心肌病2010-12-30.ppt

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 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 Hypertrophy 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 Diagosis Heredity of family symptoms Physical inspection Ultrasonic cardiogram Differential diagnosis Chest pain , ECG Q wave、ST-T change——Coronary heart disease High voltage in ECG, Ultrasonic cardiogram-- Hypertension Chest pain, murmur – Aortic stenosis Drugs should be avoided and carefully used： Digitalis(except Af or contraction insufficiency) Diuretic β-receptor blocker Management management β- 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 Management No-drug-treatment The pacing of Obstructive hypertrophic cardoiomyopathy The pacing of Obstructive hypertrophic cardoi