心肌病2010-12-30

1、Cardiomyopathy and Myocarditis,北京大学人民医院 心脏中心 郭丹杰,Cardiomyopathy,Classification,Dilated cardiomyopathy (DCM) Hypertrophic cardiomyopathy (HCM) Restrictive cardiomyopathy (RCM) Arrhythmogenic right ventricular cardiomyopathy (ARVC) Specific cardiomyopathy Unclassified cardiomyopathy WHO/ ISFC Circulat

2、ion 1996;93:841,Dilated Cardiomyopathy (DCM),Pathology,dilated cardiomyopathy (from Becker and Anderson),Clinical Findings,-Low Cardiac Output fatigue, headache, hypotension -Pulmonary Congestion -Exertional dyspnea -orthopnea paroxysmal nocturnal dyspnea -Systemic Congestion Edema Ascites Weight ga

3、in,INVESTIGATION,Examination,a X-Ray b ECG c Ultrasonic cardiogram d Cardiac catheterization e Examination of radionucide,Diagosis,Clinical cardiac enlargement、arrhythmia、heart failure Ultrasonic cardiogram Amplification of cardiac chamber Diffuse weakened motion of ventricular wall,Should exclude :

4、 All kinds of heart diseases with clear pathogeny,Differential diagnosis,Management,1. Heart failure: digoxin、diuretic、ACEI、- blocker 2. Prevent the embolism :LVEF30%、Af or have the history of embolism: Warfarin（ INR2.0-3.0） 3. Prevention of sudden death 4. Heart transplantation,CRT: Cardiac Resynch

5、ronization Therapy,1. Improved hemodynamics Increased CO Reduced LV filling pressures Reduced sympathetic activity 2. Reverse LV Decreased LVES/ED volumes Increased LVEF,Hypertrophic cardoiomyopathy (HCM）,Obstructive hypertrophic cardoiomyopathy Nonobstructive hypertrophic cardoiomyopathy,Classifica

6、tion of HCM,Pathology,Asymmetry interventricular septum hypertrophy ( 90%) Symmetry left ventricle hypertrophy( 5%) Asmmetric septal hypertrophy, ASH）(3%),Hypertrophic Cardiomyopathy,hypertrophy of cardiac muscle cell,Pathology,Pathology,Clinical situation- Symptom,Absence of symptoms (50%) Common s

7、ymptom: chest pain dyspnoea syncope sudden death,Pathophysiology,Nonobstructive No evident sign, heart enlarge Obstructive: systolic murmur at the lower left of sternum; systolic murmu in apex of 50% patients.,Clinical situation -Sign,Examination,Abnormal Q wave（30-50%）in lead II、III、AVF and V4-V6 H

8、ypertrophy of the left ventricle Alteration of ST-T, T wave inverted Arrhythmia,E C G,Ultrasonic cardiogram,Thickness of interventricular septum1.3cm Septum : free wall1.3-1.5 Left ventricular outflow tract(LVOT) narrow Diastolic dysfunction Systolic anterior motion,( SAM) -HOCM,Mechanisim of SAM,Di

9、agosis,Heredity of family symptoms Physical inspection Ultrasonic cardiogram,Differential diagnosis,Chest pain , ECG Q wave、ST-T changeCoronary heart disease High voltage in ECG, Ultrasonic cardiogram- Hypertension Chest pain, murmur Aortic stenosis,Drugs should be avoided and carefully used： Digita

10、lis(except Af or contraction insufficiency) Diuretic -receptor blocker,Management,management,- blocker Calcium antagonist Amiodarone,室间隔部分心肌切除术 室间 隔心肌剥离扩大术,Management No-drug-treatment,Adaptation： Medication difference or inefficacy； Difference pressure in outflow tract of left ventricle 50mmHg,经皮间隔

11、心肌消融术 (Transluminal Septal Myocardial Ablation，PTSMA) Sigwart于1995年首次应用于临床。,Management No-drug-treatment,Transluminal Septal Myocardial Ablation，PTSMA,PTSMA,PTSMA,ACC/ESC Expert Consensus Document on Hypertrophic Cardiomyopathy JACC 2003; 42:1,pacing Obstructive hypertrophic cardoiomyopathy,Manageme

12、nt No-drug-treatment,The pacing of Obstructive hypertrophic cardoiomyopathy,The pacing of Obstructive hypertrophic cardoiomyopathy,high risk patients（ arrhythmia） 埋藏式心脏复律除颤器（AICD）,Management No-drug-treatment,Risk Factors for Sudden Death in HCM,致心律失常性右室心肌病,（ arrhythmogenic right ventricular cardiom

13、yopathy, ARVC） （arrhythmogenic right ventricular dysplasia, ARVD） （ARVD/C）,Characteristic,Heredity disease Cardiac muscles in right ventricular are replaced by fibrins and fats Clinic represent： right heart enlarge right heart failure and/or ventricular tachycardia sudden death,Examine,ECG : LBBB ty

14、pe VT or ventricle premuture beat frequently UCG: right ventricle large，contraction weak，ventricular wall thin， Electric-physiology: slow conduction of excitation through right ventricle,Diagosis and differential diagnosis,Symptom of right heart failure ventricle premuture beat （LBBB ) Large right v

15、entricle T wave convertion on chest lead,Diagosis,Therapeutics,NO specially effective treatment Aim at right heart failrue and arrhythmia ventricular tachycardia repeatly: radiofrequency catheter ablation implantable cardioverter defibrillator,Restrictive Cardiomyopathy,Clinical Manifestations,Sympt

16、oms of right and left heart failure Jugular Venous Pulse prominent x and y descents Echo-Doppler abnormal mitral inflow pattern prominent E wave (rapid diastolic filling) reduced deceleration time ( LA pressure),Treatment,No satisfactory medical therapy Drug therapy must be used with caution diureti

17、cs for extremely high filling prssures vasodilators may decrease filling pressure ? Calcium channel blockers to improve diastolic compliance,Specific Cardiomyopathies,Disease of myocardium with definitude pathogeny or has correlation with the system disease.,特异性心肌病specific cardiomyopathies,Class of

18、specific cardiomyopathies,Ischemia cardiomypathy Valvuar cardiomypathy Hypertensive cardiomypathy Inflammatory ardiomypathy Metabolic cardiomypathy,Muscular dystrophy Neuromuscular disease Hypersusceptibility and intoxication Cardiomyopathy in perinatal period,Specific cardiomyopathies,Peripartum cardiomyopathy Drug-induced cardiomyopathy Alcoholic cardiomyopathy,心 肌 炎,（myocarditis),Viral myocarditis,1999年镇江 全国心肌炎心肌病学术