Cardiomyopathy

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Cardiomyopathy

• Dr.mirdamadi
• Cardiologist, fellowship of echocardiograpy

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Definition

• Group of diseases that primarily affect the heart
  muscle and are not the result of congenital,
  acquired, valvular, hypertensive , coronary
  arterial or pericardial abnormalities

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Classification
Dilated
primary myocardial involvement
Restrictive
secondary myocardial involvement
Hypertrophic
( infective,

Metabolic,

Connective tissue
disorders

Infiltration,

toxin,

peripartum)
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Dilated cardiomyopathy (DCM)
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• 1/3 of CHF is due to DCM
• LV and/or RV systolic function is impaired
  leading to progressive cardiac dilation
• No cause detected in many cases, but familial
  (1/4-1/3 of cases) or secondary cause like
  infectious, metabolic or toxic agents present.

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• Reversible form may be found with alcohol abuse,
  pregnancy, thyroid disease, cocaine use and
  chronic uncontrolled tachycardia.

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Clinical features

• symptoms of left and right sided CHF.
• some patients have LV dilation for months or
  even years before becoming symptomatic
• vague chest pain (typical angina is unusual and
  suggestive of IHD)
• syncope due to arrhythmias
• systemic embolism

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Physical examination

• Narrow pulse pressure and elevated JVP in
  advanced disease
• S3
• S4
• MR
• TR

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Laboratory examination

• CXR cardiomegaly , pulmonary congestion
• ECG Sinus tachycardia or AF , Ventricular
  arrhythmias, Non specific changes
• Echocardiography LV dilation , systolic
  dysfunction
• Angiography to exclude IHD

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• Endomyocardial biopsy
• is not necessary in idiopathic or familial
  DCM but may be helpful in the recognition of
  secondary CMP like amyloidosis and myocarditis

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• most patient has progressive course
• patients gt 55 years die within 4 years of the
  onset of symptoms
• Spontaneous improvement in about one- quarter of
  patient
• death is due to progressive HF or ventricular
  arrhythmia or brady arrhythmia

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Alcoholic cardiomyopathy
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• large quantities (gt90g/d) of alcohol over many
  years cause DCM
• Risk of developing CMP is partially genetically
• patient with severe CHF have a poor prognosis (lt
  1/4 of such patients survive 3 years)

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• Second presentation of alcoholic cardiotoxicity
  is recurrent supraventricular or ventricular
  tachyarrhythmias
• Holiday heart syndrome AF, Atrial flutter or
  frequeut PVC after a drinking binge.

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Peripartum cardiomyopathy
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• Cardiac dilation and CHF may develop during the
  last trimester of pregnancy or within 6 months of
  delivery.
• Mortality rate is 10.
• Patient who recover from peripartum CMP should be
  encouraged to avoid further pregnancy .

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Drugs
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• A variety of drugs may damage the myocard?ium
  acutely (myocarditis) or they may lead to chronic
  damage (like DCM)
• Anthracycline derivatives
• Doxorubicin cardiotoxicity may occur acutely but
  more commonly develops 3 months after the last
  dose.
• TCA antidepressants, phenothiazines , lithium
• Cocaine abuse (SCD , myocarditis , DCM, acute MI
  )

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Arrhythmogenic right ventricular cardiomyopathy
/ Dysphasia (ARVC/D)
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• ARVD is a familial CMP with progressive
  fibrofatty replacement of the RV and to a much
  lesser degree LV.
• Patients may present by RV failure or ventricular
  tachycardia

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Others

• Neuromuscular disease
• Tako tsubo (stress) CMP
• Non compaction CMP

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Hypertrophic cardiomyopathy
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• characterized by LV hypertrophy without obvious
  cause
• It is 1 in 500 of general population
• two features af HCM asymmetric LV hypertrophy ,
  Dynamic LVOT obstruction

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• There is a bizarre and disorganized arrangement
  of myocytes with variable degree of fibrosis.
• About a half of patients have a positive family
  history compatible with autosomal dominant
  transmission

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Normal and HCM cellular
arrengement
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• Screening by echocardiography of first- degree
  relatives between the age 12 and 20 should be
  done every 12-24 months unless diagnosis
  evaluated by genetic testing

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Clinical features

• Clinical course is variable , from asymptomatic
  to SCD
• SCD frequently occur in children and young adult
  during or after physical exertion.
• HCM is the most common cause of SCD in young
  competitive athletes
• Other symptom dyspnea , syncope , angina and
  fatigue.

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Physical examination

• Double or triple apical precordial impulse
• Fourth heart sound
• Harsh , diamond shaped systolic murmur in LSB
  due to LVOT obstruction
• Holosystolic and blowing systolic murmur at
  apex due to MR

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Hemodynamic

• Obstruction in LVOT is dynamic (not fixed) and is
  due to narrowing of the LVOT by systolic
  anterior motion (SAM) of mitral valve against the
  hypertrophied septum.

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Hemodynamic

• Three mechanisms are involved dynamic obstruction
  
• 1) increased LV contractility (exercise)
• 2) decreased ventricular preload (strain phase of
  valsalva maneuver , standing )
• 3) decreased ventricular after load

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• Squatting , sustained handgrip position with
  leg raising , expansion of volume in pregnancy
  decreased obstruction

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Laboratory evaluation

• ECG LVH and deep broad Q waves (mistake as a
  MI)
• CXR Cardiomegaly
• Echocardiography septal hypertrophy (
  septumgt1.3 times of posterior wall )
• SAM of mitral valve with MR.

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Apical HCM

• Apical hypertrophy
• Giant negative T wave on the ECG
• Spade shaped LV cavity

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Management

• Competitive sports and very strenuous activities
  should be proscribed
• dehydration should be avoided.

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• B- blockers ameliorate angina and syncope
  in1/3-1/4 of patients
• Varapamil and diltiazem may reduce the
  stiffness of LV
• Amiodaron reduced risk of SCD and arrhythmia
• Digitalis,diuretics,nitrates, dihydropyridine
  calcium blockers, vasodilators and B- agonists
  are best avoided.

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• Surgical myotomy / myectomy of septum and ethanol
  injections into the septal artey are the invasive
  treatment.
• ICD should be considered in high- risk patients

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Restrictive cardiomyopthy
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• Hallmark of the RCM is abnormal diastole
  function.
• Myocardial fibrosis,hypertrophy or infiltration
  caused rigid LV walls

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• Amyloidosis,
• hemochromatosis,
• glycogen storage disease,
• endomyocardial fibrosis,
• sarcoidosis,
• hypereosinophilic disease,
• sceleroderma,
• following mediastinal irradiation

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• Inability of the ventricles to fill, caused
  decrease cardiac out put
• exercise intolerance and dyspnea
• elevated systemic venous pressure cause edema
• ascitis and elevated JVP

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Laboratory examinations

• in infiltrative disease
• ECG often shows low voltage, nonspecific
  ST/T change and various arrhythmias.
• Echocardiography , CT and MRI showed thickened LV
  walls with normal LV systolic function and
  dilated atria.

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Differentiation of RCM from Constrictive
pericarditis important.
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• Management is usually disappointing except for
  hemochromatosis and fabrys disease.

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Myocarditis
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• myocarditis is most commonly the result of
  infectious process, frequently complicated by
  autoimmunity.
• myocarditis may also result from
  hypersensitivity to drugs (TCA ,
  antibiotics,antipsychotics) or may be caused by
  irradiation, chemicals or physical agents.

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• Most common cause is viruses especially
  coxsakievirus B
• adenovirus
• hepatitis C
• and HIV
•  

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• Patients with viral myocarditis may give a
  history of upper respiratory febrile illness or a
  flu like syndrome ,
• and viral nasopharyngitis or tonsillitis may
  be evident.

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Clinical manifestation

• clinical spectrum ranges from an asymptomatic
  state to fulminant condition with arrhythmias,
  acute CHF and death.

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• sometime myocarditis simulates an acute coronary
  syndrome ( chest pain, ECG change and elevated
  troponin level ) but typically in patients
  younger than those with coronary atherosclerosis.

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• Viral myocarditis is most often self-limited and
  without sequelae but sometime may progress to a
  chronic from and to DCM.

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