心肌疾病（英文）课件

,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,Cardiomyopathy and myocarditis,Tang Qizhu,Tel:88041911-6604 Email:qizhutyahoo,Renmin Hospital of Wuhan University,Cardiomyopathy and myocar,1,Difinition,Cardiomyopathy is a serious disease in which the heart muscle becomes inflamed and doesnt work as well as it should,It can be classified as primary or secondary. Primary cardiomyopathy cant be attributed to a specific cause, such as high blood pressure, heart valve disease, artery diseases or congenital heart defects. Secondary cardiomyopathy is due to specific causes,DifinitionCardiomyopathy is a,2,Clasification,(2019 WHO/ISFC),There are fourmain types,:,Dilated cardiomyopathy,Hypertrophic cardiomyopathy,Restrictive cardiomyopathy,Arrhythmogenic,right ventricular,dysplasia,Clasification(2019 WHO/ISFC)Th,3,Dilated cardiomyopathy,This type of cardiomyopathy is characterized by a dilatation and impaired contraction of the left or both ventricles,Dilated cardiomyopathy This ty,4,Importances,The incidence and prevalence of cardiomyopathy appears to be increasing. The reported incidence is 0.013-0.084%,Cardiomyopathy is an important cause of morbidity and mortality among the worlds aging population,Sex: men/women is 2.5/1,Age:,All age groups are affected. However, studies suggest that 50% of patients with new onset of disease are younger than 2 years,ImportancesThe incidence and p,5,CAUSES,Cardiomyopathies have many causes, including nutritional deficiencies, deposits in the heart muscle associated with medical conditions, anemia, stress, viral infections, alcoholism, coronary artery disease, and others,There is no identifiable cause, although it is suspected to be an end stage of myocarditis,CAUSES Cardiomyopathies have m,6,SYMPTOMS,Symptoms often develop gradually and usually include symptoms of right heart failure and/or left heart failure,Because the body compensates for dilated cardiomyopathy, the disease may have no symptoms initially. As the condition worsens, the heart may perform normally when a person is resting, but may cause symptoms during periods of exercise or psychological stress,SYMPTOMS Symptoms often develo,7,symptoms,fatigue,shortness of breath on exertion, orthopnea (breathing difficulty when lying down), waking up at night short of breath,swelling of the ankles,excessive urination at night,irregular heartbeat (palpitations-a feeling of racing or skipping of the heart),decreased urine output (may not include at night),symptoms fatigue,8,DIAGNOSIS,A physician diagnoses dilated cardiomyopathy after a physical examination,check for shortness of breath during exercise and weakness,The physician may also hear rales, or wet crackles, through a stethoscope, indicating fluid in the lungs,DIAGNOSISA physician diagnoses,9,Neck and Lungs,Jugular venous distention (as an estimate of central venous pressure),Hepatojugular reflux,Large,cv,wave (observed with tricuspid regurgitation),Crackles (pulmonary rales),Signs of pleural effusion may be noted,Neck and LungsJugular venous d,10,Inspection and palpation,Palpate for heaves, shifted point of maximal impulse, and cardiomegaly (broad and displaced point of maximal impulse, right ventricular heave).,The normal apical impulse should be approximately the size of a quarter and should be located in one (fourth or fifth) intercostal space. The apical impulse is normally within 10 cm of the midsternal line,Inspection and palpation,11,Auscultation,Murmurs (with appropriate maneuvers),tachycardia, S,2,at the base (paradoxical splitting, prominent P,2,), S,3, and S,4,may be noted,An irregularly irregular rhythm (atrial fibrillation) may be noted,Gallops are almost always present in persons with DCM,Auscultation,12,Abdomen and others,Percussion and palpation of the liver may reveal hepatomegaly due to elevated venous pressure, infiltrative disease, hepatojugular reflux, or ascites,Observe for cardiac cachexia, peripheral edema, cyanosis, and clubbing,Abdomen and othersPercussion a,13,TESTS FOR DETECTION,To confirm the diagnosis, the physician may order tests, including:,Blood tests;,Chest x ray;,Echocardiogram;,Electrocardiography (ECG),Cardiac catheterization and angiography,TESTS FOR DETECTION To confirm,14,X-ray,This very large heart has a globoid shape because all of the chambers are dilated. It felt very flabby, and the myocardium was poorly contractile,X-rayThis very large heart has,15,Doppler Echo,Marked dilation of the left ventricle with global hypokinesia is the hallmark of the disease. Left ventricular ejection fraction 50%. Left ventricular walls are thin,and areas of dyskinesis,may be observed. The left,atrium is also dilated.,varying degrees of mitral,regurgitation,Doppler Echo Marked dilation o,16,Electrocardiogram,ECG changes are usually nonspecific,Low voltage complexes,Presence of Q waves and inversion of T waves in leads I, II, aVL, and V,4,through V,6,(anterolateral infarction pattern),Significant arrhythmia,Electrocardiogram,17,Myocardial biopsy,The number of biopsy specimens collected should be limited to the minimum required (usually 4-8),Myocyte hypertrophy and fibrosis without lymphocytic infiltrate,Myocardial biopsy The number o,18,TREATMENTS,Hospitalization may be required of patients when symptoms of dilated cardiomyopathy are severe. Treatment for dilated cardiomyopathy is focused on relief of symptoms, just as is for other types of cardiomyopathy, and is essentially the same as the treatment of heart failure,TREATMENTS Hospitalization may,19,Medications,Medications may include digitalis,Angiotensin converting enzyme (ACE) inhibitors,Anticoagulants,Beta-blockers,Calcium channel blockers, Vasodilators,diuretics, nutritional supplements, or other cardiac medications,Medications Medications may i,20,Surgical Care,A heart transplant may be considered if heart function is very poor,Surgical Care A heart transpla,21,Diet and others,A low-salt diet may be recommended to patients and fluid intake may be restricted in some cases.,Physical activity may be restricted as symptoms progress,Smoking and alcohol cessation recommendations may be given, because these habits make symptom of dilated cardiomyopathy worse,Diet and others A low-salt di,22,COMPLICATIONS,Congestive heart failure,Cardiac arrhythmias, including lethal arrhythmias,Pulmonary (lung) edema,Total failure of the heart to function (circulatory collapse),Side effects of medications, including low blood pressure (hypotension), light headedness, fainting, lupus reaction, headache, GI upset, and digitalis toxicity,COMPLICATIONSCongestive hear,23,HYPERTROPHIC CARDIOMYOPATHY,Hypertrophic cardiomyopathy is a primary cardiac disease characterized by an inappropriate myocardial hypertrophy, which occurs in the absence of an obvious hemodynamic load such as aortic stenosis or systemic hypertension,HYPERTROPHIC CARDIOMYOPATHYHyp,24,HISTORY AND OTHER NAMES,Hypertrophic Cardiomyopathy was first recognised in the late 1950s. The condition has been known by a number of names including Hypertrophic Obstructive Cardiomyopathy (HOCM), Idiopathic Hypertrophic Sub-aortic Stenosis (,IHSS),and Muscular Sub-aortic Stenosis. The general term,Hypertrophic Cardiomyopathy (HCM),is now most widely used,HISTORY AND OTHER NAMES,25,THE CAUSE,The cause of Hypertrophic Cardiomyopathy is not yet known. In the majority of cases the condition is inherited. In others there is either no evidence of inheritance or there is insufficient information about the individuals family to assess inheritance. In affected families the condition usually passes from one generation to the next and generations are not skipped,THE CAUSEThe cause of Hypertro,26,A Family Tree,A Family Tree,27,NEW DISCOVERIES,Recently research has identified abnormalities in at least 6 related genes that are important in the development of heart muscle cells. In approximately 50-60% of families, affected individuals are found to have a mutation in the gene for,myosin,troponin T, alpha tropomyosin, cardiac myosin binding protein-C, or the essential and regulatory light chains. These are important proteins for the contraction of the heart,NEW DISCOVERIES Recently resea,28,pathology,an excessive thickening of the muscle.,Abnormal myocardial fiber arrangement which interferes with the proper diastolic sequence of relaxation.,Abnormal myocardial calcium ion handling resulting in high cellular calcium ion concentration in the region of myofibrillar contractile proteins and delayed re-uptake of calcium ion by the sarcoplasmic reticulum resulting in prolonged relaxation.,Ischemia.,Myocardial fibrosis,pathologyan excessive thickeni,29,心肌疾病（英文）课件,30,LEFT VENTRICULAR OUTFLOW TRACT (LVOT) OBSTRUCTION,Approximately 25% of patients with hypertrophic cardiomyopathy manifest variable degree of LVOT obstruction. In some, the obstruction or gradient is absent at rest but this can be provoked by exercise or other physiologic or pharmacologic means,LEFT VENTRICULAR OUTFLOW TRACT,31,mechanism of LVOT obstruction,Asymmetric Septal Hypertrophy,mechanism of LVOT obstruction,32,mechanism of LVOT obstruction,Asymmetric Septal Hypertrophy with Obstruction,mechanism of LVOT obstructio,33,The mechanism of LVOT obstruction,The thick upper septum protrudes in the LVOT reducing the orifice size. Blood flowing through this narrowed LVOT causes a Venturi (suction) effect which draws the anterior leaflet of the mitral valve towards the septum, further reducing the LVOT orifice size. Simultaneously, as a result of systolic anterior movement of the mitral valve (SAM), there is functional mitral regurgitation,The mechanism of LVOT obstruct,34,symptom,dyspnea,:Shortness of breath,E,xercise capacity may be limited by breathlessness and fatigue,Chest pain,pain may occur at rest or during sleep and may persist,Palpitation,Syncopy/,dizziness,Sudden death,symptom dyspnea :Shortness of,35,PHYSICAL EXAMINATION,The systolic murmur is characteristically crescendo-decrescendo and starts a little after the onset of S1. It is best heard at the left lower sternal border and radiates to the base, but unlike systolic murmur of valvular aortic stenosis, it does not radiate to the carotids.,PHYSICAL EXAMINATION,36,murmur,The intensity of the murmur can be varied by various physiologic and pharmacologic interventions. Tthe murmur (and gradient) can be intensified by decreasing preload (decrease venous return) by abrupt assumption of upright posture or by Valsalvas maneuver. The murmur intensity can be diminished by increasing afterload (squatting), or by increasing preload (leg raising in supine position),The murmur of mitral regurgitation is prominent in the apical region and shows the same directional changes in response to the interventions noted above,murmurThe intensity of the mur,37,Electrocardiogram or ECG,Main features include left ventricular hypertrophy (LVH) pattern and the presence of prominent Q waves which resemble Q waves of myocardial infarction (pseudo-infarction pattern). Ambulatory electrocardiogram may reveal atrial and ventricular arrhythmias including non-sustained ventricular tachycardia (NSVT),Electrocardiogram or ECG,38,Echocardiogram or ECHO,include hypertrophic walls, ASH, and septal wall to posterior wall ratio of 1.3:1. In patients with HOCM, there is presence of SAM. Left ventricular chamber is normal or small; left atrium may be enlarged. Color Doppler studies provide additional hemodynamic information,Echocardiogram or ECHOinclude,39,Cardiac Catheterisation,shows a gradient between the main left ventricular chamber and the subaortic chamber proximal to the obstruction. This pull back tracing differentiates obstructive hypertrophic cardiomyopathy from aortic stenosis,Cardiac Catheterisationshows a,40,Genetic testing,Genetic testing may play an important role in helping doctors rule out other diagnoses. For example, in patients who have a small increase in wall thickness (like trained athletes with ventricular hypertrophy), and some patients with constant high blood pressure who are thought to have HCM,Genetic testingGenetic testing,41,TREATMENT,Drug Treatment,Drug treatment or medication is primarily given when a person has some or all of the symptoms described earlier,Alcohol Septal Ablation,A catheterization is performed, alcohol is injected into the septum through a small coronary artery. This causes a controlled myocardial infarction,Pacemaker,dual chambered pacing would relieve obstruction, eliminate most symptoms and thin the walls of the heart,S,urger,y,re,moving a small amount of muscle from the ventricular wall,TREATMENT,42,Drug Treatment,Beta-Blockers,These drugs reduce heart rate, decrease myocardial contractility, prolong diastolic filling time and possess anti-arrhythmic properties especially against exercise- induced arrhythmias,Calcium Antagonists,Anti-Arrhythmic Drugs,Anticoagulants,Diuretics,Antibiotics,Drug Treatment,43,Dual Chamber Pacemaker,It is believed that pacemaker therapy alters the sequence of contraction which reduces LVOT gradient and improves symptoms,Long term benefits, however, are questionable,Dual Chamber Pacemaker,44,Restrictive cardiomyopathy,It is a disorder of the heart muscle in which the walls of the ventricles become stiff, but not necessarily thickened, such that they resist normal filling with blood. There is no cure. Treatment aims to ameliorate symptoms caused by the backup of blood into the lungs and veins of the neck and liver,Restrictive cardiomyopathyIt i,45,Symptoms,Patients with RCM present with signs and/or symptoms of heart failure such as fatigue, shortness of breath, tissue swelling (oedema) and abdominal enlargement,Up to a third of patients may present with an embolic complication. Abnormal heart rhythms and palpitations are common whatever the underlying cause of the disease,SymptomsPatients with RCM pres,46,diagnosis,The diagnosis of RCM is usually based a physical examination, an electrocardiogram (ECG) and an echocardiogram. Magnetic resonance imaging (MRI) can provide additional information about the structure of the heart. In some patients a precise diagnosis may require catheterisation of the heart to measure pressures and to perform a biopsy of the heart muscle (removal and microscopic examination of a specimen), which may enable the doctor to identify the infiltrating substance,diagnosisThe diagnosis of RCM,47,Treatment,As the onset of symptoms in RCM is often very insidious, the diagnosis of RCM may be made late in the course of the disease. There is no specific treatment for RCM, and the aims of medical therapy are to improve symptoms of heart failure, treat cardiac rhythm disturbance and to prevent thromboembolism. Pacemakers are indicated for patients with slow heart rates or heart block,TreatmentAs the onset of symp,48,Arrhythmogenic right ventricular dysplasia,ARVD is a rare type of,cardiomyopathy,in which the muscle tissue in the lower-right chamber of the heart (right,ventricle,) dies. The dead muscle tissue is replaced by fat,Patients can develop dangerous,arrhythmias,or even go into,cardiac,arrest, particularly when under physical or emotional stress. Studies have shown that ARVD is a significant cause of sudden cardiac,death among young athletes,Arrhythmogenic right ventricul,49,Names,Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC),Even though this disease has been recognised only recently, These are not formal guidelines for assessment or treatment,Names Arrhythmogenic Right Ven,50,Muscle cells become disorganised and are replaced by fibrous and fatty tissue,Muscle cells become disorganis,51,Patchy involvement of the right ventricle may lead to one or more sites of abnormal electrical activity,Patchy involvement of the righ,52,SYMPTOMS of ARVC,Palpitation,light-headedness,fatigue,blackout or collapse or,syncope,symptoms of,heart failure,SYMPTOMS of ARVCPalpitation,53,RHYTHM DISTURBANCES,Ventricular ectopics (VE/VPB/VPC),Ventricular tachycardia (VT),Ventricular fibrillation (VF),Atrial fibrillation (AF),Sudden death,RHYTHM DISTURBANCES,54,TREATMENT,At present there is no cure for ARVC. Clinical management aims to identity people at risk of complications and then to use prophylactic therapy to try to prevent these,Drug treatment,Cardioversion,Ablation,Implantable Cardioverter Defibrillator (ICD),TREATMENT,55,myocarditis,Myocarditis is inflammation of heart muscle,Myocarditis can be caused by a variety of conditions such as a virus,sarcoidosis, and immune diseases (such as systemic lupus, etc.), pregnancy, and others. The most common cause of myocarditis is infection of the heart muscle by a virus, Coxsackie B,myocarditisMyocarditis is inf,56,symptoms of myocarditis,Myocarditis can be mild and cause virtually no noticeable symptoms. When it is more serious, it leads to weakening of the heart muscle. Myocarditis can then cause heart failure (with symptoms of shortness of breath, fatigue, fluid accumulation in the lungs, etc.) as well as heart rhythm irregularities from inflammation and/or scarring of the electrical system of the heart,symptoms of myocarditisMyocard,57,Treatment,Except in systemic sarcoidosis and immune inflammation (such as from systemic lupus) where myocarditis can respond to corticosteroids, no proven effective medications are currently available for treating myocarditis. Current treatment of myocarditis mainly involves alleviating heart failure (salt restriction, water pills,ACE inhibitors, etc.) and treating as well as monitoring heart rhythm abnormalities,TreatmentExcept in systemic sa,58,心肌疾病（英文）课件,59,