Primary cardiomyopathy-What is a primary cardiomyopathy?
Primary cardiomyopathy is a group of slow onset, the cause is unknown, the characteristics of cardiac enlargement, and finally the development of heart failure heart disease. Initially may be no discomfort, shortness of breath, palpitations, chest tightness, difficulty breathing and other symptoms after tiredness or mild labor. Increase in heart weight, expansion of the various chambers of the heart, myocardial gray and loose; near-normal ventricular wall thickness, endocardial thickening, heart chamber enclosing the mural thrombus, often myocardial fibrosis, but also satisfying the wall into a piece of damaged cardiac pacing system can also be invaded.
Primary cardiomyopathy - Disease OverviewPrimary cardiomyopathy primary cardiomyopathy is a group of unknown cause heart disease cardiomyopathy. The disease is divided into three types: ① The dilated cardiomyopathy: the type characteristic for the left or right ventricle or both ventricles expand, and associated with cardiac hypertrophy, ventricular systolic dysfunction, with or without congestive heart failure, arrhythmia common condition was progressively increased, the death of the disease can occur at any stage, this type is most common, accounting for 70% to 80%. The ② hypertrophic cardiomyopathy: ventricular hypertrophy, typical in the left ventricular septal staggering, even showed concentric hypertrophy, normal or reduced left ventricular cavity volume, and occasionally lesions in the right ventricle, usually as an autosomal dominant inheritance. The type is more common, accounting for about 10% to 20%. ③ restrictive cardiomyopathy: invasive or non-invasive lesions of this type is characterized by primary cardiomyopathy, or myocardial endocardial fibrosis, diastolic dysfunction caused by obstruction of cardiac filling, very rare.Primary cardiomyopathy - disease etiologyPrimary cardiomyopathy dilated cardiomyopathy(1) virus infection, the immune response theory: that cardiomyopathy is caused by the autoimmune myocardial damage after viral infection, dilated cardiomyopathy, the incidence of doctrine.(2) intramyocardial small coronary artery lesions caused by spasm and the obstruction caused.(3) some cases there is a lack of certain enzymes in the process of myocardial metabolism may be one of the mechanisms.(4) in some cases and nutritional disorders, such as the lack of trace element selenium in food may cause myocardial changes.Hypertrophic cardiomyopathy that disease and genetic.Restrictive cardiomyopathy of unknown etiology.Primary cardiomyopathy - signs of illnessPrimary cardiomyopathy dilated cardiomyopathyApex beat to the left under the shift can be flattering beats the heart dullness to the left to expand, you can often hear the third and fourth heart sounds, rapid heart rate was gallop, mitral or tricuspid valve auscultation area can be heard systolic murmur hair samples, improved the heart function of the noise, reduce the blood pressure is normal, but patients with advanced blood pressure can drop small, pulse pressure, heart failure, diastolic blood pressure increased slightly, heart failure can occur when alternating pulse The pulse is often weakened, can smell the end of the lungs and moist rales, and visible enlargement of the liver, edema, or even chest, ascites, and a variety of arrhythmias may occur, led by the key performance, and a variety of arrhythmias in combination a complex arrhythmia can occur repeatedly. In addition, there are signs of embolism in the brain, kidneys, lungs, etc..(2) common signs of hypertrophic cardiomyopathy heart dullness to the left to expand the apex beat to the left shift, visible lifting of the impulse or apex pulsation; left sternal border apex can be heard inside the contraction of the mid-or late injection noise to the apical spread, may be associated with a systolic thrill, seen in patients with left ventricular outflow tract obstruction. Increase myocardial contractility or reduce the cardiac load measures (such as giving digitalis, isoproterenol, amyl nitrite, nitroglycerin, do the Valsalva maneuver, manual labor or premature beats) may make the noise enhancement; where decreased myocardial contractility or increase cardiac load measures (such as giving vasopressors, beta blockers, squat, clenched fist, etc.) can make the noise diminished to about half of patients could be heard and mitral regurgitation murmur; the second heart sound was unusual split, exhale significantly (due to left ventricular spurting blocked aortic valve delay the closure due to). Arrhythmia to atrial fibrillation. Non-obstructive hypertrophic cardiomyopathy only mild heart increases, the pulmonary valve sound split, anomalous reverse split, less and less noise.Lesions of restrictive cardiomyopathy with left ventricular-based can be the performance of left heart failure and pulmonary hypertension, such as could be heard the end of the lungs and rales, pulmonary second sound hyperthyroidism, etc.; lesions with right ventricular-based performance of the right ventricle returning blocked, such as neck vein distention, the inspiratory expansion of the (library's sign), hepatomegaly, leg edema, ascites, apex beat often weakened, mild increase of cardiac dullness, heart sound is low and blunt, heart rate fast, diastolic gallop and arrhythmias, pericardial effusion can exist, may have visceral embolism.
Primary cardiomyopathy - the clinical symptomsPrimary cardiomyopathy dilated cardiomyopathyMainly in congestive heart failure, which the shortness of breath and swelling are the most common. Initially in labor or exertion, shortness of breath, shortness of breath after mild activity or rest when, or paroxysmal nocturnal shortness of breath, and common dizziness, chest pain and other symptoms, a small number of patients with syncope, a variety of arrhythmias are visible , but also the occurrence of embolism and sudden death.(2) hypertrophic cardiomyopathyOnset of hypertrophic cardiomyopathy and more slowly. About 1/3 of patients have a family history, symptoms mostly start in before the age of 30, men and women suffering from the same. The main symptoms are: (1) difficulty in breathing, after more than tired; ② precordial pain, it resembles the tired, like angina, but may from typical; (3) fatigue, dizziness and fainting, and more in the activities; ④ palpitations; ⑤ The heart failure, more common in patients with advanced, often associated with atrial fibrillation.(3) restrictive cardiomyopathyRare and occurred in the south, was sporadic distribution, the onset is relatively slow. Early may have fever, and the gradual emergence of fatigue, dizziness, shortness of breath, shortness of breath than dilated cardiomyopathy lighter, lower extremity edema, ascites for the outstanding performance.Primary cardiomyopathy - disease diagnosisThe diagnosis of primary cardiomyopathy primary cardiomyopathy need except for other causes of heart disease. Such as clinical enlargement of the heart, arrhythmia, syncope, congestive heart failure, without a common rheumatic heart disease, coronary heart disease, congenital heart disease, high blood pressure, pulmonary heart disease or pericardial diseases should be considered when the disease. Further asked in detail about the history and physical examination, echocardiography, X-ray examination, electrocardiogram, myocardial enzymes and other tests to help diagnosis and classification of a definite diagnosis is sometimes dependent on myocardial biopsy, cardiac angiography.Primary cardiomyopathy - differential diagnosisPrimary cardiomyopathy, rheumatic heart diseaseCardiomyopathy can also be a systolic murmur of mitral or tricuspid valve, but generally not associated with diastolic murmur, and heart failure occurs when the noise than the ring, and heart failure after controlling noise weaken or disappear, and rheumatic heart disease, dilated cardiomyopathy is often suspicious of the cavity at the same time increases, unlike rheumatic heart disease, enlarged left atrium, left ventricle or right ventricular-based. Echocardiography contribute to the difference.(2) pericardial effusionCardiomyopathy apex beat to the left shift, in line with the left outer edge of the cardiac dullness; pericardial effusion the apex beat is often not obvious or in the left outer edge of the inside of the heart dullness. Ultrasound pericardial effusion see large amounts of liquid level in the pericardial segment or dark areas; cardiomyopathy, mainly for the expansion of the heart, although can also be a small amount of pericardial effusion, but not enough to cause cardiac tamponade, nor to affect the heart function and signs, in addition to cardiomyopathy were more audible heart murmur, electrocardiogram, see ventricular hypertrophy, abnormal Q spread a variety of complex arrhythmias, significant abnormality in cardiomyopathy, pericardial disease, normal systolic time interval (STI).Hypertensive heart disease, cardiomyopathy, may have a temporary blood pressure, but diastolic blood pressure does not exceed 14.67kPa (110mmHg), and acute heart failure, heart failure improves drop in blood pressure, eyes, urine, normal renal function tests.Coronary heart disease between the two is often difficult to identify, hypertension, high cholesterol or diabetes risk factors, wall activity was segmental abnormal coronary artery disease. A clear distinction between sometimes have to rely on coronary angiography.(5) the majority of congenital heart disease has obvious signs, it is easy to identify. Ebstein malformation of the noise of the tricuspid area, and can gallop, diminished heart sounds, right heart dilatation and failure, to identify with cardiomyopathy, but the former appears in the early years, left ventricular, cyanosis; ultrasound echocardiography can confirm the diagnosis.Secondary cardiomyopathy systemic diseases such as systemic lupus erythematosus, scleroderma, hemochromatosis, amyloidosis, glycogen storage disease, neuromuscular diseases, has its primary disease manifestations can be identified. In particular, the difference with myocarditis, acute myocarditis often occurs in viral infection at the time or shortly thereafter, chronic myocarditis and cardiomyopathy is more difficult to distinguish between the endomyocardial biopsy of some help. Followed by laboratory tests and antibodies, complement fixation or hemagglutination inhibition test was positive, coronary angiography negative helps to identify.Primary cardiomyopathy - pathophysiologyPrimary cardiomyopathy dilated cardiomyopathyIncrease in heart weight, expansion of the various chambers of the heart, myocardial gray and loose; near-normal ventricular wall thickness, endocardial thickening, heart chamber enclosing the mural thrombus, often myocardial fibrosis, but also satisfying the wall into a piece of damaged cardiac pacing system can also be invaded.Myocardial fiber hypertrophy, nuclear condensation, deformation or disappearance of intracytoplasmic vacuolization can be seen under the microscope. Increase in fibrous tissue, cardiac muscle fibers can be split by the cord-like fibrous tissue, collagen and elastic fibers in the endocardium also increased, showing varying degrees of degeneration, myocardial cells dissolve, especially in cases of long duration. Electron microscope, swelling of mitochondria of myocardial cells, cristae, or disappear, muscle serosal clearance to expand the fibrous material, and granular lipofuscin; the myofibrillar can disappear.(2) hypertrophic cardiomyopathy① hypertrophic obstructive cardiomyopathy: prominent septal high degree of hypertrophy to left ventricular cavity, contraction caused by left ventricular outflow obstruction.② hypertrophic non-obstructive cardiomyopathy: septal hypertrophy to a lesser extent, systolic left ventricular outflow tract without causing obvious obstruction.(3) restrictive cardiomyopathyIn invasive disease caused by restrictive cardiomyopathy, amyloidosis, sarcoidosis, hemochromatosis, glycogen storage disorders such species.
Primary cardiomyopathy - disease surveillancePrimary cardiomyopathy ECGA dilated cardiomyopathy, cardiac enlargement, mainly myocardial damage and cardiac arrhythmias. Left ventricular hypertrophy more common, often associated with myocardial strain, the late right ventricular hypertrophy, left or right atrial hypertrophy; often ST segment depression, T wave flat, two-way or upside down, 10% of patients with pathologic Q wave (more parts of the first interval of the V1, V2 lead), the common atrial and ventricular premature beats, atrial fibrillation, bundle branch block, atrioventricular block and arrhythmias.Hypertrophic cardiomyopathy more than 80% of patients with ST-T changes, a few seen huge inverted T wave; 60% of patients had left ventricular hypertrophy signs; visible abnormal Q wave in I, aVL, V5 and V6 leads Q-wave deep rather than wide, sometimes in II, III, aVF, V1 and V2 leads can also be Q-wave left atrial wave abnormalities; about 25% of patients: some patients with WPW syndrome.The low voltage of restrictive cardiomyopathy, atrial or ventricular hypertrophy, bundle branch block, the ST-T changes, atrial fibrillation, can also be abnormal Q wave in V1 and V2 leads.X-ray examinationA dilated cardiomyopathyGenerally increases the heart, was spherical, resembling a pericardial effusion; a small number of increases to the left ventricle, left atrium or right ventricle, similar to the appearance of mitral valve disease. Seen under fluoroscopy heart beat than normal for the weak, the aorta is generally not expansion. Longer course of pulmonary congestion and pulmonary interstitial edema often also common pleural effusion.(2) hypertrophic cardiomyopathyOrdinary chest X-ray to see increased left ventricle, but also in the normal range, late left atrial enlargement, pulmonary congestion. The aorta does not expand, the X-ray or radionuclide angiocardiography may show septal thickening, reduced left ventricular cavity. Radionuclide myocardial scan display cardiac hypertrophy of the location and extent.(3) restrictive cardiomyopathyVisible heart shadow to expand the shadow of the endomyocardial calcification, ventricular angiography shows the ventricular cavity narrow.Primary cardiomyopathy echocardiographyA dilated cardiomyopathyEarly you can see the chambers of the heart were enlarged, especially in left ventricular wall motion decreases, the late chambers of the heart to expand, the interventricular septum and left ventricular posterior wall movement weakened, the mitral valve the Ye Shuangfeng may disappear, but before and after the leaves are different to the activities , left ventricular-spurting index often reduced to less than 50%, myocardial shortening to reduce the number of a small amount of pericardial effusion.(2) hypertrophic cardiomyopathyVisible asymmetric septal hypertrophy, septal thickness and left ventricular posterior wall thickness ratio greater than 1.3:1; of mitral valve systolic forward; left ventricular cavity narrow outflow tract obstruction; left ventricular diastolic dysfunction. including compliance, reduced rapid filling time, Yung-time extension.(3) restrictive cardiomyopathyVisible endocardial thickening, apical ventricular cavity occlusion, myocardial endocardial structure density anomaly ultrasound echo wall motion decreased. Cardiomyopathy early myocardial enzymes check the activity increased, especially in the creatine kinase isoenzyme (CPK-MB), lactate dehydrogenase isoenzyme (of LDH1) increased valuable.Cardiac catheterizationA dilated cardiomyopathyEarly near-normal left and right ventricular end-diastolic pressure can be slightly increased, heart failure, cardiac index decreased, arteriovenous oxygen difference, pulmonary artery and left atrial pressure increased. Ventricular angiography showed a heart chamber to expand the weakened wall motion.(2) hypertrophic cardiomyopathyShow ventricular end-diastolic pressure, left ventricular outflow tract obstruction by systolic pressure difference between the ventricular chamber and outflow tract.(3) restrictive cardiomyopathyShow ventricular end-diastolic pressure gradually increased, resulting in subsidence after the platform wave, mainly in the left ventricle to pulmonary artery pressure increased, mainly in the right ventricular right atrial pressure, right atrial pressure curve in a significant wave of v replaced by a wave.
Primary cardiomyopathy - treatmentPrimary myocardial disease of unknown etiology of the disease, there is no effective therapy. Mainly for the treatment of myocardial damage, heart failure, arrhythmias and thromboembolism. Different pathological changes in the symptomatic treatment was also different.A dilated cardiomyopathyControl heart failure, prevention of cardiac arrhythmias, prevention of embolism.(1) General treatment: to avoid fatigue, adequate rest, heart failure should rest in bed, the infection should be actively control the infection and to prevent disease progression.(2) to correct ventricular dysfunction:① cardiac glycosides: Gexin to 0.125 ~ 0.25mg, orally, once daily. Attention to this disease due to extensive myocardial damage, digitalis drugs easy to poisoning, should be reduction application.② Non-digitalis cardiac drugs: dobutamine per minute 2.5μg ~ 10μg/kg speed static point 1 to 2 minutes after administration of onset. Ammonia pyrazole ketone intravenous loading dose of 1.5 to 3mg/kg per minute 10μ/kg intravenous infusion to maintain oral 100mg, 3 times a day.③ vasodilators: isosorbide dinitrate 5 ~ 10mg, 3 to 4 times daily, open Capoten 12.5mg ~ 25mg, 3 times a day, severe cases can be used sodium nitroprusside 25 to 50μg / static point.④ Diuretics: Hydrochlorothiazide 25 ~ 50mg, 1-3 times daily; triamterene 50 ~ 100mg, 1 to 3 times a day; edema or acute left ventricular failure furosemide can be used when oral or intravenous administration.(3) to correct the arrhythmia:① atrial premature beats, atrial fibrillation: dilated cardiomyopathy can be used to Gexin 0.125mg, oral, once daily, the ventricular rate is too fast, plus the service of small doses of beta blockers such as atenolol 6.25mg 2 or 3 times.The ② frequency or multiple derived PVCs or ventricular tachycardia, available mexiletine 100 ~ 200mg, 3 to 4 times daily; ethylamine iodine furosemide ketone 0.1 ~ 0.2g, 3 times a day. Intravenous drug use is optional, lidocaine, procainamide, propafenone, bromo benzylamine. Note that the anti-arrhythmic drugs on cardiac inhibition. Medical therapy, endangering the life or consciousness where forfeiture could be considered a shock cardioversion.③ slow arrhythmias may consider the installation of artificial cardiac pacemaker.Primary cardiomyopathy - prognosis processingCourse of primary cardiomyopathy dilated cardiomyopathy of varying lengths short by death within 1 year after the onset of the elderly can survive for more than 20 years. About 50% of the patients died due to heart failure and cardiac arrhythmias in 3 to 5 years. Where the heart is significantly expanded, heart failure, persistent or refractory arrhythmia poor prognosis. There are many patients with sudden death.Hypertrophic cardiomyopathy is relatively slow progression, prognosis is uncertain, can be stabilized unchanged for years, but once the symptoms gradually deteriorated. Sudden death and heart failure as the leading cause of death. A small number of patients with infective endocarditis complicated by thrombosis.The course of the development of restrictive cardiomyopathy is slow or fast, the prognosis is slightly better than the main left ventricular lesions than right ventricular disease. In recent years, surgical treatment of this type have a certain hope.
