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CARDIOMYOPATHIES COMPILED BY : DR.ALIREZA HOGHOOGHI.

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Presentation on theme: "CARDIOMYOPATHIES COMPILED BY : DR.ALIREZA HOGHOOGHI."— Presentation transcript:

1 CARDIOMYOPATHIES COMPILED BY : DR.ALIREZA HOGHOOGHI

2 CMP s: Heterogenous group of diseases of myocardiom Associated with functional and structural abnormalities

3 Four main cmps DILATED CARDIOMYOPATHIES(DCM) HYPERTROPHIC CARDIOMYOPATHIES(HCM) RESTRICTIVE CARDIOMYOPATHIES(RCM) ARRYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA

4 Familial (genetic )and non familial (acquired)form of the diseases have been describe

5 DCM Characterized by: enlargement of LV or LV OR RV Impaired systolic function of LV or LV and RV DCM can be famillial or acquired ¼ of DCM are familial(genetic mutation)

6 Some specific mutations involve genes encode proteins of sarcomers,cytoskeeton,nuclear membrane,mitochondria and many remains unknown Mode of inheretance is typically A.D NON FAMILIAL DCM HAS DIFFERENT CAUSES Mostly believed to be a result of acute viral myocarditis Exposure to cardiac toxins can also lead DCM

7 Anthracyclines (doxorubicin).daunorbicin have dose dependant cardiac toxicity Long term exposure to alohol is an important preventable cause of DCM

8 TABLE 59-7 -- CAUSES OF DILATED CARDIOMYOPATHY CARDIOVASCULAR DISORDERS Systemic hypertension Ischemic heart disease Valvular heart disease Myocarditis Peripartum cardiomyopathy TOXINS Alcohol Catecholamines Anthracyclines Radiation Cocaine ASSOCIATED SYSTEMIC DISEASES S ystemic lupus erythematosus Polyarteritis nodosa Rheumatoid arthritis Scleroderma Dermatomyositis MUSCULAR DISORDERS Duchenne's muscular dystrophy Becker-type muscular dystrophy Myotonic dystrophy Mitochondrial disorders HIGH-OUTPUT STATES Thiamine deficiency Thyrotoxicosis Severe anemia Arteriovenous fistulas/shunts Incessant tachycardia

9 Deficiency of nutrients.thiamin vit C,selenium,phosphate,calcium Peripartum DCM is a form of idiopathic DCM in last month of pregnancy or several month of delivery Pathogenesis is unknown autoimmune,viral myocarditis,hemodynamic stress PROLONGED SVT OR VT CAN LEAD TO DCM (tachycardia induced CMP) STRUCTURAL AND FUNCTIONAL CHANGES REVERSE AFTER CONTROL OF HR

10 Development of DCM can be gradual and many are asymptomatic first presentation is usually due to symptoms of heart failure :fatigue,weakness,dyspnea,edema in some presenting episode is arrythmia On physical exam : Tachycardia is often present,narrow pulse pressure,tachypnea,jvp distention Laterally displaced apex,s3 gallop common,murmur of MR and TR RALES,PLEURAL EFFUSION, IN SOME RIGHT SIDED HF IS PROMINENT :ASCITES,HEPATOMEGALY,EDMEMA,ANASARA

11 BNP LEVEL IS ELEVATED ECG NON SPECIFIC ST T CHANGE ECHO : EVALUATION OF LV SIZE AND FUNCTION AND ABNORMALITY OF VALVES, AND LV THROMBUS SO DO MRI MYOCARDIAL BIOPSY IS INDICATED IF ETIOLOGY OF DCM IS IN QUESTION IN PATIENT WITH STRONG FH REFERRAL FOR GENETIC STUDY IS CONSIDERED

12 THERAPY POTENTIAL REVERSIBLE CAUSES SHOULD BE ADDRESSED LOOP DIURETICS FOR CONGESTED PERSONS GOOD FOR SYMPTOMS BUT EFFECT OF SURVIVAL HAS NOT BEEN EVALUATED ACEI.BETABLOCKERS,HYDRALASINE NITRATES,ALDACTONE,DIG,CRT,CRTD,TRANSPLANT LV ASSISTED DEVICE

13 HCM CHARACTERIZED BY LVH AND SMALL LV AND ABSENCE OF AN APPARENT CAUSE FOR HYPERTROPHY COMON GENETIC DISEASE 1/500 AND IS A.D >400 MUTATION IN 11 DIFFERENT GENE MUTATION OF BETA MYOSIN HEAVY CHAIN IS FREQUENT THERE IS SPORADIC FORM MICROSPIC PHENOTYPE.CARDIOMYOCYTE HYPERTROPHY,MYOFIBRILLAR AND INTERESTITIAL FIBROSIS

14 NEW ADDED TO HCM METABOLIC STORAGE DISEASE THAT RESULT IN CHANGES IN MYOCARDIAL APPEARANCE WHICH RESEMBLES HCM GYCOGEN STORAGE STORAGE DISEASE LYSOSOMAL STORAGE DISEASE DISORDER OF FATTY ACID METABOLISM

15 PHENoTYPICAL EXPRESSION Is DIFFERENT BECAUSE OF DIFFERENT PENETARTION Main pathophysiologic abnormalities seen in HCM are LVOT OBSTRUCTION DIASTOLIC DYSFUNCTION.MR.ARRYTHMIA

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17 Presentation Some are asymptomatic Symtom result of lvoto and diastolic dysfunction Most frequent dyspnea on exertion,which causes marked lv filling pressure and pulmonary venous pressures and congestion Ischemic chest pain in absence of CAD Syncope and presyncope In some sudden death due to arrythmia is first presentation

18 Physical exam Bisferiens pulse Forceful and sustained apical impulse Decreased complince of the LV during atrial contraction may lead to an audible s4 Harsh crescendo and decrscendo sm best heard in lsb radiation to base of the heart May be apical holosystolic murmur of MR

19 Intensity of murmur will increase with valsalva,standing and use of tng or inotrops Intensity of murmur will decrease with squating,volume loading,use of beta blockers

20 Ecg in hcm :increased QRS voltage suggestive of LVH.SECONDARY ST CHANGE AND T INVERSION.,PSUDOINFARCT PATTERN WITH Q IN INF,LAT OR ANT LEADS ECHO AND MRI ARE HELPFL IN CONFIRMING THE DIAGNOSIS ECHO IS USEFUL IN SCREENING CATH IF ECHO IS NOT ADEQUATE AND CAN CONFIRM INTRA CAVITARY GRADIENT

21 MANAGEMENT IS AIMED TO REDUCING TH LVOTOBSTRUCTION,IMPROVING DIASTOLIC DYSFUNCTION AND REDUCING RISK OF SUDDEN DEATH BETABLOCKERS AND CALCIUM CHANNEL BLOCKERS AND DISOPYRAMIDE CAN REDUCE SYMPTOMS

22 PPM, SURGERY SEPTAL MYECTOMY,ALCOHOL ABLATION ICD FOR SUDEN DEATH : 1.PRIOR CARDIAC ARREST 2.SUSTAIN VT 3.>30 MM VENTRICULAR THICK NESS 4.SYNCOPE 5.FIRST DEGREE RELATIVE SUDDEN DEATH

23 RCM RCM IS RARE CHARACTERIZED BY IMPAIRED VENTRICULAR FILLING OR DECREASED DIASTOLIC VOLUME OF EITHER OR BOTH VENTRICLE VENTRICULAR PRESSURE RISE SIGNIFICNT SYSTOLIC FUNCTION USUALLY IS PRESERVED ARE FAMILLIAL OR NONFAMILLIAL

24 ONE OF THE COMMON FORM OF FAMILLIAL FORM IS FAMILIAL AMYLOIDOSIS { "@context": "http://schema.org", "@type": "ImageObject", "contentUrl": "http://images.slideplayer.com/1595312/5/slides/slide_23.jpg", "name": "ONE OF THE COMMON FORM OF FAMILLIAL FORM IS FAMILIAL AMYLOIDOSIS

25 DIAGNOSIS OF RCM SHOULD BE CONSIDERED IN PATIENT WITH PREDOMINANTLY RV FAILURE WITHOT EVIDENCE OF CARDIOMEGALY OR SYSTOLIC DYSFUNCTION CONSTRICTIVE PERICARDITIS CAN PRESENT SIMILARLY TO RCM TREATMENT IS FOCUSED ON ALLEVIATATING THE SYMPTOMS OF HEART FAILURE

26 ARVD IS A.D MALE PREDOMINANCE PROGRRESIVE REPLACEMENT OF RV MYOCARDIUM BY FIBROUS AND ADIPOSE TISSUE INSOME INVOLVES LV IN THESE PATIENT PRESENTATION MAY RESEMBLE DCM PREVALNCE 1/1000 TO 1/5000 PRESENTING IN YOUNG ADULT PRESENTING SYMPTOMS IS USUALLY ARRYTHMIA,PALPITATION,SYNCOPE,,SUDDEN DEATH,SYMPTOM OF RV FAILURE IS RARE

27 ,BY CLINICAL PRESENTATION DIAGNOSIS IS MADE Resting ECG FAMILY HISTORY IMAGING STUDY ECG USUALY NORMAL BUT SOME MAY HAVE INCOMPLETE OR COMPLETE RBB AND EPSILON WAVE AND INVERTED T WAVES IN PRECORDIAL VT MOOMORPHIC WITH LBBB PATTERN TREATMENT BY ICD

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