Endocardial fibroelastosis
Introduction
Introduction to endocardial elastinosis 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, etc. Membrane elastic fibrosis, called secondary endocardial fibroelastosis. basic knowledge The proportion of illness: 0.001% Susceptible people: infants and young children Mode of infection: non-infectious Complications: cardiogenic shock, cerebral embolism, pneumonia
Cause
Endocardial elastinosis
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 virus infection: caused by inflammatory reaction caused by viral infection in 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 elastic fiber. Coxsackie B virus was isolated from the myocardium of children with hyperplasia, and histological changes were also seen in histology. Some people were infected with mumps virus in chicken embryos, and women with mumps in early pregnancy were born. Occurrence of this disease, it has also been reported that diffuse myocarditis caused by infectious mononucleosis can develop into endocardial fibroelastosis, in addition, pathological examination of death from interstitial myocarditis and endocardial fibroelastosis It is found that the pathological changes of the two often coexist, and the myocarditis changes obviously in the short course of the disease, and the endocardial elastic fiber does not proliferate; the course of disease is longer, and the change of the cardiomyelitis is slight from the onset to death for more than 4 months. Endocardial elastic fiber proliferation is significant, so it is believed that myocarditis and endocardial fibroelastosis may be different stages of the same disease manifesting myocarditis as endocardial elasticity Formerly Victoria hyperplasia, Beijing Children's Hospital pathological data of 102 cases, 65 cases of myocarditis seen changes in the incidence of 4 cases 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.
The heart is enlarged, the endocardium is thickened, it is milky white or grayish white, smooth and bright. In most cases, the whole heart is involved, but the left ventricle is the main, the left atrium and the right ventricle are the second, the papillary muscle, the chordae, the mitral valve. And the aortic valve can also be involved, the ventricular wall may have a wall thrombosis, enlarged under the microscope, sometimes visible interstitial mononuclear hyperplasia, myocardial cell fibrosis, according to the size of the left ventricle can be divided into two types:
1 expansion, left ventricular mild hypertrophy, this type is the most common, accounting for about 95%.
2 narrowing: left ventricular cavity is small, poor development, right atrioventricular enlargement, myocardial thickening, left and right intima thickening, this type is rare, mainly seen in newborns.
Prevention
Endocardial elastinosis prevention
The prognosis of the disease is serious, the mortality rate is about 20% to 25%, the age of onset is large, the response to digitalis is good, the prognosis is good, and the clinical cure can be obtained. The long-term follow-up study of 52 cases with confirmed diagnosis is average. For 47 months (1 to 228 months), the survival rate was 93% for half a year, 83% for 1 year, and 77% for 4 years. The heart index and ejection fraction decreased significantly, and the prognosis was poor, which was more than early death.
Complication
Endocardial elastinosis complications Complications cardiogenic shock cerebral embolism pneumonia
A small number of children presented with cardiogenic shock, visible irritability, gray complexion, cold limbs and accelerated pulse and weak symptoms, the disease is most often complicated by pneumonia, and the condition is serious, the mortality rate is high, seriously threatening the lives of children, and can be complicated with brain embolism.
1, pneumonia
Pneumonia is inflammation of the lung parenchyma, which can be caused by a variety of pathogens, such as bacteria, viruses, fungi, parasites, etc. Others such as radioactivity, chemistry, allergic factors can also cause pneumonia, when the elderly or the body's immune system is accompanied by pneumonia, The mortality rate is particularly high, clinically feverish, palpitations, shortness of breath, lung infiltration, signs of inflammation and certain X-ray findings. After the pneumonia is cured, scars are generally left, and the lungs can restore their original structure and function.
2, brain embolism
Due to damage of the endocardium, the heart is enlarged, the blood is slowly stagnant, and it is easy to form a wall thrombus. When the atrial fibrillation is irregular, the blood flow is easy to cause the wall thrombus to fall off, block the cerebral blood vessels, and cause local brain ischemia. Oxygen forms a cerebral embolism.
Symptom
Endocardial elastinosis symptoms common symptoms irritability liver enlargement tachycardia systolic murmur complexion pale hairy heart enlargement limbs wet cold refuse to eat breathing difficulties
The characteristics of this disease are:
Most of the infants under the age of 11 suddenly develop heart failure from February to June.
The heart of the 2X chest radiograph is enlarged by the left ventricle and the heart beat is weakened.
3 The heart has no obvious murmur.
4 ECG showed left ventricular hypertrophy, or V5, 6 lead T wave inversion.
5 echocardiography showed left ventricular enlargement, endocardial echo thickening, decreased systolic function, and histologically confirmed endocardial myocardial thrombus.
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
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, scattered wheezing or dry rales in the lungs, liver enlargement, also Visible edema, both signs of congestive heart failure, a small number of children presented with cardiogenic shock, visible irritability, pale complexion, cold limbs and accelerated pulse and weak symptoms, the age of this type of disease is more than 6 months 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 brain due to the fall of the wall thrombus Embolism, etc., most died of heart failure, a small number of treatment can be alleviated.
(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 due to recurrent Death in heart failure.
Most of the children are acute, and the 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 uterus. He died a few hours after birth.
2, physical aspects
The heart is more than moderately enlarged. In the chronic children, the anterior palpebral bulge is seen, the apex beats weakened, the heart sounds blunt, the tachycardia is too fast, there may be galloping, generally no murmur or only mild systolic murmur, a few sick children Combined mitral regurgitation or relative mitral regurgitation due to enlarged heart, systolic murmurs can be heard at the apex, generally grade II to III.
Examine
Endocardial elastinosis examination
1. X-ray examination: the left ventricle is enlarged, the heart shadow is generally enlarged, the aortic heart shadow is approximated, and the left heart beat is weakened. Especially in the left anterior oblique position, the left ventricular beat disappears and the right ventricle beats. Normal, more diagnostic, left atrial enlargement, increased lung texture, and obvious pulmonary blood stasis.
2, ECG examination: most of the 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, occasionally premature beat and atrioventricular conduction Blocking, narrowing type is right ventricular hypertrophy and right axis of the ECG axis.
3, echocardiography: visible left ventricular enlargement, left ventricular posterior wall motion amplitude, left ventricular endocardial echo enhanced, left ventricular systolic function decreased, short axis shortening rate and ejection fraction were reduced.
4, cardiac catheterization: can show left atrium, pulmonary artery mean pressure and left ventricular end-diastolic pressure increased, left ventricular selective angiography can be found left ventricular enlargement, intra-orbital contrast agent emptying delay, mitral and aortic valve closure Not always common.
Diagnosis
Diagnosis and differentiation of endocardial elastic fiber hyperplasia
The disease must be differentiated from heart failure in infancy, 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; while endocardial weak fibrosis is left ventricular hypertrophy, RV5,6 The voltage is high, RV5, 6 is inverted, and sometimes it is necessary to perform endocardial myocardial biopsy 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, especially should pay attention to the clinically easily misdiagnosed as pneumonia, must pay attention to the heart Examination, resulting in early diagnosis and treatment, chest X-ray and echocardiography is very important for the diagnosis of this disease, because the left heart of the huge heart close to the chest wall, and misdiagnosed as pleural effusion or mediastinal tumor, should be vigilant.
