Download Cardiomyopathy

Cardiomyopathy
Dr.mirdamadi
Cardiologist, fellowship
of echocardiograpy
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
Classification:
Dilated
primary myocardial involvement
Restrictive
secondary myocardial involvement:
Hypertrophic
( infective,
Metabolic,
Connective tissue disorders
Infiltration,
toxin,
peripartum)
Dilated cardiomyopathy
(DCM)
• 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.
• Reversible form may be found with alcohol
abuse, pregnancy, thyroid disease,
cocaine use and chronic uncontrolled
tachycardia.
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
Physical examination
• Narrow pulse pressure and elevated JVP in
advanced disease
• S3
• S4
• MR
• TR
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
• Endomyocardial biopsy :
is not necessary in idiopathic or familial
DCM but may be helpful in the recognition of
secondary CMP like amyloidosis and
myocarditis
• most patient has progressive course
• patients > 55 years die within 4 years of
the onset of symptoms
• Spontaneous improvement in about onequarter of patient
• death is due to progressive HF or
ventricular arrhythmia or brady arrhythmia
Alcoholic cardiomyopathy
• large quantities (>90g/d) of alcohol over
many years cause DCM
• Risk of developing CMP is partially
genetically
• patient with severe CHF have a poor
prognosis (< 1/4 of such patients survive
3 years)
• Second presentation of alcoholic
cardiotoxicity is recurrent supraventricular
or ventricular tachyarrhythmia’s
• Holiday heart syndrome: AF, Atrial flutter
or frequeut PVC after a drinking binge.
Peripartum
cardiomyopathy
• 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 .
Drugs
• A variety of drugs may damage the
•
•
•
•
myocardium 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 )
Arrhythmogenic right
ventricular cardiomyopathy
/ Dysphasia (ARVC/D)
• 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
Others
• Neuromuscular disease
• Tako – tsubo (stress) CMP
• Non compaction CMP
Hypertrophic
cardiomyopathy
• 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
• 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
Normal and HCM cellular
arrengement
• 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
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.
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
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.
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
Squatting , sustained handgrip position
with leg raising , expansion of volume in
pregnancy decreased obstruction
Laboratory evaluation
• ECG : LVH and deep broad Q waves
(mistake as a MI)
• CXR : Cardiomegaly
• Echocardiography : septal hypertrophy (
septum>=1.3 times of posterior wall )
• SAM of mitral valve with MR.
Apical HCM
• Apical hypertrophy
• Giant negative T wave on the ECG
• Spade – shaped LV cavity
Management
• Competitive sports and very strenuous
activities should be proscribed
• dehydration should be avoided.
• 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.
• Surgical myotomy / myectomy of septum and
ethanol injections into the septal artey are the
invasive treatment.
• ICD should be considered in high- risk patients
Restrictive
cardiomyopthy
• Hallmark of the RCM is abnormal diastole
function.
• Myocardial fibrosis,hypertrophy or
infiltration caused rigid LV walls
• Amyloidosis,
hemochromatosis,
glycogen storage disease,
endomyocardial fibrosis,
sarcoidosis,
hypereosinophilic disease,
sceleroderma,
following mediastinal irradiation
• 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
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.
Differentiation of RCM from
Constrictive pericarditis
important.
• Management is usually disappointing
except for hemochromatosis and fabry’s
disease.
Myocarditis
• 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.
• Most common cause is viruses especially
coxsakievirus B
adenovirus
hepatitis C
and HIV
• 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.
Clinical manifestation
• clinical spectrum ranges from an asymptomatic
state to fulminant condition with arrhythmias,
acute CHF and death.
• 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.
• Viral myocarditis is most often self-limited and
without sequelae but sometime may progress to a
chronic from and to DCM.