Endocardial fibroelastosis
Introduction
Introduction Endocardial fibroelastosis (EFE), also known as endocardial sclerosis, has not yet been elucidated. It is a common type of primary cardiomyopathy in children, also known as primary endocardial fibroelastosis. 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 this section.
Cause
Cause
As early as 1816, it was suggested that this disease may be caused by intrauterine infection of intrauterine infection. The cause of the disease is still unclear, and the following views have been proposed:
1 virus infection: caused by inflammatory reaction caused by viral infection during fetal or postnatal period. It is believed that Coxsackie B virus, mumps virus and infectious mononucleosis virus infection are associated with this disease. Coxsackie B virus was isolated from the myocardium of children with endocardial fibroelastosis and histological changes were seen in histology. Some people inoculate mumps virus in chicken embryos, and women with mumps in early pregnancy can develop this disease in babies born. It has also been reported that diffuse myocarditis caused by infectious mononucleosis can progress to endocardial fibroelastosis. In addition, from the pathological examination of interstitial myocarditis and endocardial fibroelastosis death, it is found that the pathological changes of both often coexist, and the myocarditis changes obviously in the short course of the disease, and the endocardial elastic fiber does not proliferate; Long, from onset to death for more than 4 months, the change of cardiomyelitis is slight, 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. There were 102 cases of pathological data in Beijing Children's Hospital. Among them, 65 cases had myocarditis changes, 4 of which occurred in the neonatal period.
2 intrauterine hypoxia caused by endocardial developmental disorders.
3 genetic factors: 9% of cases showed familial disease, which is considered to be autosomal inherited.
4 Inherited metabolic diseases: Endocardial fibroelastosis occurred in children with cardiac glycogen accumulation disease, mucopolysaccharidosis and carnitine deficiency.
5 secondary to hemodynamic changes: when the ventricular height is enlarged, the tension of the ventricular wall is increased, and the hemodynamic effect causes the endocardial elastic fibers to proliferate, which is considered to be a non-specific change in endocardial elastic fiber proliferation.
Examine
an examination
Related inspection
Ultrasound diagnosis of cardiovascular disease
The characteristics of this disease are: most of the infants under 11 years old suddenly have heart failure from 2 to 6 months; 2X chest X-ray enlarges the left ventricle, the heart beats weakly; 3 the heart has no obvious murmur; 4 ECG shows left ventricle Hypertrophy, or V- and 6-lead T-wave inversion. 5 echocardiography showed left ventricular enlargement, endocardial echo thickening, and reduced systolic function. Histologically confirmed endocardial myocardial thrombosis.
The age of onset of 2/3 sick children is within 1 year of age. Clinical manifestations are mainly congestive heart failure, often occurring after respiratory infections.
General symptoms
According to the severity of the symptoms, it can be made into three types:
(1) fulminant: sudden onset of illness, sudden dyspnea, vomiting, refusal to eat, perioral cyanosis, pale, irritability, tachycardia. There are scattered wheezing or dry rales in the lungs, the liver is swollen, and edema is also seen, both of which are signs of congestive heart failure. A small number of children presented with cardiogenic shock, which showed symptoms such as irritability, gray complexion, cold and cold limbs, and accelerated and weak pulse. This type of sick child is more than 6 months old and 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 rales in the lungs. Some children develop cerebral embolism due to the loss of the wall thrombus. Most died of heart failure, and a small number of treatments were relieved.
(3) Chronic type: The incidence is slightly slow, and the age is more than 6 months. Symptoms are acute, but progress is slow, and some children's growth and development are affected. It can be relieved after treatment, and it can live in adulthood. It can also die from repeated heart failure.
Most of the sick children are acute. Chronic type accounts for about 1/3. There are fewer cases in the neonatal period, often narrowed, and the clinical manifestations are symptoms of left ventricular obstruction. Occasionally, heart failure occurs in the palace, and death occurs hours after birth.
2. Signs
The heart is enlarged more than moderately, and the anterior region of the heart can be seen in chronic children. The apex beats weakened, the heart sounds blunt, the tachycardia is too fast, there may be galloping horses, generally no noise or only mild systolic murmur. A small number of patients with mitral regurgitation or relative mitral regurgitation due to enlarged heart can hear systolic murmurs at the apex, generally grade II to III.
3. X-ray inspection
The left ventricular enlargement is obvious, the heart shadow is generally enlarged, and the aortic heart shadow is approximated. The left heart beat is weakened. Especially in the left anterior oblique position, the left ventricular beat disappears and the right ventricular beat is normal. significance. The left atrium often increases. The lungs have increased texture and the lungs have obvious blood stasis.
4. ECG examination
Most of them showed left ventricular hypertrophy, and ST segment and T wave changed. Long-term heart failure, when the pulmonary artery pressure is increased, right ventricular hypertrophy or simultaneous left and right ventricular hypertrophy may occur. In addition, occasionally premature beats and atrioventricular block. The narrowed type is right ventricular hypertrophy and the right axis of the electrocardiogram.
5. Echocardiography
It can be seen that the left ventricular cavity is enlarged, the amplitude of the posterior wall of the left ventricle is weakened, and the echo of the left ventricular endocardium is enhanced.
Left ventricular systolic dysfunction, short axis shortening rate and ejection fraction were reduced.
6. Cardiac catheterization
It can show the left atrium, the average pressure of the pulmonary artery and the increase of left ventricular end-diastolic pressure. Left ventricular selective angiography revealed a left ventricular enlargement and delayed intraluminal contrast. Mitral and aortic regurgitation are common.
Diagnosis
Differential diagnosis
Endocardial elastic fiber hyperplasia must be differentiated from heart failure in infants with heart failure, no obvious murmur, and left ventricular enlargement:
1 acute viral myocarditis: history of viral infection, electrocardiogram performance with QRS wave low voltage, QT new period prolonged and ST-T changes; and endocardial weak fibrosis is left ventricular hypertrophy, RV5, 6 The voltage is high and RV5 and 6 are inverted. Endometrial myocardial biopsy is sometimes necessary to distinguish.
2 left coronary artery originated from pulmonary malformation, due to myocardial ischemia, children with extreme irritability, crying, angina, electrocardiogram often shows the pattern of anterior myocardial infarction, I, aVL and RV5, 6 lead ST segment rise or fall And QS wave type.
3 heart type glycogen storage syndrome: children with low muscle strength, large tongue, EC period PR interval is often shortened, bone bone biopsy can be identified.
4 aortic stenosis: lower extremity arterial pulsation weakened or disappeared, upper limb blood pressure increased, pulse enhancement can be identified.
5 dilated cardiomyopathy: more common in children over 2 years old. In addition, it must be differentiated from pneumonia, bronchiolitis, pericarditis and pericardial effusion. In particular, it should be noted that this disease is easily misdiagnosed as pneumonia in the clinic, and it is necessary to pay attention to the heart examination, which will lead to early diagnosis and treatment. Chest X-ray and echocardiography are very important for the diagnosis of this disease. Because the left heart of the giant heart is close to the chest wall and misdiagnosed as pleural effusion or mediastinal tumor, it should be vigilant.
The characteristics of this disease are: most of the infants under 11 years old suddenly have heart failure from 2 to 6 months; 2X chest X-ray enlarges the left ventricle, the heart beats weakly; 3 the heart has no obvious murmur; 4 ECG shows left ventricle Hypertrophy, or V- and 6-lead T-wave inversion. 5 echocardiography showed left ventricular enlargement, endocardial echo thickening, and reduced systolic function. Histologically confirmed endocardial myocardial thrombosis.
The age of onset of 2/3 sick children is within 1 year of age. Clinical manifestations are mainly congestive heart failure, often occurring after respiratory infections.
General symptoms
According to the severity of the symptoms, it can be made into three types:
(1) fulminant: sudden onset of illness, sudden dyspnea, vomiting, refusal to eat, perioral cyanosis, pale, irritability, tachycardia. There are scattered wheezing or dry rales in the lungs, the liver is swollen, and edema is also seen, both of which are signs of congestive heart failure. A small number of children presented with cardiogenic shock, which showed symptoms such as irritability, gray complexion, cold and cold limbs, and accelerated and weak pulse. This type of sick child is more than 6 months old and 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 rales in the lungs. Some children develop cerebral embolism due to the loss of the wall thrombus. Most died of heart failure, and a small number of treatments were relieved.
(3) Chronic type: The incidence is slightly slow, and the age is more than 6 months. Symptoms are acute, but progress is slow, and some children's growth and development are affected. It can be relieved after treatment, and it can live in adulthood. It can also die from repeated heart failure.
Most of the sick children are acute. Chronic type accounts for about 1/3. There are fewer cases in the neonatal period, often narrowed, and the clinical manifestations are symptoms of left ventricular obstruction. Occasionally, heart failure occurs in the palace, and death occurs hours after birth.
2. Signs
The heart is enlarged more than moderately, and the anterior region of the heart can be seen in chronic children. The apex beats weakened, the heart sounds blunt, the tachycardia is too fast, there may be galloping horses, generally no noise or only mild systolic murmur. A small number of patients with mitral regurgitation or relative mitral regurgitation due to enlarged heart can hear systolic murmurs at the apex, generally grade II to III.
3. X-ray inspection
The left ventricular enlargement is obvious, the heart shadow is generally enlarged, and the aortic heart shadow is approximated. The left heart beat is weakened. Especially in the left anterior oblique position, the left ventricular beat disappears and the right ventricular beat is normal. significance. The left atrium often increases. The lungs have increased texture and the lungs have obvious blood stasis.
4. ECG examination
Most of them showed left ventricular hypertrophy, and ST segment and T wave changed. Long-term heart failure, when the pulmonary artery pressure is increased, right ventricular hypertrophy or simultaneous left and right ventricular hypertrophy may occur. In addition, occasionally premature beats and atrioventricular block. The narrowed type is right ventricular hypertrophy and the right axis of the electrocardiogram.
5. Echocardiography
It can be seen that the left ventricular cavity is enlarged, the amplitude of the posterior wall of the left ventricle is weakened, and the echo of the left ventricular endocardium is enhanced.
Left ventricular systolic dysfunction, short axis shortening rate and ejection fraction were reduced.
6. Cardiac catheterization
It can show the left atrium, the average pressure of the pulmonary artery and the increase of left ventricular end-diastolic pressure. Left ventricular selective angiography revealed a left ventricular enlargement and delayed intraluminal contrast. Mitral and aortic regurgitation are common.
