MRI examination without pericardial calcification or thickening.

5 echocardiography showed bilateral atrial enlargement, apical ventricular occlusion, and thickened ventricular wall.

6 endomyocardial biopsy can help the diagnosis and differential diagnosis of this disease.

Differential diagnosis

Diagnosis should be differentiated from Ebstein malformation, dilated cardiomyopathy and constrictive pericarditis.

Constrictive pericarditis

It is difficult to distinguish from constrictive pericarditis, because the clinical manifestations are similar, and the hemodynamic changes are the same. One case of Beijing Children's Hospital has performed pericardial exploration, but no pericardial lesions, RCM and narrowing. The identification of pericarditis is important because the latter can be treated surgically, and the constrictive pericarditis has a history of Staphylococcus aureus or tuberculosis infection. X-ray examination showed that the heart shadow was not obvious, and the outer edge of the heart was stiff and enveloped. Electrocardiogram is mainly based on low voltage and T wave changes. Echocardiography is more meaningful for identifying the two. The ventricular cavity is normal in constrictive pericarditis, the endocardium and valve are normal, and there is no thickening or insufficiency. Pericardial thickening or calcification, Doppler blood flow spectrum analysis, the maximum flow velocity of early tricuspid valve diastolic blood flow during expiration is significantly reduced, recently reported transesophageal ultrasound observation of pulmonary venous blood flow respiration phase changes, which is conducive to the difference between the two, The above points are helpful for identification,

2. Dilated cardiomyopathy

Dilated cardiomyopathy is mainly caused by left ventricular enlargement. Although Ebstein malformation is similar to restricted cardiomyopathy, two-dimensional echocardiography has a tricuspid valvular deformity in addition to right atrial enlargement. Endocardial myocardial biopsy was performed.

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