specific cardiomyopathy
Introduction
Introduction to specific cardiomyopathy Specific cardiomyopathy refers to a myocardial disease associated with a clear cause or a systemic disease, previously known as a specific cardiomyopathy. Specific cardiomyopathy, including ischemic cardiomyopathy, valvular cardiomyopathy, hypertensive myocardium (characterized by left ventricular hypertrophy with dilated or restrictive heart failure), inflammatory cardiomyopathy (specific autoimmune and infection) Sexual cardiomyopathy (such as glycogen storage disease, glycolipidosis, amyloidosis, etc.), muscular dystrophy, neuromuscular disease, allergies and poisoning reactions (ethanol, catecholamines, anthracyclines, irradiation, etc.) ), perinatal cardiomyopathy, etc. Specific cardiomyopathy refers to a specific heart disease or specific system. Most specific cardiomyopathy has ventricular dilatation and various arrhythmias or conduction disorders due to myocardial lesions. Endocardial biopsy can confirm the diagnosis. basic knowledge The proportion of illness: 0.12% Susceptible people: no specific population Mode of infection: non-infectious Complications: arrhythmia congestive heart failure alcoholic cirrhosis
Cause
Specific cardiomyopathy
Metabolic cardiomyopathy (18%):
Endocrine such as hyperthyroidism, adrenal insufficiency, pheochromocytoma, acromegaly and diabetes. Familial cumulative and invasive judging diseases, such as hemochromatosis, glycogen accumulation disease, Hurler syndrome, Refsum, comprehensive subject, Neimann-Pick disease, Hand-Christian disease, Fabry-Anderson disease, and Morquio-Ullrich disease, lack of cardiomyopathy, such as potassium metabolism Disorders, magnesium deficiency dystrophies (such as malignant malnutrition anemia vitamin B deficiency and selenium deficiency). Amyloidosis, such as primary secondary familial and hereditary cardiac amyloidosis, familial Mediterranean fever and senile amyloidosis.
Allergies and poisoning reactions (16%):
Alcoholic cardiomyopathy, which is damaging to the radiation of ethanol catecholamine anthracyclines, may be excessive alcohol consumption. It is not yet known whether ethanol is pathogenic or conditional, and there is no exact diagnostic criteria.
Ischemic cardiomyopathy (10%):
It is characterized by dilated cardiomyopathy with systolic dysfunction and cannot be explained by the extent of coronary lesions or ischemic injury.
Prevention
Specific cardiomyopathy prevention
Alcoholic should stop drinking. Lack of effective preventive measures, mainly treatment, early positive treatment cure rate is higher.
Complication
Specific cardiomyopathy complications Complications arrhythmia congestive heart failure alcoholic cirrhosis
1. damage to the heart
(1) Arrhythmia: Ethanol damage to the myocardium can be manifested as arrhythmia, with more cases of supraventricular arrhythmia, of which atrial fibrillation is the most common. There is even a danger of sudden death.
(2) Congestive heart failure: Ethanol causes diffuse myocardial damage, decreased cardiac output, and can cause heart failure. Most of the symptoms of left heart failure are obvious, but at the same time, there is often a manifestation of right heart failure.
2. Damage to organs and organs other than the heart
(1) Alcoholic myopathy: Alcohol damages skeletal muscle.
(2) Alcoholic cirrhosis: 80% to 90% of ethanol is metabolized in the liver, and the metabolite acetaldehyde has great damage to liver cells, which can cause hepatocyte membrane lipid peroxidation and destroy the microtubule structure of liver cells. Injury to mitochondria, promote the proliferation of fibrous tissue of the liver interstitial, and cause inflammatory cell infiltration in the liver. Long-term damage of liver cells and fibrous tissue hyperplasia often lead to the occurrence of cirrhosis.
(3) Malnutrition and vitamin deficiency: Many drinkers often do not eat or eat other foods, and long-term protein and some vitamins will not be adequately supplemented.
Symptom
Specific cardiomyopathy symptoms Common symptoms Palpitation arrhythmia Breathing difficulties Heart failure Weak heart enlargement Heart rate increases
Its clinical manifestations are similar to dilated cardiomyopathy. However, amyloid cardiomyopathy can be similar to restrictive cardiomyopathy, and glycogen storage disease is similar to hypertrophic cardiomyopathy.
Specific cardiomyopathy refers to a specific heart disease or specific system. Most specific cardiomyopathy has ventricular dilatation and various arrhythmias or conduction disorders due to myocardial lesions. Endocardial biopsy can confirm the diagnosis.
Type of disease
1, valvular cardiomyopathy: manifested as ventricular dysfunction and exceeded its abnormal load.
2, hypertensive cardiomyopathy: often manifested as left ventricular hypertrophy with dilated or restrictive cardiomyopathy heart failure characteristics.
3, inflammatory cardiomyopathy: inflammatory cardiomyopathy with myocarditis with cardiac insufficiency, specific autoimmune and infectious.
4. Systemic diseases: including connective tissue diseases such as systemic lupus erythematosus, nodular polyarteritis, rheumatoid arthritis, scleroderma and dermatomyositis; infiltration and edema such as sarcoidosis and leukemia.
5, muscular dystrophy: including Duchenne muscular dystrophy, Becker muscular dystrophy, myotonic dystrophy.
6, neuromuscular disease: including hereditary ataxia, Noonan syndrome and color spot disease.
7, perinatal cardiomyopathy: the first time in the perinatal period may be a group of difficult diseases.
Examine
Specific cardiomyopathy
After the onset of clinical symptoms, it can be diagnosed by relying on various examinations. Echocardiography is a non-invasive and effective examination method. Myocardial endocardial biopsy, if positive for specific findings, is helpful in diagnosis and may also reveal invasive lesions.
X-ray: Checking the enlargement of the heart, you may see the shadow of endocardial myocardial calcification. Ventricular angiography showed a reduction in ventricular lumen.
Electrocardiogram examination: low voltage, atrial or ventricular hypertrophy, bundle branch block, ST-T change.
Echocardiography: visible endocardial thickening, apical ventricular occlusion, myocardial endocardial structure ultrasound echo density abnormalities, wall motion is weakened. In the primary patient, the wall of the patient is not thickened, the wall of the invasive lesion can be thickened, the early filling is fast, and the middle and the late is extremely slow. The pericardium is generally not thickened.
Cardiac catheterization: the end-diastolic pressure of the ventricle is gradually increased, resulting in a posterior plateau wave pattern. In the left ventricle, the pulmonary artery pressure can be increased. In the right ventricle, the right atrial pressure is high, and the right atrial pressure curve is significant. The wave replaces the a wave. The measurement of the contraction time interval is not normal.
Diagnosis
Diagnosis and identification of specific cardiomyopathy
Most specific cardiomyopathy is similar to dilated cardiomyopathy, with ventricular dilatation, various arrhythmias, or conduction disorders.
Amyloidosis cardiomyopathy: manifests as a restricted cardiomyopathy.
Glycogen storage disease: similar to hypertrophic cardiomyopathy.
Endocardial biopsy can confirm the diagnosis.
