What Is Endocardial Fibroelastosis?

Endocardial elastic fibrous hyperplasia (EFE), also known as endocardial sclerosis, is still unknown. It is a more common form of pediatric primary cardiomyopathy, also known as primary endocardial elastic fiber hyperplasia. Congenital heart disease such as aortic constriction, aortic valve stenosis, aortic valve atresia and other concurrent endocardial elastic fibrous hyperplasia, which is called secondary endocardial elastic fibrous hyperplasia, and its clinical significance depends on the primary Heart malformation.

Basic Information

nickname: Endocardial sclerosis, primary endocardial elastofibrosis
English name: endocardial fibroelastosis
Visiting department: Internal medicine

Multiple groups: Children
Common locations: heart
Common symptoms: Dyspnea, vomiting, refusing to eat, cyanosis around the mouth, pale, irritable, tachycardia, etc.

Causes of Endocardial Elastic Fibrous Hyperplasia

This disease may be caused by fetal endocarditis due to intrauterine infection. The cause is still unknown, and the following views have been proposed:

Virus infection

Myocardial inflammatory response caused by viral infection during fetal or postnatal period. Coxsackie B virus, mumps virus, and infectious mononucleosis virus infection are thought to be related to the disease. Coxsackie group B virus has been isolated from the myocardium of children with endometrial elastofibrosis, and myocarditis has also been seen histologically. Some people were inoculated with mumps virus in chicken embryos, and the chickens that hatched developed endocardial elastofibrosis. Pregnant women suffer from mumps during early pregnancy, and this disease can occur in babies born. It has also been reported that diffuse myocarditis caused by infectious mononucleosis can develop into endocardial elastofibrosis. In addition, pathological examinations of death cases of interstitial myocarditis and endocardial elastofibrosis have found that the pathological changes of the two often coexist, and the myocarditis in the short course has obvious changes, and the endocardial elastofibrosis does not have significant changes. Myocarditis changes slightly from the onset to death for more than 4 months, and the endocardial elastic fiber hyperplasia is significant. Therefore, myocarditis and endocardial elastic fiber hyperplasia may be manifested in different stages of the same disease, and myocarditis is endocardial Predecessor of elastofibrosis.

2. Other factors

Such as intrauterine hypoxia caused by endocardial developmental disorders. Genetic factors, 9% of the cases were familial, and the disease was considered to be autosomal.

Clinical manifestations of endocardial elastic fibrosis

Two-thirds of the sick children are younger than one year old. There are fewer cases during neonatal period, and heart failure occurs occasionally in the uterus, and it will die within a few hours after birth. The clinical manifestations are mainly congestive heart failure, which often occurs after respiratory infections.

General symptoms

According to the severity of symptoms, the symptoms are three types:

(1) Explosive onset, sudden onset, sudden onset of dyspnea, vomiting, food refusal, cyanosis around the mouth, pale face, irritability, and tachycardia. There are scattered wheezing or dry snoring in the lungs, hepatomegaly, and edema, all of which are signs of congestive heart failure. A small number of children presented with cardiogenic shock, showing symptoms of irritability, gray complexion, cold limbs, and weak and accelerated pulses. Children with this type of disease are mostly under 6 months old and can cause sudden death.

(2) The onset of the acute type is also rapid, but the development of congestive heart failure is not as rapid as that of the fulminant type, often accompanied by pneumonia, accompanied by fever, and wet snoring in the lungs. Some children have cerebral embolism due to the loss of mural thrombus. Most die from heart failure, and a few can be relieved by treatment.

(3) Chronic onset is slightly slower, and the age is more than 6 months. Symptoms are acute, but progress is slow, and the growth and development of some children are affected. It can be relieved after treatment, live to adulthood, or die from recurrent heart failure.

Most sick children are acute. The chronic type accounts for about 1/3.

2. Signs

The heart was enlarged more than moderately, and anterior cardiac bulges were seen in chronic children. The apical pulsation is weakened, the heart sounds are dull, and tachycardia may be caused by galloping horses. Generally, there is no murmur or only mild systolic murmurs. A small number of children with relative mitral valve insufficiency due to enlarged heart can hear systolic murmurs in the apex of the heart, usually grades to .

Endocardial Elastic Fibrous Hyperplasia

1. X-ray inspection

The left ventricular enlargement is the main one, and the heart shadow is generally enlarged, similar to the aortic heart shadow. Seeing that the left ventricular pulsation weakens under perspective, especially when the left ventricular pulsation disappears and the right ventricular pulsation is normal when observing left anterior oblique, it is more diagnostic. Left atrium often increases. Increased lung texture and marked pulmonary congestion.

2. ECG examination

Most of them showed left ventricular hypertrophy with changes in ST segment and T wave. Long-term heart failure can cause right ventricular hypertrophy or simultaneous left and right ventricular hypertrophy when pulmonary artery pressure increases. In addition, atrial or ventricular premature beats and atrioventricular block are visible.

3. Echocardiography

It can be seen that the left ventricular cavity is enlarged, the amplitude of the left ventricular posterior wall motion is weakened, and the left ventricular endocardial echo is enhanced. Left ventricular systolic function was reduced, and the short-axis shortening rate and ejection fraction were reduced.

4. Cardiac catheterization

Can show increased left atrial and pulmonary arterial pressure and left ventricular end-diastolic pressure. Selective left ventricular angiography can reveal enlarged left ventricle and delayed delay of contrast media emptying. Mitral and aortic valve insufficiency are common.

Diagnosis of Endocardial Elastic Fiber Hyperplasia

The characteristics of this disease are:

Most infants under 1.1 years of age suddenly develop heart failure between 2 and 6 months;

2. X-ray chest radiograph shows that the heart enlargement is mainly left ventricular enlargement, and the heartbeat is weakened;

3. There is no obvious noise in the auscultation of the heart;

4. ECG showed left ventricular hypertrophy, or inversion of T wave in leads V5 and 6.

5. Echocardiography showed enlarged left ventricle, thickened endocardial echo, and decreased systolic function.

6. Histological diagnosis requires endocardial myocardial biopsy.

Differential diagnosis of endocardial elastofibrosis

The disease must be distinguished from heart disease with infantile heart failure, no obvious murmur, and enlarged left ventricle:

Acute viral myocarditis

With a history of viral infection, the ECG manifestations are mainly QRS wave low voltage, prolonged QT interval, and ST-T changes; while endocardial weak fibrous hyperplasia is left ventricular hypertrophy, and V5 and 6 lead R wave voltage is high , T wave is inverted.

2. The left coronary artery originates from a pulmonary artery malformation

Due to myocardial ischemia, the child is extremely irritable, crying, and angina pectoris. Electrocardiogram often shows the pattern of anterior wall myocardial infarction. The ST segment of lead , aVL, and V5 and 6 are elevated or decreased and show QS wave pattern.

3. Heart Glycogen Accumulation

Children with low muscle strength, large tongue, ECG PR interval often shortened, skeletal muscle biopsy can be identified.

4. Aortic constriction

Lower limb arterial pulsation weakens or disappears, upper limb blood pressure increases, and pulse increase can be identified.

5. Dilated cardiomyopathy

More common in children over 2 years of age.

In addition, it must be distinguished from pneumonia, bronchiolitis, pericarditis, and pericardial fluid. Special attention should be paid to the fact that this disease is easily misdiagnosed as pneumonia in the clinic, and heart examination must be taken seriously in order to obtain early diagnosis and treatment. Chest X-ray and echocardiography are very important for the diagnosis of this disease. As the left heart margin of the giant heart is close to the chest wall, it should be vigilant that it is misdiagnosed as pleural effusion or mediastinal tumor.

Complications of Endocardial Elastic Fibrosis

A small number of children presented with cardiogenic shock, showing symptoms of irritability, gray complexion, cold limbs, and weak and accelerated pulses. The disease is most often complicated by pneumonia, and the condition is severe, with a high mortality rate, which seriously threatens the lives of children. In addition, cerebral embolism can be complicated. Due to the damage of the endocardium, the heart enlarges and the blood stagnates slowly, making it easy to form mural thrombus. The mural thrombus falls off, blocking the cerebral blood vessels, causing local cerebral tissue ischemia and hypoxia, and forming cerebral embolism.

Endocardial elastic fibrous hyperplasia treatment

The main treatment is to control heart failure. Acute heart failure requires the use of diuretics, intravenous injection of cedilan, etc., and long-term maintenance of digoxin should be taken, which can reach 2 to 3 years or several years, until the heart retracts to normal, premature withdrawal can lead to The condition worsened. Kaibotong can be given orally for a long time, which has certain effects on improving cardiac function. Critical cases were treated with dopamine, dobutamine and corticosteroids. Antibiotics should be used to control lung infections. For patients with severely enlarged heart, severely reduced ejection fraction, and poor response to medication, consider heart transplantation.

Considering that the pathogenesis of this disease may be related to immune dysfunction, in recent years, immunosuppressive therapy has been used, mainly with prednisone, until the electrocardiogram is normal, the heart of X-ray chest radiography is close to normal, and the drug is gradually stopped. The course of treatment is 1 to 1.5 years.

Prognosis of endocardial elastofibrosis

The prognosis of this disease is serious, and the hospital mortality is 20% to 25%. Older age, good response to digitalis treatment, good prognosis, can be cured clinically.