Transcript CARDIOMYOPATHIES

CARDIOMYOPATHIES
COMPILED BY :
DR .ALIREZA HOGHOOGHI
CMP s:
Heterogenous group of diseases of •
myocardiom
Associated with functional and structural •
abnormalities
Four main cmps
DILATED CARDIOMYOPATHIES(DCM) •
HYPERTROPHIC CARDIOMYOPATHIES(HCM) •
RESTRICTIVE CARDIOMYOPATHIES(RCM) •
ARRYTHMOGENIC RIGHT VENTRICULAR •
DYSPLASIA
Familial (genetic )and non familial
(acquired)form of the diseases have
been describe
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)
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
Anthracyclines (doxorubicin).daunorbicin have dose dependant
cardiac toxicity
Long term exposure to alohol is an important •
preventable cause of DCM •
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
•
•
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
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
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
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
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
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
PHENoTYPICAL EXPRESSION Is
DIFFERENT BECAUSE OF DIFFERENT
PENETARTION
Main pathophysiologic abnormalities seen in •
HCM are LVOT OBSTRUCTION
DIASTOLIC DYSFUNCTION .MR .ARRYTHMIA •
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
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 •
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
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
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
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 •
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 •
ONE OF THE COMMON FORM OF FAMILLIAL FORM IS
FAMILIAL AMYLOIDOSIS<TRANSTHYRETIN AND
APOLIPOPROTEINS
FAMILIAL HEMOCHROMATOSIS •
MUTATION IN DESMIN •
AQUIRED •
FORMS:AMYLOIDOSIS,SARCOIDOSIS,CARCINOI
D HEART DISEASE ,SCLERODERMA
ENDOCARDIAL PATHOPHYSIOLOGY OF •
STIFFNESS IS ALSO
PRESENT:HYPEREOSINOPHILIC SYNDROME
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
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
DIAGNOSIS IS MADE ,BY CLINICAL PRESENTATION
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 •
CARDIOMYOPATHIES •