Endocardial fibroelastosis in children

Introduction to endocardial fibroelastosis in children Endocardial fibroelastosis (EFE), also known as endocardial sclerosis, has not yet been elucidated. It is a more common type of infant cardiomyopathy, also known as primary endocardial fibroelastosis. The incidence rate has declined in the past decade. Congenital heart disease such as aortic coarctation, aortic stenosis, aortic valve atresia and other endocardial fibroelastosis, called secondary endocardial fibroelastosis, its clinical significance depends on the primary heart Malformations are not included in the scope described here. Endocardial fibroelastosis has been previously referred to as "fetal endocarditis", "sclerosing endocarditis", "endocardial myocardium fibrosis" and the like. The main pathological changes were subendocardial elastic fibers and collagen fibrosis. basic knowledge Sickness ratio: 0.5% Susceptible people: children Mode of infection: non-infectious Complications: cardiogenic shock pneumonia cerebral embolism mitral regurgitation

Causes of endocardial fibroelastosis in children

(1) Causes of the disease

As early as 1816, it was suggested that this disease may be caused by intrauterine infection of fetal endocarditis. The cause is still unknown. The following views have been proposed:

1. Viral infection caused by viral infection in the fetal period or after birth. It is believed that Coxsackie B virus, mumps virus and infectious mononucleosis virus infection are related to this disease, and have been from endocardial elasticity. In the myocardium of children with fibroplasia death, Coxsackie B virus was isolated, and histological changes were also seen in histology. Some people inoculated mumps virus in chicken embryos, and hatched chickens developed endocardial elastic fiber hyperplasia. Symptoms, pregnant women with mumps in early pregnancy, the birth of the baby can occur, some people have reported diffuse myocarditis caused by infectious mononucleosis, can develop into endocardial fibroelastosis, in addition, from the interstitial In the pathological examination of death cases of myocarditis and endocardial fibroelastosis, it is found that the pathological changes of the two often coexist, and the myocarditis of the short course of the disease changes obviously, the endocardial elastic fiber does not proliferate; the course of disease is longer, from the onset The change in myocarditis is mild to deaths of more than 4 months.

Endocardial elastic fiber proliferation is significant, so it is believed that myocarditis and endocardial fibroelastosis may be manifested in different periods of the same disease. Myocarditis is the predecessor of endocardial fibroelastosis, and the primary endocardial elasticity of Beijing Children's Hospital Of the 102 cases of fibroproliferative disease, 65 cases had obvious changes of myocarditis, 4 of which were in the neonatal period, but the course of disease was not related to endocardial fibroelastosis and myocarditis. In recent years, polymerase chain reaction (PCR) was reported. And reverse transcriptase-polymerase chain reaction (RT-PCR) method for the detection of viral nucleic acid in the myocardium, 29 cases of myocardial specimens of endocardial fibroelastosis autopsy, and 65 cases of myocardial specimens of other heart autopsy (congenital There were 53 cases of heart disease and 12 cases of hypertrophic cardiomyopathy. The results showed that 26 cases (90%) were positive for viral nucleic acid in the endocardial fibroelastosis group, and only 1 case was positive for enterovirus RNA in other heart disease groups (1.5%). Endometrial fibroelastosis detected 21 cases of mumps virus RNA (72%), 8 cases of adenovirus DNA (27.5%), 1 case of cytomegalovirus DNA and enterovirus RNA, mumps virus Positive 21 In the example, there are 5 cases of other viral nucleic acids: 3 cases of adenovirus, 1 case of cytomegalovirus and 1 case of enterovirus. This data suggests that viral infection is one of the causes of endocardial fibroelastosis and supports endocardial elasticity. Fibroplasia is a consequence of viral myocarditis, especially mumps viral myocarditis.

2. Intrauterine hypoxia causes endocardial developmental disorders.

3. Genetic factors 9% of cases are familial, which is considered to be autosomal inherited.

4. Inherited metabolic defects have been reported in cardiac glycogen storage disease, endometrial fibroelastosis in children with mucopolysaccharidosis and carnitine deficiency.

5. Secondary to the hemodynamic changes in the ventricular height, the stress on the ventricular wall is increased, the hemodynamic effect causes the endocardial elastic fibers to proliferate, and the endocardial elastic fiber proliferation is considered to be a non-specific change.

(two) pathogenesis

Classification

(1) primary: simple left ventricle, left atrium, right ventricle, right atrium, endocardial elastic fiber hyperplasia, without other malformations, can be accompanied by valve involvement, mainly mitral and aortic valve thickening, Deformation, often caused by mitral regurgitation.

(2) secondary: coexist with other congenital heart diseases, such as aortic coarctation, aortic valve atresia, mitral atresia, left ventricular dysplasia syndrome.

2. Pathological classification can be divided into dilatation type (type I, left ventricular enlargement) and constriction type (type I, left ventricular volume is normal or decreased, and left and right atrium and right ventricle increase) according to left ventricular size.

3. Hemodynamic abnormalities due to endocardial thickening, myocardial contraction and diastolic limitation, left ventricular diastolic pressure increased significantly, resulting in decreased cardiac output, affecting myocardial function, increased venous pressure, pulmonary edema, and ultimately Heart failure.

Pediatric endocardial fibroproliferative prevention

We should do a good job in pregnancy care, actively prevent various infections, especially viral infectious diseases; prevent intrauterine hypoxia, actively prevent and treat pregnant women with high blood pressure, diabetes and other diseases; at the same time, we must do a good job in genetic disease consultation.

Complications of endocardial fibroelastosis in children Complications, cardiogenic shock, pneumonia, cerebral embolism, mitral regurgitation

Can be complicated by heart failure, cardiogenic shock, pneumonia, cerebral embolism, mitral regurgitation and so on.

Pediatric endocardial fibroelastosis symptoms common symptoms systolic murmur vocal tachycardia limbs wet cold anti-feed pulse accelerate heart failure liver swelling hairpin pale

The age of onset of 2/3 sick children is less than 1 year old, and the clinical manifestations are mainly congestive heart failure, often occurring after respiratory infection.

1. General symptoms can be divided into 3 types according to the severity of symptoms:

(1) fulminant: sudden onset of illness, sudden dyspnea, vomiting, refusal to eat, perioral cyanosis, pale, irritability, tachycardia, scattered wheezing or dry voice in the lungs, liver enlargement Also, edema can be seen, which is a sign of congestive heart failure. A small number of children present with cardiogenic shock, showing irritability, gray complexion, cold and cold limbs, and accelerated and weak pulse. The age of this type of sick child is more than 6 months. Within, it can cause sudden death.

(2) acute type: onset is also faster, but the development of congestive heart failure is not as sharp as the fulminant, often complicated by pneumonia, accompanied by fever, wet voice in the lungs, some children due to the collapse of the wall thrombus Most cases of cerebral embolism are due to heart failure, and a few can be relieved by treatment.

(3) Chronic type: the disease is slightly slow, the age is more than 6 months, the symptoms are acute, but the progress is slow, some children's growth and development are affected, can be relieved after treatment, live to adulthood, but also because Repeated heart failure and death, most of the children are acute, chronic type accounts for about 1/3, less in the neonatal period, often narrowed, clinical manifestations of left ventricular obstruction symptoms, occasionally in the palace Heart failure occurs within a few hours after birth.

2. The heart is moderately enlarged in terms of signs. In the chronic children, the anterior region of the heart is raised, the apex is weakened, the heart sound is blunt, the tachycardia is too fast, there may be galloping, and generally there is no murmur or only mild systolic murmur. A small number of patients with mitral regurgitation or relative mitral regurgitation due to enlarged heart can hear systolic murmur at the apex, generally grade II to III. The characteristics of this disease are:

Most of the infants under the age of 1.1 suddenly develop heart failure at 2 to 6 months.

2. The X-ray chest radiograph is enlarged by the left ventricle and the heart beat is weakened.

3. The heart has no obvious noise.

4. ECG showed left ventricular hypertrophy, or V5, 6 lead T wave inversion.

5. Echocardiography showed enlargement of the left ventricle, thickening of the endocardial echo, decreased systolic function, and histologically confirmed endocardial myocardial biopsy.

Examination of pediatric endocardial fibroelastosis

In patients with concurrent infection, peripheral blood leukocytes and neutrophils increased; often hemoglobin decreased and other changes.

1. X-ray examination showed that the left ventricular enlargement was obvious, the heart shadow was generally enlarged, and the aortic heart shadow was similar. The left heart edge beat was weakened. Especially in the left anterior oblique position, the left ventricular beat disappeared and the right ventricular beat was normal. It is more diagnostic, the left atrium is often enlarged, the lung texture is increased, and the pulmonary congestion is obvious.

2. Most of the electrocardiogram examination showed left ventricular hypertrophy, ST segment and T wave changes, long-term heart failure, resulting in increased pulmonary artery pressure, right ventricular hypertrophy or left and right ventricular hypertrophy, in addition, occasional pre-contraction and atrioventricular Conduction block, narrowed type of right ventricular hypertrophy and right axis of the ECG axis.

3. Echocardiographic examination showed that the left ventricular cavity was enlarged, the left ventricular posterior wall motion was weakened, the left ventricular endocardial echo was enhanced, the left ventricular systolic function was decreased, and the shortening fraction and ejection fraction were decreased.

4. Cardiac catheterization can show the left atrium, pulmonary artery mean pressure and left ventricular end-diastolic pressure increase, left ventricular selective angiography can be found left ventricular enlargement, wall thickening, contraction and diastolic ventricular size is almost fixed, left ventricular Contrast agent emptying delay, mitral and aortic regurgitation are common.

5. Cardiovascular angiography showed left ventricular dilatation, hypertrophy, small changes in systolic and diastolic volume, left ventricular contrast agent delayed emptying, narrowed type showed right ventricular dilatation, left ventricular cavity was normal or small, left ventricular ventricle The air delay is increased, the left atrial pressure is increased, and the pulmonary artery pressure is close to the systemic circulation pressure.

Diagnosis and identification of endocardial fibroelastosis in children

Diagnosis is based on clinical performance and examination.

The disease must be differentiated from heart failure in infancy, no obvious murmur and left ventricular enlargement:

1. Acute viral myocarditis has a history of viral infection. Electrocardiogram is characterized by QRS wave low voltage, QT interval prolongation and ST-T change; while endocardial fibroelastosis is left ventricular hypertrophy, RV5,6 voltage High, TV5, 6 inverted, sometimes need to be endocardial myocardial biopsy to distinguish.

2. The left coronary artery originated from pulmonary artery malformation due to myocardial ischemia, the child is extremely irritated, crying, angina, electrocardiogram often shows the pattern of anterior myocardial infarction, I, aVL and V5, 6 lead ST segment rises or falls And QS wave type, heart color ultrasound can be clearly diagnosed.

3. Type II glycogen storage disease children with low muscle strength, large tongue, EC interval PR interval is often shortened, skeletal muscle biopsy can be identified.

4. Aortic coarctation Lower extremity arterial pulsation weakened or disappeared, upper limb blood pressure increased, pulse enhancement can be identified.

5. Dilated cardiomyopathy is more common in children over 2 years old. In addition, it is still necessary to distinguish from pneumonia, bronchiolitis, pericarditis and pericardial effusion. In particular, it should be noted that this disease is easily misdiagnosed as pneumonia in the clinic. Examination, leading to early diagnosis and treatment, chest X-ray and echocardiography is very important for the diagnosis of this disease, because the left heart of the giant heart close to the chest wall, and misdiagnosed as pleural effusion or mediastinal tumor, should be vigilant.

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