（内科学课件）07心肌病心肌炎心包炎课件

1 Cardiomyopathy 周晓莉 重医附一院心内科 2016.10 2 Definition Disease of the myocardium associated with cardiac dysfunction. not the result of disease or dysfunction of other cardiac structures myocardial infarction, systemic hypertension, valvular stenosis or regurgitation” 3 Classification (WHO 1995) accepted world-wide primary 1.Dilated Cardiomyopathy (DCM) 2.Hypertrophic Cardiomyopathy (HCM) 3.Restrictive Cardiomyopathy (RCM) 4.Arrhythmogenic right ventricular Cardiomyopathy (ARVC) 5.Unclassified Cardiomyopathy Specific Dilated Cardiomyopathy, DCM Dilated cardiomyopathy is a progressive disease of heart muscle that is characterized by ventricular chamber enlargement and contractile dysfunction with normal left ventricular (LV) wall thickness. The right ventricle may also be dilated and dysfunctional. 5 Etiology Inherited disease Infections, viral Toxins Nutritional Infiltrative Metabolic genetic mutations the current 5 (LMNA, MYH7, TNNT2, SCN5A, MYBPC3) novel mutations (TTN, BAG3) in familial dilated cardiomyopathy. Finding a specific cause for an individual case may be difficult. 6 Epidemiology The true incidence of cardiomyopathies is unknown. 19 cases per 100,000 in China, recently reported. 3-10 cases per 100,000; 20,000 new cases per year in the U.S.A. 7 Pathology Increased heart size and weight Ventricular dilatation, normal wall thickness Fibrosis Thrombosis 8 Fig 1 Dilated Cardiomyopathy Dilated heart, wall thickness 9 Fig 2 Dilated Cardiomyopathy Myocardial fibrosis 10 Fig 3 Dilated Cardiomyopathy Thrombosis 11 Pathophysiology Early compensatory mechanisms lead to further myocardial injury, dysfunction, and geometric remodeling Neurohormonal activation Circulating cytokines as mediators of myocardial injury 12 Clinical manifestation Occurs in any age, esp. in 3050 years old. Men 2 times more frequent than women. The incidence is about 4-8 per 100 000 person- years and the prevalence 36.5 per 100 000 individuals. Symptoms may be gradual. 13 Idiopathic Dilated Cardiomyopathy Observed Survival of 104 Patients Years Am J Cardiol 1981; 47:525 14 Symptoms heart failure pulmonary congestion (left HF) dyspnea (exertional, at rest, paroxysmal nocturnal dyspnea, orthopnea) systemic congestion (right HF) edema, nausea, abdominal pain, nocturia low cardiac output fatigue and weakness Arrhythmias (VPB,VT,AF) Cardiac sudden death 15 Signs cardiac dilatation q The 3rd,4th heart sounds q Gallop rhythm q Mitral and tricuspid regurgitation murmurs Embolization 16 Auxiliary examination Echocardiography big chamber sizes, normal wall thickness, relatively smaller valvular cavity, lower amplitude of the heart pulsate and thrombosis. Chest radiographs cardiomegaly, Kerley B lines, alveolar edema. Electrocardiography Nonspecific Catheterization Endomyocardial biopsy 17 Two-dimensional echocardiography in DCM The heart chambers are dilated. 18 M-model echocardiography in DCM EPSS 19 Doppler echocardiography in DCM Regurgitation of the mitral and tricuspid valve. 20 Two-dimensional echocardiography in DCM Enlarged heart. Lateral thrombus is shown at the LV apex. 21 Fig 4 the enlarged heart 22 Diagnosis 1. The symptoms and signs of systolic dysfunction. 2. The typical presentation of echocardiogram. 3. Rule out other cardiac diseases. 23 Essentially the same as treatment of chronic heart failure (CHF). Drug classes used include the following: Treatment Angiotensin-converting enzyme inhibitors(ACEI) Angiotensin II receptor blockers (ARB) Beta-blockers Aldosterone antagonists Cardiac glycosides Diuretics Vasodilators 24 Treatment continued Anticoagulants Warfarin Patients in atrial fibrillation, with known mural thrombus, with embolism history or with lower ejection fraction values(30%). Antiarrhythmics Amiodarone Dofetilide Implantable cardioverter defibrilator (ICD) 25 Treatment continued Cardiac resynchronization therapy (CRT) Biventricular pacing with leads positioned in the right atrium, right ventricle, and coronary sinus. The benefits were confirmed in multiple studies from the mid-1990s. 26 Fig 5 Biventricular Pacing The angiography of coronary sinus Position of the lead 27 CRT indications NYHA class II or IV heart failure and intraventricular conduction delay. LBBB，LVEF35、QRS 120 ms. None LBBB, LVEF35、QRS 150 ms. Persistent symptoms despite optimal medical therapy with ACE inhibitors, beta-blockers, and/or other appropriate pharmacologic measures. 28 Treatment continued Surgical Care Left ventricular assist devices Heart transplantation 29 Hypertrophic cardiomyopathy，HCM a genetic disorder that typically is inherited in an autosomal dominant fashion with variable expressivity. Characteristic of the asymmetric myocardial hypertrophy, the obstruction of LV outflow tract and the abnormal diastolic function. 30 Synonyms Idiopathic hypertrophic subaortic stenosis (IHSS) ; asymmetric septal hypertrophy (ASH). 31 Epidemiology Reported in 0.5% of the outpatient population referred for echocardiography. estimated to occur in 0.05-0.2% of the population. Reported 1.3/100,000 in Jiangsu, China. Slightly more common in males than in females. in spite of the autosomal dominant pattern. The ratio is about 1.6:1 in China. 32 Epidemiology Age any age. may show itself during 20-30 years old. Sudden death in children. Family history approximately 25% of first- degree relatives of patients with HCM suffered. 33 Etiology 1. Genetic causes the common mutated-gene located on the long arm of chromosome 14, encoding for beta cardiac myosin heavy chain and chromosome 11,encoding for cardiac myosin binding protein-C. autosomal dominant Mendelian-inherited way in about 50% of cases. sporadic one by spontaneous mutations. phenotypic expression of a given mutation is various. 34 Etiology con 2. abnormal calcium kinetics abnormal calcium fluxes, an increase in intracellular calcium concentration, increase in the number of calcium channels, produce hypertrophy and cellular disarray. 3. Other causes Abnormal sympathetic stimulation Abnormally thickened intramural coronary arteries Subendocardial ischemia Cardiac structural abnormalities 35 Pathology Asymmetric septal hypertrophy( ASH) Septal wall thickness is from 20mm to 52mm. Myocardial cell hypertrophy and disarray. The abnormal intramural coronary arteries. The abnormal mitral valve setup. 36 Fig1 HCM 37 Fig 2 Microscopic section of myocardium in HCM myocyte disarray. Curr Probl Cardiol 2004;29:233-91. 38 Pathophysiology Dynamic pressure gradient across the LV outflow tract. further narrowing of an already small outflow tract. Systolic anterior motion (SAM) of the mitral valve against the hypertrophied septum. Venturi effect Abnormal diastolic function. impairs ventricular filling and increases filling pressure. Subendocardial ischemia Arrhythmia. 39 Fig 3 Schematic drawing of HCM in systole. Mitral leaflet is distorted toward septum (SAM) resulting in outflow tract obstruction and posteriorly directed mitral regurgitation. Curr Probl Cardiol 2004;29:233- 91. 40 Symptom 1.Dyspnea most common. a consequence of elevated LV diastolic filling pressures. 2.Syncope or Presyncope inadequate cardiac output upon exertion or from cardiac arrhythmia. high risk of sudden death. 3.Sudden cardiac death highest incidence in preadolescent and adolescent children, related to extreme exertion. 80% of cases are due to ventricular fibrillation. 41 Symptom con 4.Angina no detectable coronary atherosclerosis. From markedly increased myocardial oxygen consumption. 5.Palpitations and Dizziness common, from arrhythmias. 6.Congestive heart failure in the late stage. 42 Sign q Systolic ejection murmur best heard between the apex and left sternal border. diminish with any increase in preload (eg, Mueller maneuver, squatting), afterload (eg, handgrip) or decrease in heart contractility. increase with any decrease in preload (eg, Valsalva maneuver, nitrate administration, diuretic administration, standing), afterload (eg, vasodilator administration) or increase in heart contractility. 43 Auxiliary examination Echocardiography 1. Septal hypertrophy with septal-to-free wall ratio greater than 1.4:1 (1.31.5:1). 2. A abnormal systolic anterior leaflet motion of the mitral valve. 3. The narrowing of the LV outflow tract 4. Decreased mid aortic flow, and partial systolic closure of the aortic valve in mid systole. 44 Two-dimensional echocardiography in HCM Asymmetric hypertrophy IVS LVPW 45 SAM in HCM CD CD 46 Doppler echocardiogram in HCM The mixed color in the LV outflow tract. 47 Cardiac Imaging con Electrocardiography ST-T wave abnormalities and LV hypertrophy. Differentiated from coronary heart disease. Heart catheter the pressure gradient between LV and outflow tract is above 30mmHg。 48 The ECG changes in HCM ST-T and Q wave changes 49 Diagnosis Echocardiogram can identify. 50 Treatment Medication Beta-adrenergic blocking agents Propranolol, Metoprolol Calcium channel blockers Verapamil, Diltiazem Antiarrhythmics Amiodarone 51 Medication Warning u Avoid inotropic drugs if possible u Avoid nitrates and sympathomimetic amines, except in concomitant coronary artery disease u Avoid digitalis u Use diuretics with caution 52 Treatment Left ventricular myomectomy the benefit is usually sustained. Documented dramatic gap pressure. Pacemaker implantation Dual chamber pacing.The RV septal preexcitation leads to a “pulling away“ of the septum from the outflow region, allowing for a decrease in LV outflow tract obstruction. ICD may needed. Catheter septal ablation a therapeutic infarction of the proximal interventricular septal myocardium. Analogous to a surgical myomectomy. 53 Further outpatient care p Avoid strenuous exercise. p Avoid inappropriate medication usage. p Carefully monitor medication dose and adverse effects. p Diminish the likelihood of sudden death. 54 Thanks for your attention! 55 Myocarditis 56 Definition Myocarditis is defined clinically as inflammation of the heart muscle. with a wide range of clinical presentation, from subtle to devastating. 57 Epidemiology Asymptomatic, so frequency is difficult to ascertain. Incidence usually is estimated at 1-10 per 100,000 persons. According to estimates, as many as 1-5% of patients with acute viral infections may have involvement of the myocardium. 58 Epidemiology con Sex: Incidence is similar between males and females, although young males are particularly susceptible. Age: Patients usually are fairly young. The median age of patients affected with lymphocytic myocarditis is 42 years. 59 Etiology Infection Viral Bacterial, rickettsial, spirochetal Protozoal, Metazoal Fungal Toxic anthracyclines, catecholamines, Interleukin- 2, alpha 2 interferon Hypersensitivity Drugs 60 Pathogenesis 1. Direct cytotoxic effect of the causative agent 2. Secondary immune response, which can be triggered by the causative agent 3. Cytokine expression in the myocardium (eg, tumor necrosis factor -alpha, nitric oxide synthase) 4. Aberrant induction of apoptosis 61 Clinical Manifestations clinically silent Antecedent flulike symptoms accompanied by fever, arthralgia, and malaise. heart failure symptoms Chest pain , palpitation , syncope , cardiac shock. AV block or ventricular arrhythmia Sudden death 62 Auxiliary examination Elevated cardiac enzymes (MYO, CK or CK-MB, AST, LDH). Elevated cardiac troponin (cTn-I or cTn -T) 89% specificity and 34% sensitivity and increases more frequently than creatine kinase MB subunits. 63 Auxiliary examination Electrocardiogram Sinus tachycardia is most common Diffuse ST-T wave changes Ventricular arrhythmias Conduction delay, even complete heart block causing Adams-Stokes attack . 64 Auxiliary examination Echocardiography LV systolic dysfunction is common with segmental wall motion abnormalities LV size is typically normal or mildly dilated Wall thickness may be increased Ventricular thrombi detected in 15% 65 Auxiliary examination Cardiac angiography To rule out coronary ischemia as a cause of new-onset heart failure, especially when clinical presentation mimics acute myocardial infarction. 66 Auxiliary examination Right ventricular endomyocardial biopsy Criterion standard for diagnosis of myocarditis, although limited sensitivity and specificity, as inflammation can be diffuse or focal. The Dallas criteria for biopsy-based diagnosis established in 1987, consist of myocyte necrosis and inflammation. 67 Viral myocarditis: lymphocyte-rich infiltrate 68 Giant-cell myocarditis Diffuse infiltration with numerous giant cells, lymphocytes, neutrophils, and eosinophils. Myocyte damage is also present. 69 Diagnosis Acute myocarditis should be suspected whenever a patient, especially a young male, presents with otherwise unexplained cardiac abnormalities of new onset, such as heart failure, arrhythmias, or conduction disturbances. A history of recent upper respiratory infection or enteritis may also be present. 70 Diagnosis con Congenital, valvular, ischemic, and pulmonary heart disease should be ruled out When it comes to the presumptive diagnosis of cardiomyopathy. Should be made on the basis of the clinical and laboratory presentations. The definitive diagnosis of myocarditis can be made only by endomyocardial biopsy. 71 Treatment Majority of patients have a self-limited disease. Supportive care is the first line of therapy. Bed rest. 72 Treatment con Management of LV dysfunction Consider anticoagulation to prevent thromboembolia. Consider temporary pacer for complete AV block Intensive immunosuppressive therapy (eg, corticosteroids, azathioprine, cyclosporine) . uncertain effect. 73 Follow up Recover thoroughly. Remained ECG changes. Dysfunction of LV Cardiomyopathy Death 74 Ending 心 包 炎 心包 由壁层和脏层组成的一 个潜在腔隙，即心包腔 ，其间有少量液体（ 50mL) 定义及分类 心包炎是指由多种致病因素引起的心包 脏层和壁层的炎性病变 按病程心包炎可分为急性心包炎、亚急 性渗出性缩窄性心包炎、慢性心包积液 和慢性缩窄性心包炎等 是由多种原因所致的心包脏层和壁层 的急性炎症性纤维化反应 以胸痛、心包摩擦音、特异性心电图 表现为特征 急性心包炎 （Acute Pericarditis） 病 因 一、特发性：国外多见 二、感染性：细菌、病毒、结核、真菌、原 虫和艾滋相关性等，我国结核多见 。 三、肿瘤：原发性（如间皮细胞瘤）或继发 性（乳腺、肺、淋巴瘤等）。 四、免疫性：RA、SLE、硬皮病、急性风湿热 、 皮肌炎等。 病 因 五、炎性反应性：心梗后、外伤、外科 手术、放射性、药物性等。 六、代谢性：尿毒症、痛风、黏液性水 肿等。 七、邻近器官疾病：胸膜炎、主动脉夹 层、肺梗死等。 病 理 一、早期：心包的脏层和壁层间渗出纤维蛋 白和白细胞等。称为纤维蛋白性或干性心包 炎。 二、进展期：渗出物中液体增加，液量可多 至23L，称为渗出性或湿性心包炎。渗液的 性质可为纤维蛋白性、浆液血性或化脓性等 。 三、后期：渗出物可完全吸收；或某些心包 炎机化为瘢痕甚至钙化，最终发展成为缩窄 性心包炎。 纤维蛋白性心包炎 病理生理 1.渗液慢，心包可逐渐伸展，致使心包压力不 增加，可以无症状。积液使心包腔内压力逐渐 上升，当达到一定程度就限制心脏扩张。 2.渗液快，心包压力急剧增加，心室舒张期充 盈减少，导致静脉压增加，心搏量降低，血压 下降。 机体代偿机制：心肌收缩力增加、心率加快、 周围小动脉阻力增加以维持血压。 病理生理 3.如心包渗液继续增加，心包腔内压力进一 步提高达到右房右室舒张压水平，其压差等 于零时，心脏压塞或心包填塞（cardiac tamponade）即可发生。 此时代偿机制衰竭，即升高的静脉压不能 增加心室的充盈；过快的心率使心室舒张期 缩短和充盈减少，而致心排血量下降；小动 脉收缩达到极限，动脉压下降至循环衰竭， 而产生心源性休克。 心脏压塞是否发生，取决于心包积液的容 量、速度及心包本身的韧性及心肌功能状态 。10 临床表现纤维蛋白性心包炎 症状： 主要症状：心前区疼痛为多 见 疼痛性质：可尖锐，也可呈 压榨性 与呼吸运动有关，常因咳嗽 、深呼吸、变换体位或吞咽 而加重 可放射至颈部、左肩、左臂 等 体征： 心包摩擦音为典型 体征 心前区的抓刮样粗 噪音 与心房收缩、心室 收缩和心室舒张相 一致的双相音 积液增多时摩擦音 消失 临床表现渗出性心包炎 症状：呼吸困 难是最突出的 症状，严重者 端坐呼吸，身 体前倾，可有 发绀， 或压迫症状： 干咳、声音嘶 哑，吞咽困难 体征：触诊：心尖搏动弱； 叩诊：心浊音界向两侧扩大 ，Ewart征；听诊：心率快 ，心音遥远 收缩压降低，舒张压变化不 大，脉压变小；心脏压塞严 重时可出现奇脉 出现颈静脉怒张、肝肿大及 下肢水肿 临床表现心脏压塞 急性心脏压塞，以心输出量下降肺静脉压增 高肺循环淤血为主要表现：心排血量下降、 心动过速、紫绀、脉压变小、收缩压下降甚 至休克 Beck三联征：血压突然下降或休克，颈静脉 显著