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The ventricular wall stiffness, characterized by a lack of compliance, myocardial disease, limiting one or both ventricular diastolic filling to the left is the most common.
The least common cardiomyopathy.
Etiology and pathology
Cause is usually unknown. Amyloidosis involving the heart muscle is often the body, like the infiltration of iron as hemochromatosis. Fabry disease like Liu Bing and the meat of myocardial involvement, nodal conduction tissue can also be affected. Loffler's disease (mainly involving the heart disease eosinophilia subtype) is restrictive cardiomyopathy one of the reasons, the disease occurs in tropical regions, starting with acute arterial inflammation eosinophilia, and then in the intima, on the tendons and the atrioventricular valve thrombosis, progressing to fibrosis. Endomyocardial fibrosis in temperate regions, involving only the left ventricle.
Restrictive cardiomyopathy can be divided into diffuse non-occlusive and occlusive II, the former myocardial infiltration of abnormal substances (such as amyloidosis), which occurs endocardial and intimal fibrosis (such as endomyocardial fibrosis) . Both can be presented non-diffuse, lesions involving only the one involving the cardiac chambers or uneven part of a heart chamber.
Pathophysiology
Results include the pathophysiology of endocardial thickening or myocardial infiltration with myocardial cell loss, compensatory hypertrophy and fibrosis, the results of any kind can cause atr


ioventricular valve dysfunction, mitral or tricuspid valve regurgitation caused. Results or conduction tissue involvement led to sinus node dysfunction, and some cases showed varying degrees of heart block. Amyloidosis can affect coronary arteries.
The main pathological state of these hemodynamic results is diastolic dysfunction, heart chamber stiffness, lack of compliance, filling pressure is increased. If infiltration or fibrosis of the heart chamber inadequate compensatory hypertrophy, systolic function may worsen, mural thrombus and systemic embolism can be complicated or occlusion at the restrictive cardiomyopathy.
Symptoms, signs and diagnosis
Similar with hypertrophic cardiomyopathy, the main dysfunction is abnormal compliance and one or both abnormal ventricular diastolic filling, left ventricular most common. Symptoms due to diastolic blood pressure, causing pulmonary hypertension, manifested as exertional dyspnea and orthopnea, at right ventricular involvement appeared edema. The resistance of the ventricular filling caused by CO fixed, the results are restricted in the activities. Angina and syncope rare, but the atrial and ventricular arrhythmias and heart block are not uncommon.
Physical examination showed no significant apex beat, low volume carotid arterial pulse, fast, lung collect medical education network. Finishing sound and obvious jugular vein engorgement, with rapid Y-tilt. All cases can actually hear the S4, S 3 can also occur, but to be different with the pericardial knock sound. Usually no noise. Some cases, can be heard functional mitral, tricuspid regurgitation murmur, the murmur is the shape of tendons or ventricular endocardium with myocardial infiltration or fibrosis were changed. Thus the signs and symptoms resemble constrictive pericarditis, non-invasive test, including CT, prove useful in differential normal pericardium, but occasionally even cardiac catheterization can not confirm the diagnosis, this time to be exploratory thoracotomy the pericardium.
Often non-specific ECG abnormalities, manifested as ST segment and T wave changes, and sometimes showed low voltage. Sometimes pathological Q wave myocardial infarction in those without previous. As compensatory hypertrophy, left ventricular hypertrophy can sometimes show. Chest X line, more than normal or reduced heart size, but late amyloidosis or hemochromatosis can be expanded.
Echocardiography systolic function, atrial often expanded. Cardiac amyloidosis showed abnormal bright echoes. Echocardiography showed thickening of the pericardium, may help differentiate constrictive pericarditis, but the interval between the two contradictory movements can be seen. Cardiac hypertrophy usually occurs in restrictive cardiomyopathy. Magnetic resonance imaging (MRI) can show abnormal myocardial lesions infiltrating myocardial structure (such as amyloid or iron).
Cardiac catheterization is usually required and myocardial biopsy. Increased atrial pressure was significantly associated with Y-tilt, medical education network to collect. Finishing ventricular diastolic pressure curve shows the drop of early diastolic followed was a high platform. Unlike constrictive pericarditis, left ventricular diastolic pressure often higher than the right ventricle of several mmHg. Angiography showed normal ventricular cavity size, normal or reduced systolic shortening. Myocardial and papillary muscle infiltration or intimal thickening can cause functional atrioventricular valve regurgitation. Biopsy can confirm that fibrosis and thickening of the endocardial, myocardial iron or amyloid infiltration, or chronic myocardial fibrosis. Normal coronary arteries, in addition to the rare involvement of epicardial coronary arteries than patients with amyloidosis.
Should look for restrictive cardiomyopathy due to primary diseases (such as rectal biopsy in the diagnosis of amyloidosis, iron studies or biopsy hemochromatosis).
Prognosis and treatment
Poor prognosis, with dilated congestive cardiomyopathy similar.
Most patients without appropriate treatment, diuretics reduce preload should be used with caution, because of lack of compliance of the ventricle is the front load to maintain cardiac output. Digitalis almost does not change the hemodynamic abnormalities of amyloid myopathy change of heart there is a danger, because of digitalis often extremely sensitive. Afterload reduction drug can cause significant hypotension, often have little value.
Periodic phlebotomy to reduce body iron storage operation, hemochromatosis can be improved. Biopsy confirmed the activity of meat samples Liubing patients effectively with corticosteroids. High eosinophilia syndrome acute patients on corticosteroids and cytotoxic drugs (such as hydroxyurea) responded. Chronic phase, almost no proliferation of endocardial fibrous or elastic tissue in the surgical removal of patients Loffler endocardial thickening and fibrosis and thrombotic release after the tendons and heart valves can be improved. Sometimes atrioventricular valve replacement for severe functional atrioventricular regurgitation useful. There was some compensatory hypertrophy, calcium antagonists may have some value. During the start of treatment to confirm the hemodynamic effect is worth considering.


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