Cardiomyopathies
description
Transcript of Cardiomyopathies
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CardiomyopathiesBy:Dawit Ayele(MD,Internist)
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a group of diseases that affect the heart muscle itself and are not the result of hypertension , congenital, valvular , coronary, or pericardial abnormalities
Definition
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based on LV cavity size, wall thickness, and systolic contraction
dilated (myocyte necrosis, profound dilation, and systolic dysfunction),
hypertrophic(disproportionate septal thickening, obstructive or non-obstructive), or
restrictive (generalized wall thickening with both systolic and diastolic impairment)
Classification
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Is mainly due to Prolonged, uninterrupted biomechanical overload
is characterized by eccentric hypertrophy(dilatation) , loss of cardiac contractile function(hypokinesis), and loss of cardiomyocytes due to apoptosis.
Dilated Cardiomyopathy
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Prevalence is increasing
About 1 in 3 cases of CHF in the west is due to dilated cardiomyopathy
More common in middle aged men & African Americans than whites
Epidemiology
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Idiopathic Familial/genetic- 20-40% mostly autosomal
dominant transmission infection, (viral esp) inflammation, Toxins-(alcohol,cocain) Pregnancy Thyroid disease Chronic uncontrolled tachycardia collagen vascular disease, and musculoskeletal disease
Causes
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Of left & right sided CHF
Some present with left ventricular dilatation for months to yrs before diagnosis
Vague chest pain-but typical angina is unusual
Syncope 20 to arrhythmia or systemic embolism
Clinical Manifestations
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Variable degrees of cardiac enlargement Advanced disease-narrow pulse pressure -Raised JVP -S3 & S4 sounds common -Mitral & tricuspid
regurgitation may occur
Physical Examination
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CXR-enlargement of cardiac silhoutte -may show evidence of pulmonary
venous hypertension -may show interstitial & alveolar edema ECG- *Echocardiography Cardiac catheterization Coronary angiography Transvenous endomyocardial biopsy-
usu.not necessary
Lab examinations
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Majority esp >55 yrs die within 3 yrs of onset of sxs Target: - Avoid toxins including alcohol
-Control CHF-standard Management
-Chronic anticoagulation –prevent embolism
-Sometimes pacemaker & use of implantable cardioverter defibrillator
-cardiac transplantation for refractory cases
Treatment
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is a genetic disorder characterized by disproportionate hypertrophy of the left ventricle, and occasionally of the right ventricle
HYPERTROPHYIC CARDIOMYOPATY
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Prevalence ◦1:500[0.2%] in general
population(may be the most common genetically transmitted cardiac disorder)
◦0.5% in non-selected Echo referral Most common cause of Sudden cardiac
death in the young in the USA Usually in third or fourth decades
Epidemiology
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Etiology - Genetic: Familial HCM-
Autosomal dominant(50%)
Sporadic due to spontaneous mutations
- Idiopathic
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Pathology(Gross) -marked in myocardial
mass-more LV involvement in
hypertrophy-small ventricular
cavities -dilated atria &
hypertrophy (vent. filling resistance )
- MV& elongation -anomalous papilary
ms. insertion.
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Pathology(Gross)-variable pattern & extent
of hypertrophy(Maj.IVS and
anterolateral free wall)
-Eventual burned-out phase in 5-10% of patient◦ Resemble DCM
Thinning, dilation, systolic dysfunction
-Clinically silent remodeling with subtle regression of hypertrophy in some patient
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Pathology (microscopic)
-Myocardial hypertrophy-Gross disorganization
of muscle bundles(whorled pattern)
-Cell to cell arrangement abnormality(disarray)
-Prominent fibrosis-interstitial connective
tissue elements.-Abnormal intramural
coronary arteries(80%)
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◦ Asymptomatic in majority [90%] Sudden death can be the 1st event [esp. during
extreme exertion]◦ Variable pattern and severity of symptoms
Dyspnea most common [90%] Angina [75%] Fatigue, presyncope and syncope common [not
ominous in adult] Palpitation, PND and dizziness less common
◦ Exacerbating factors Exercise Erect posture [graying out spells]
Clinical feature ---Symptoms
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Physical Examination - Normal except for an S4
Asymptomatic without – pressure gradient Mild hypertrophy Apical hypertrophy
- Usually prominent in presence of pressure gradient Characteristic carotid
pulse [“spike and dome”]
Prominent “a” wave Displaced, diffuse and
abnormally forceful AI
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Narrow or paradoxical S2 split S3 may be present [no prognostic value
unlike in valvular AS] Occasionally systolic ejection click Systolic murmurs…midsystolic and
hollosystolic [associated MR]◦ Correlate with SAM and pressure gradient◦ Labile intensity and duration◦ *Influenced by maneuvers
Diastolic murmurs…..apical rumbling and high pitched AR
Physical exam…
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1-ECG 2-CXR(N/cardiomegaly+/-calcification) 3-Echocardiography 4-Other imaging-radionuclide scan,cardiac
MRI(when Echo is technically inadequate) 5-Cardiac catheterization(CAD,invasive Rx) 6-Biopsy 7-Genetic analysis?
Investigation
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The subcostal view from the two dimensional echocardiogram shows extremely hypertrophied and
asymmetric septum which is 35 mm in thickness.
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Septal hypertrophy
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Concentric hypertrophy
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Guiding principles:Three important goals Symptoms alleviation Complication prevention Death risk reduction
Majority require only medical treatment At least 50% with sever symptoms improveInvasive modalities required in only 5-
10% **Myotomy-myectomy[ gold standard..70-
90%]
Management
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CLINICAL ALGORITHM
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Condition & medics that better be avoided - physical exertion&competitive sports
- digitalis glycosides unless A-fib or systolic dysfunction
-B-adrenergic agonist -Nifedipine -excessive use of diuretics & dehydration
Management…
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Restrictive cardiomyopathy
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Abnormal diastolic function
Excessively rigid ventricular walls that impede filling
Unimpaired systolic function
*Has functional resemblance with constrictive pericarditis
*Is the least common of the main 3 CMPs
Hallmark of Constrictive Pericarditis
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Myocardial-Non Infiltrative:*Idiopathic CMP Familial Scleroderma -Infiltrative:*Amyloidosis *Sarcoidosis -Storage disease Hemochromatosis..
Endomyocardial -*Endomyocardial fibrosis -*Radiation -*Toxic effects of anthracyclin -eosinophilic syndrome -metastatic cancers -Drugs(Serotonin,ergotamin,busulfan..)
Classification of types according to cause
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Pathologic processes
- Myocardial fibrosis
- Infiltration
- Endomyocardial scarring
- Myocyte hypertrophy in idiopathic variety
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Depends on which ventricle & AV valve show predominant involvement
Thromboembolic complication ~1/3 Sx-Exercise intolerance & dyspnea are
the most prominent sxs.(inability to CO by tachycardia w/o compromising vent filling)
-Exertional chest pain -Dependent edema,ascites &
enlarged tender & often pulsatile liverpersistently venous pressure.
General Clinical Features
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Distended JVP-doesn’t fall normally - +/- kussmaul’s sign *apex pulse is usually palpable
Ht sounds may be distant
S3, S4 or both
Physical Exam
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ECG
CXR to R/O ddx-eg-constrictive pericarditis
Echocardiography –
Cardiac catheterization –
Endomyocardial biopsy, computed tomography or magnetic resonance imaging & others help distinguish restrictive & constrictive disease
Investigations
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Echocardiography of restrictive cardiomyopathy
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Endomyocardial biopsy cardiac amyloidosis
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No specific therapy other than symptomatic is available for the idiopathic form of RCM
Few secondary forms may benefit from targeted treatment regimens:
Hemochromatosis: deferoxamine has been helpful in reducing myocardial iron content
Chronic anticoagulation is often recommended to reduce the risk of embolization from the heart.
The prognosis in RCM is variable: usually it is one of relentless symptomatic progression and high mortality.
Treatment & prognosis
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Thanks