CardiomyopathyCardiomyopathy

Dr.mirdamadi Dr.mirdamadi

Cardiologist, Cardiologist, fellowship of fellowship of

echocardiograpyechocardiograpy

Definition:Definition:

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

Classification:

Dilated primary myocardial involvement

Restrictive secondary myocardial involvement:

Hypertrophic ( infective,

Metabolic,

Connective tissue disorders Infiltration,

toxin,

peripartum)

Dilated Dilated cardiomyopathy (DCM)cardiomyopathy (DCM)

• 1/3 of CHF is due to DCM 1/3 of CHF is due to DCM • LV and/or RV systolic function is LV and/or RV systolic function is

impaired leading to progressive impaired leading to progressive cardiac dilation cardiac dilation

• No cause detected in many cases, No cause detected in many cases, but familial (1/4-1/3 of cases) or but familial (1/4-1/3 of cases) or secondary cause like infectious, secondary cause like infectious, metabolic or toxic agents present. metabolic or toxic agents present.

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

Clinical features Clinical features

• symptoms of left and right sided CHF.symptoms of left and right sided CHF.

• some patients have LV dilation for some patients have LV dilation for months or even years before months or even years before becoming symptomatic becoming symptomatic

• vague chest pain (typical angina is vague chest pain (typical angina is unusual and suggestive of IHD)unusual and suggestive of IHD)

• syncope due to arrhythmias syncope due to arrhythmias

• systemic embolism systemic embolism

Physical examination Physical examination

• Narrow pulse pressure and elevated Narrow pulse pressure and elevated JVP in advanced disease JVP in advanced disease

• S3S3

• S4S4

• MRMR

• TRTR

Laboratory examinationLaboratory examination

• CXR : cardiomegaly , pulmonary CXR : cardiomegaly , pulmonary congestion congestion

• ECG : Sinus tachycardia or AF , ECG : Sinus tachycardia or AF , Ventricular arrhythmias, Non specific Ventricular arrhythmias, Non specific changes changes

• Echocardiography : LV dilation , systolic Echocardiography : LV dilation , systolic dysfunction dysfunction

• Angiography : to exclude IHDAngiography : to exclude IHD

• Endomyocardial biopsy :Endomyocardial biopsy :

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

• most patient has progressive course most patient has progressive course

• patients > 55 years die within 4 patients > 55 years die within 4 years of the onset of symptomsyears of the onset of symptoms

• Spontaneous improvement in about Spontaneous improvement in about one- quarter of patient one- quarter of patient

• death is due to progressive HF or death is due to progressive HF or ventricular arrhythmia or brady ventricular arrhythmia or brady arrhythmia arrhythmia

Alcoholic Alcoholic cardiomyopathy cardiomyopathy

• large quantities (>90g/d) of alcohol large quantities (>90g/d) of alcohol over many years cause DCM over many years cause DCM

• Risk of developing CMP is partially Risk of developing CMP is partially genetically genetically

• patient with severe CHF have a poor patient with severe CHF have a poor prognosis (< 1/4 of such patients prognosis (< 1/4 of such patients survive 3 years)survive 3 years)

• Second presentation of alcoholic Second presentation of alcoholic cardiotoxicity is recurrent cardiotoxicity is recurrent supraventricular or ventricular supraventricular or ventricular tachyarrhythmia’s tachyarrhythmia’s

• Holiday heart syndrome: AF, Atrial Holiday heart syndrome: AF, Atrial flutter or frequeut PVC after a flutter or frequeut PVC after a drinking binge. drinking binge.

Peripartum Peripartum cardiomyopathycardiomyopathy

• Cardiac dilation and CHF may Cardiac dilation and CHF may develop during the last trimester of develop during the last trimester of pregnancy or within 6 months of pregnancy or within 6 months of delivery. delivery.

• Mortality rate is 10%. Mortality rate is 10%.

• Patient who recover from peripartum Patient who recover from peripartum CMP should be encouraged to avoid CMP should be encouraged to avoid further pregnancy .further pregnancy .

DrugsDrugs

• A variety of drugs may damage the A variety of drugs may damage the myocard ium acutely (myocarditis) or they myocard ium acutely (myocarditis) or they may lead to chronic damage (like DCM)may lead to chronic damage (like DCM)

• Anthracycline derivatives : Anthracycline derivatives : • Doxorubicin cardiotoxicity may occur Doxorubicin cardiotoxicity may occur

acutely but more commonly develops 3 acutely but more commonly develops 3 months after the last dose.months after the last dose.

• TCA – antidepressants, phenothiazines , TCA – antidepressants, phenothiazines , lithium lithium

• Cocaine abuse (SCD , myocarditis , DCM, Cocaine abuse (SCD , myocarditis , DCM, acute MI )acute MI )

Arrhythmogenic right Arrhythmogenic right ventricular ventricular

cardiomyopathy / cardiomyopathy / Dysphasia (ARVC/D)Dysphasia (ARVC/D)

• ARVD is a familial CMP with ARVD is a familial CMP with progressive fibrofatty replacement of progressive fibrofatty replacement of the RV and to a much lesser degree the RV and to a much lesser degree LV.LV.

• Patients may present by RV failure or Patients may present by RV failure or ventricular tachycardia ventricular tachycardia

OthersOthers

• Neuromuscular disease Neuromuscular disease

• Tako – tsubo (stress) CMPTako – tsubo (stress) CMP

• Non compaction CMP Non compaction CMP

Hypertrophic Hypertrophic cardiomyopathycardiomyopathy

• characterized by LV hypertrophy characterized by LV hypertrophy without obvious cause without obvious cause

• It is 1 in 500 of general population It is 1 in 500 of general population

• two features af HCM : asymmetric LV two features af HCM : asymmetric LV hypertrophy , Dynamic LVOT hypertrophy , Dynamic LVOT obstruction obstruction

• There is a bizarre and disorganized There is a bizarre and disorganized arrangement of myocytes with arrangement of myocytes with variable degree of fibrosis.variable degree of fibrosis.

• About a half of patients have a About a half of patients have a positive family history compatible positive family history compatible with autosomal dominant with autosomal dominant transmission transmission

Normal and HCM Normal and HCM cellular arrengementcellular arrengement

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

Clinical featuresClinical features

• Clinical course is variable , from Clinical course is variable , from asymptomatic to SCDasymptomatic to SCD

• SCD frequently occur in children and SCD frequently occur in children and young adult during or after physical young adult during or after physical exertion.exertion.

• HCM is the most common cause of SCD in HCM is the most common cause of SCD in young competitive athletes young competitive athletes

• Other symptom : dyspnea , syncope , Other symptom : dyspnea , syncope , angina and fatigue.angina and fatigue.

Physical examinationPhysical examination

• Double or triple apical precordial Double or triple apical precordial impulse impulse

• Fourth heart sound Fourth heart sound

• Harsh , diamond – shaped systolic Harsh , diamond – shaped systolic murmur in LSB due to LVOT murmur in LSB due to LVOT obstruction obstruction

• Holosystolic and blowing systolic Holosystolic and blowing systolic murmur at apex due to MRmurmur at apex due to MR

Hemodynamic Hemodynamic

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

HemodynamicHemodynamic

• Three mechanisms are involved Three mechanisms are involved dynamic obstruction : dynamic obstruction :

• 1) increased LV contractility 1) increased LV contractility (exercise) (exercise)

• 2) decreased ventricular preload 2) decreased ventricular preload (strain phase of valsalva maneuver , (strain phase of valsalva maneuver , standing )standing )

• 3) decreased ventricular after load3) decreased ventricular after load

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

Laboratory evaluationLaboratory evaluation

• ECG : LVH and deep broad Q waves ECG : LVH and deep broad Q waves (mistake as a MI)(mistake as a MI)

• CXR : Cardiomegaly CXR : Cardiomegaly

• Echocardiography : septal Echocardiography : septal hypertrophy ( septum>=1.3 times of hypertrophy ( septum>=1.3 times of posterior wall )posterior wall )

• SAM of mitral valve with MR.SAM of mitral valve with MR.

Apical HCMApical HCM

• Apical hypertrophy Apical hypertrophy

• Giant negative T wave on the ECGGiant negative T wave on the ECG

• Spade – shaped LV cavity Spade – shaped LV cavity

ManagementManagement

• Competitive sports and very Competitive sports and very strenuous activities should be strenuous activities should be proscribed proscribed

• dehydration should be avoided.dehydration should be avoided.

• B- blockers ameliorate angina and syncope B- blockers ameliorate angina and syncope in1/3-1/4 of patientsin1/3-1/4 of patients

• Varapamil and diltiazem may reduce the Varapamil and diltiazem may reduce the stiffness of LV stiffness of LV

• Amiodaron reduced risk of SCD Amiodaron reduced risk of SCD andand arrhythmiaarrhythmia

• Digitalis,diuretics,nitrates, dihydropyridine Digitalis,diuretics,nitrates, dihydropyridine calcium blockers, vasodilators and B- calcium blockers, vasodilators and B- agonists are best avoided.agonists are best avoided.

• Surgical myotomy / myectomy of septum Surgical myotomy / myectomy of septum and ethanol injections into the septal artey and ethanol injections into the septal artey are the invasive treatment.are the invasive treatment.

• ICD should be considered in high- risk ICD should be considered in high- risk patientspatients

Restrictive Restrictive cardiomyopthycardiomyopthy

• Hallmark of the RCM is abnormal Hallmark of the RCM is abnormal diastole function.diastole function.

• Myocardial fibrosis,hypertrophy or Myocardial fibrosis,hypertrophy or infiltration caused rigid LV walls infiltration caused rigid LV walls

• Amyloidosis,Amyloidosis,

hemochromatosis,hemochromatosis,

glycogen storage disease, glycogen storage disease,

endomyocardial fibrosis,endomyocardial fibrosis,

sarcoidosis,sarcoidosis,

hypereosinophilic disease,hypereosinophilic disease,

sceleroderma,sceleroderma,

following mediastinal irradiation following mediastinal irradiation

• Inability of the ventricles to fill, Inability of the ventricles to fill, caused decrease cardiac out putcaused decrease cardiac out put

• exercise intolerance and dyspneaexercise intolerance and dyspnea

• elevated systemic venous pressure elevated systemic venous pressure cause edemacause edema

ascitis and elevated JVPascitis and elevated JVP

Laboratory examinationsLaboratory examinations

• in infiltrative disease in infiltrative disease

ECG often shows low – voltage, ECG often shows low – voltage, nonspecific ST/T change and various nonspecific ST/T change and various arrhythmias.arrhythmias.

• Echocardiography , CT and MRI showed Echocardiography , CT and MRI showed thickened LV walls with normal LV thickened LV walls with normal LV systolic function and dilated atria.systolic function and dilated atria.

Differentiation of RCM Differentiation of RCM from Constrictive from Constrictive

pericarditis important.pericarditis important.

• Management is usually disappointing Management is usually disappointing except for hemochromatosis and except for hemochromatosis and fabry’s disease.fabry’s disease.

MyocarditisMyocarditis

• myocarditis is most commonly the myocarditis is most commonly the result of infectious process, frequently result of infectious process, frequently complicated by autoimmunity.complicated by autoimmunity.

• myocarditis may also result from myocarditis may also result from hypersensitivity to drugs (TCA , hypersensitivity to drugs (TCA , antibiotics,antipsychotics) or may be antibiotics,antipsychotics) or may be caused by irradiation, chemicals or caused by irradiation, chemicals or physical agents.physical agents.

• Most common cause is viruses Most common cause is viruses especially coxsakievirus Bespecially coxsakievirus B

adenovirusadenovirus

hepatitis C hepatitis C

and HIVand HIV

  

• Patients with viral myocarditis may Patients with viral myocarditis may give a history of upper respiratory give a history of upper respiratory febrile illness or a flu like syndrome ,febrile illness or a flu like syndrome ,

and viral nasopharyngitis or and viral nasopharyngitis or tonsillitis may be evident.tonsillitis may be evident.

Clinical manifestationClinical manifestation

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

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

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