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Description restrictive cardiomyopathy disease (), is the heart
Restrictive cardiomyopathy, myocardial fibrosis subendocardial membrane, causing relaxation and filling of hard to stretch limited severely impaired diastolic function, and systolic function remained normal or only mildly impaired heart disease. In the 3 types of unexplained cardiomyopathy, restrictive cardiomyopathy, and hypertrophic dilated than rare. Mainly refers to the disease occurs in tropical regions Endomyocardial fibrosis (endomyocardialfibrosis, EMF) and more common in temperate regions of increased eosinophils cardiomyopathy (L? Ffler'scardiomyopathy). In recent years, clinical and experimental studies show that the two different types of disease, may be the same disease in different stages of the performance of the early clinical manifestations in the disease they are different, but critical stage, clinical manifestations are systemic congestive obstructive myocardial pathological changes consistent between the two, so out in a discussion. Symptoms and Signs 1. The general performance of the disease mostly occurs in tropical and temperate, tropical slightly more than
Temperate restrictive cardiomyopathy. Each age group can be sick, sick men than women, male to female ratio is about 3:1. Only the early fever, general malaise, more common in eosinophilic hyperplasia. Late heart failure and multiple pulmonary circulation embolism. 2. Ventricular dysfunction or right ventricular performance of


biventricular lesions, right heart failure often the main clinical manifestations resemble constrictive pericarditis. Left ventricular lesions due to diastolic limited, especially in the coexistence of mitral regurgitation, may appear obvious respiratory difficulties in the performance of severe left heart failure and angina. 3. Signs, including blood vessels and abnormal cardiac signs. Are common jugular vein distention, Kussmaul sign, odd pulse. Normal or slightly expanded community center, the first heart sound with low blunt, P2 normal or hyperactivity, could be heard and gallop and systolic murmur. Disease etiology unknown etiology may be related to malnutrition, food, 5 - HT poisoning, allergies and autoimmune related infections. Small number of cases have familial, may be associated with skeletal muscle disease and atrioventricular block. Recent studies suggest that eosinophils are closely related with this type of cardiomyopathy. Pathophysiology of restrictive cardiomyopathy in the pathogenesis is still unclear, may be a variety of
Restrictive cardiomyopathy factors, such as viral infection endocardium, malnutrition, autoimmune and so on. Recent studies suggest that eosinophils are closely related with such heart disease. Increase in eosinophils may be part of endomyocardial reasons, before the onset of heart attack often increased eosinophils, which has the vacuoles of eosinophils and degranulation of the morphological abnormalities of eosinophils granulysin, increased oxidative metabolism, and release of cytotoxic proteins, mainly cationic protein, can damage the myocardial cells, and role in serosal and muscle enzymes in the mitochondrial respiratory chain composition, degree of injury depends on endomyocardial addicted acidic granulocyte infiltration of the endomyocardial severity and duration. In addition, the release of degranulation of cationic proteins can also affect the coagulation system, easy to form a mural thrombus. Also can damage the endothelial cells, inhibition of endothelial cell growth. Myocardial infiltration of eosinophils caused by myocarditis, inflammation of the distribution is confined to the inner layer of myocardial microcirculation can be rearranged to explain. Therefore, necrosis and thrombosis have entered the period of healing and fibrosis of the final entry. On the eosinophil infiltration of the myocardium and cause degranulation of eosinophils causes is not clear, may be some special virulence factors, such as viruses, parasites and other infections, and these factors and myocardial tissue with the same antigen clusters, induced autoimmune response, causing restrictive cardiomyopathy. In addition, some patients with unknown etiology, manifested as ventricular diastolic filling of the obstacles and limited relaxation, patients have endocardial thickening or fibrosis of unknown cause, has been associated with increased eosinophil disease. Most patients with myocardial fibrosis, namely primary (or idiopathic) restrictive cardiomyopathy. It has been reported, the disease was familial disease sometimes may be associated with skeletal muscle disease and atrioventricular block. Under normal circumstances diagnostic tests diagnosis, RCM significantly higher than the signs and symptoms, misdiagnosis few opportunities.
Diagnosis of restrictive cardiomyopathy points: ventricular cavity and normal or near normal systolic function; diastolic dysfunction, early diastolic ventricular pressure curve was fast sinking, while the late rise was shaped platform; characteristic pathological changes, such as heart endomyocardial fibrosis, increased eosinophils endocarditis, cardiac amyloidosis, and scleroderma, etc., can be confirmed. Laboratory tests: blood tests: blood eosinophilia. Other auxiliary examinati. Electrocardiogram P wave very high angle, QRS showed a low voltage can, ST segment and T wave changes in common, there may be premature systole and cardiac arrhythmias such as bundle branch block, about 50% of patients with atrial fibrillation can occur. Easy invasion 2.X line and right ventricular disease, about 70% showed increased cardiothoracic ratio, with a right atrial enlargement, a heart of impact can be spherical. Left ventricular involvement can often see pulmonary congestion. Individual patients is still visible endocardial calcification. 3. Echocardiography confirmed the importance of RCM method, about 82% of the patients showed a small ventricular cavity, the apex occlusion, echogenic endocardium, AV valve insufficiency, atrial enlargement and mural thrombus, mitral valve was more than layer reflection, after the leaves often no activity. Ventricular diastolic diameter can increase, led by the mitral E peak acceleration point, but the E peak deceleration time shorter (), often 150ms, Doppler flow image shows an abrupt end diastolic rapid filling; diastolic middle and late ventricular diameter without continuing to expand, A peak reduction, E / A ratio increases, the specific criteria: E peak 1.0m / s, A peak of 0.5m / s, E / A ratio 2.0, isovolumic relaxation shorten the time 70ms. 4. Cardiac catheterization is the identification of RCM and an important method of constrictive pericarditis. Half of the cases of ventricular pressure curve and constrictive pericarditis may appear similar to the typical "square root" shape changes and right atrial pressure is increased and the Y valley deep. However, patients with RCM left and right ventricular diastolic pressure difference between the often than 5mmHg, right ventricular end diastolic pressure <1 / 3 of right ventricular systolic pressure, right ventricular systolic pressure o 50mmHg. Ventricular cavity left ventricular angiography shows narrowing of the apex obtuse angle, and a mural thrombus and mitral regurgitation. Smooth but stiff left ventricular shape, ventricular systolic function was normal. 5. Endomyocardial biopsy is an important means of diagnosis of RCM. Genju endomyocardial disease may be at different stages of necrosis, thrombosis, fibrosis, three kinds of pathological changes. Endocardium may be accompanied by thrombosis, thrombus occasionally eosinophils; could showed endocardial inflammation, necrosis, granuloma, fibrosis and other changes; myocardial cell degeneration and necrosis may be associated with interstitial fibrosis change. Some people endomyocardial biopsy and hemodynamic findings combined analysis of the characteristics and types of RCM that end diastolic volume <100ml/m2 and left ventricular end-diastolic pres 18mmHg is a prominent feature of primary RCM. Some people meet the above criteria and cardiac hemodynamics in patients with endometrial biopsy system, results suggest that: who simply restrictive ventricular weight / volume ratio 1.2g/ml 0.5g/ml, ejection fraction 58% 5%, left ventricular end diastolic volume 67.5ml/m2 12.6ml/m2, left ventricular end-diastolic pressure 26.7mmHg 3.5mmHg; ventricular hypertrophy were combined restrictive weight / volume ratio 1.5g/ml 0.07g/ml , ejection fraction 62% 1%, left ventricular end diastolic volume 69ml/m2 10ml/m2, left ventricular end-diastolic pressure of 30mmHg 7mmHg; those with mild expansion of restrictive ventricular weight / volume ratio 0.9g/ml, left ventricular end-diastolic volume 98ml/m2, and left ventricular end-diastolic pressure of 40mmHg. Histology and electron microscopy showed that there were various types of myocardial and myofibrillar derangement and endomyocardial fibrosis. 6.CT and magnetic resonance is the identification of RCM and constrictive pericarditis the most accurate non-invasive examination means. RCM were not thickened pericardium, pericardial thickness 4mm constrictive pericarditis can be ruled out; and pericardial thickening supports the diagnosis of constrictive pericarditis. 7. Radionuclide ventriculography imaging of the right heart-shaped features of RCM are: the right atrium was significantly expanded with radionuclide retention; right ventricular to left shift, the apex showed clear, in the right ventricle left ventricle left back widening of the right ventricular outflow tract and right ventricular phase retardation, decreased right ventricular function; poor lung imaging, lung radionuclide through the time delay; phase and left ventricular function is usually in the normal range. Differential Diagnosis 1. Constrictive pericarditis constrictive pericarditis following points will help the diagnosis:
restrictive cardiomyopathy have a history of active pericarditis; odd clock; no atrioventricular conduction disturbances electrocardiogram; CT MRI, pericardial thickening; chest X ray had pericardial calcification; Echocardiography septal cut track, and reduced motor coordination can be seen ventricle; characteristics of ventricular pressure curve left and right ventricular filling pressure is almost equal to the difference <5mmHg; endomyocardial biopsy or other non-cardiac amyloidosis, the performance of invasive disease. 2. Hypertrophic cardiomyopathy when thick ventricular cardiomyopathy can be symmetrical or non-symmetry was thickening, ventricular diastolic compliance and decreased diastolic blood pressure, often in patients with breathing difficulties, chest pain, syncope. Obstructive hypertrophic cardiomyopathy who can be heard and shrinkage of the middle and late ejection murmur, often accompanied by tremor. The strength of noise related to drug and body position. Echocardiography lesions mainly involving the interventricular septum. RCM of the disease is not unique to the early diastolic rapid filling and diastolic, the late slow filling of the features that will help identify. 3. Ischemic cardiomyopathy and hypertensive cardiac hypertrophy in the two situations may have different degrees of myocardial fibrosis, and were reduced ventricular compliance, diastolic pressure is increased and decreased cardiac output were similar to other and RCM However, ischemic cardiomyopathy have clear evidence of coronary artery disease, coronary angiography can be diagnosed; hypertensive cardiac hypertrophy and more long-term blood pressure and left ventricular dysfunction in history; In addition, the two are left heart in the clinical involvement and is characterized by left ventricular dysfunction, while the RCM is often chronic right heart failure more prominent. Treatment 1. Diuretic drug treatment of heart failure and vasodilator drugs can relieve symptoms, but should be noted that small doses to avoid the impact of reduced ventricular filling and cardiac output. Calcium channel blockers may be effective in improving ventricular compliance. With obvious diastolic dysfunction, atrial fibrillation in the event of digitalis preparations can be applied to improve ventricular filling. A mural thrombus, and (or) should have been added with thrombosis anticoagulant and antiplatelet agents. 2. Surgical treatment including removal of mural thrombus and endocardial fibrosis, mitral and tricuspid valve replacement. Operative mortality rate of about 20%. Has survived for 5 years in patients, heart function improved in 70% to 80%. Effective treatment for a heart transplant, but the need to appear before cachexia. Complications of the disease often complicated by heart failure, arrhythmia, arterial embolism, and complications such as pericardial effusion. 1. Failure restrictive cardiomyopathy due to endocardial and subendocardial fibrosis in a wide range, resulting in reduced myocardial compliance, ventricular diastolic restriction, filling blocked, pulmonary circulation, and (or) systemic vascular congestion and tissue lack of blood perfusion performance severely impaired diastolic function, and systolic function remained normal or only mildly damaged. 2. Arrhythmia restrictive cardiomyopathy with cardiac arrhythmia and the conduct of subendocardial myocardial fibrosis and calcification. More common are sinus tachycardia, atrial flutter or fibrillation, right bundle branch block and the shrinkage of the former. 3. Artery embolization endocardium of the disease and subendocardial fibrosis, leading to ventricular diastolic restriction, filling blocked, pulmonary and systemic congestion, can lead to heart cavity and peripheral venous thrombosis, once off can cause thrombosis. 4. Pericardial effusion and pericardial effusion in this disease endocardium and subendocardial myocardial fibrosis, ventricular diastolic restriction, filling impairment, pulmonary and systemic congestion, elevated venous pressure. Bedridden patients and chronic malnutrition may be associated with hypoproteinemia. Prognosis and prevention of prognosis: The disease course of varying lengths, the light survival up to 25 years, the cause of death as heart failure or pulmonary embolism more. Lesions involving the left ventricular heart function class ~ class (NYHA), severe mitral and tricuspid insufficiency and thrombosis more than a poor prognosis. Endocarditis may be due to progressive increase of heart failure and died within a few months, a few can be transformed into chronic, and primary RCM or endocardial fibrosis, and prognosis of myocardial damage depends on the extent of endocardial fibrosis . Prevention: to strengthen publicity and education, improve the patient's level of awareness of this disease. Actively eliminate the cause, to avoid complications. Improve living standards, not tired, to prevent infection. Increased eosinophil epidemiology cardiomyopathy more common in temperate regions, endomyocardial fibrosis is occurs in tropical and subtropical regions, such as Africa, Uganda, Burundi and Cameroon, Brazil and Venezuela in South America, Asia, India, Sri Lanka and China and other countries have reported that in temperate regions such as Britain, France and Switzerland there are sporadic cases reported. Although China has just over 90 cases reported in the literature, but in fact the number of patients the incidence is much more than this number, mainly in the Guangxi Zhuang Autonomous Region, Guangdong and Guizhou provinces and other sub-tropical regions, temperate regions as well as sporadic cases reported. In the Guangxi Zhuang Autonomous Region, endomyocardial fibrosis patients with primary cardiomyopathy accounts for about year 3% of hospitalized patients. Health Tips strengthen publicity and education, improve the patients level of awareness of this disease. Actively eliminate the cause, to avoid complications. Improve living standards, not tired, to prevent infection.


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