Myocardial Myocardial diseasesdiseases By Dr. Osman Bukhari By Dr. Osman Bukhari

11 - -MyocarditisMyocarditis

22 - -CardiomyopathyCardiomyopathy

MyocarditisMyocarditis This is acute inflammation of the This is acute inflammation of the

myocardium due to myocardium due to diff causesdiff causes which are diff to establish during which are diff to establish during routine clinical practiceroutine clinical practice..

11 - -InfectiveInfective-: -:

a- Virusesa- Viruses ++++ (Cox, Echo, Adeno, ++++ (Cox, Echo, Adeno, HIV and others.)HIV and others.)

b- Bacteriab- Bacteria (Rh fever, Diphtheria,) (Rh fever, Diphtheria,)

c- Spirochaetalc- Spirochaetal (Lyme, (Lyme, Leptospirosis)Leptospirosis)

d- Parasiticd- Parasitic ( Chagas , ( Chagas , Toxoplasmosis )Toxoplasmosis )

e- Rickettsiale- Rickettsial f- Chlamydialf- Chlamydial

g- Fungalg- Fungal

22 - -Non infectiveNon infective-: -:

a- Toxic a- Toxic b- Radiation b- Radiation

c- Autoimmunec- Autoimmune d-Drugs d-Drugs (Antibiotics by (Antibiotics by hypersensitivity, CQ & Emetine hypersensitivity, CQ & Emetine

by direct action )by direct action )

Clinical pictureClinical picture-:-: - -Usually acuteUsually acute onset, but may be onset, but may be gradual gradual

- - There may be a There may be a history of recent febrile history of recent febrile or upper respiratory illness or upper respiratory illness . .

- - Pleural- pericardial chest painPleural- pericardial chest pain is common is common

- - Tachycardia, low pulse volume, hypoten, Tachycardia, low pulse volume, hypoten, soft HS, S3, gallop, signs of HF soft HS, S3, gallop, signs of HF

- - Pericardial rubPericardial rub

- - SubclinicalSubclinical, presenting later with CM, presenting later with CM

- - Sudden deathSudden death

InvestigationsInvestigations-:-:- - CXR : CXR : Cardiomegaly Cardiomegaly

- - ECGECG : ST & T wave abn, : ST & T wave abn, arrhythmias. HT block with diph, arrhythmias. HT block with diph, Chagas & LymeChagas & Lyme..

- - Increased Increased cardiac enzymescardiac enzymes in active in active disease disease

- - EchoEcho

- - Endomyovardial biopsyEndomyovardial biopsy in in specialized unitsspecialized units

- - Serology for virusesSerology for viruses

TreatmentTreatment-:-: - - Bed restBed rest

- - TR of z causeTR of z cause

- - Management of HF & arrhythmiasManagement of HF & arrhythmias

- - SteroidsSteroids? ?

Prognosis:Prognosis: depends on z aetiology & is depends on z aetiology & is usually good withusually good with remission remission in in most cases. Few run most cases. Few run fatal coursefatal course. . Chronic CMChronic CM may ensue may ensue

CardiomyopathiesCardiomyopathies (CM) (CM)

CM are a group of CM are a group of cardiac musclecardiac muscle diseasesdiseases not due tonot due to HT, IHD, HT, IHD, Valvular or cong heart disease. Valvular or cong heart disease. Many cases are idiopathic (Pry) Many cases are idiopathic (Pry) but some have specific causesbut some have specific causes..

Cardiomyopathies are classified Cardiomyopathies are classified according to the clinical according to the clinical presentation to: 1- presentation to: 1- Dilated CM Dilated CM 2- Hypertrophic CM 2- Hypertrophic CM 3- Restrictive CM 3- Restrictive CM 4- Arrhythmogenic RV CM 4- Arrhythmogenic RV CM

Dilated cardiomyopathyDilated cardiomyopathy DCM is characterized by DCM is characterized by dilatation dilatation

andand impaired systolic functionimpaired systolic function of of the LV and/ or RV in the absence of the LV and/ or RV in the absence of HT, valvular or IHDHT, valvular or IHD

CausesCauses-:-:11 - -Idiopathic DCMIdiopathic DCM ( primary) diagnosed ( primary) diagnosed

by exclusion of 2ndry causes. In by exclusion of 2ndry causes. In 25 % it is 25 % it is familial.familial. 2- Secondary DCM2- Secondary DCM

a- Post infectivea- Post infective

b- Alcoholb- Alcohol

c- Conn. tissue dis. c- Conn. tissue dis. d- Autoimmune d- Autoimmune

e- Endocrinee- Endocrine (DM, acromegaly, (DM, acromegaly, myxoedema, thyrotoxicosis) myxoedema, thyrotoxicosis)

f- Amyloidosis f- Amyloidosis g- Haemochromatosis g- Haemochromatosis

h- Thiamine & Se deficiency h- Thiamine & Se deficiency i- Glycogen storage dis. i- Glycogen storage dis. j- Neuromuscular disorders j- Neuromuscular disorders ( Friedrieck’s ataxia, dystrophia ( Friedrieck’s ataxia, dystrophia myotonica & Duch)myotonica & Duch)

k- Small vessel coronary disk- Small vessel coronary dis . .

l- Drugsl- Drugs ( Doxorubicin, daunorubicin, ( Doxorubicin, daunorubicin, cocaine, cyclophosphamide ) cocaine, cyclophosphamide )

m- Post partum CM m- Post partum CM n- Radiation n- Radiation

o- SS dis. o- SS dis. P- Sarcoidosis P- Sarcoidosis

ClinicallyClinically_:_: - -Symptom & signs of Lt & Rt heart Symptom & signs of Lt & Rt heart

failurefailure

- - Syncope from arrhythmias & Syncope from arrhythmias & conduction defectsconduction defects..

- - Systemic or pulm embolism Systemic or pulm embolism - Asymptomatic cardiomegaly - Asymptomatic cardiomegaly

- - Cardiomegaly, S3 gallop, MR & TRCardiomegaly, S3 gallop, MR & TR

- - Sudden deathSudden death

InvestigationsInvestigations-:-: - -CXRCXR: Cardiomegaly & pulm : Cardiomegaly & pulm

congestioncongestion

-- EchoEcho: Dilated ventricles with poor : Dilated ventricles with poor global systolic functionglobal systolic function

-- ECGECG: Diffuse non specific ST segment : Diffuse non specific ST segment & T wave changes, tachycardia, & T wave changes, tachycardia, arrhythmias, conduction abnarrhythmias, conduction abn..

- -Angiography Angiography to exclude CAD in those to exclude CAD in those at riskat risk

TreatmentTreatment-:-: Aim is Aim is to relieve symptoms, retard disease to relieve symptoms, retard disease

progression & prevent complications progression & prevent complications..

- -Alcohol must be discontinuedAlcohol must be discontinued..

- - TR secondary causesTR secondary causes

- -Conventional TR of HFConventional TR of HF. . ACE- I & BBACE- I & BB are are indicated in most cases. indicated in most cases. DigoxinDigoxin for AF. for AF. Severe Vt dilatation, AF & history of Severe Vt dilatation, AF & history of embolization are indications for embolization are indications for anticoagulationanticoagulation

- -Antiarrhythmic therap, permanent Antiarrhythmic therap, permanent pacingpacing

or or implantable cardiovertorimplantable cardiovertor (defibrillators in specific cases(defibrillators in specific cases..

- -Cardiac transplantationCardiac transplantation for advanced for advanced dis. refractory to med. TRdis. refractory to med. TR

- -HF is resistant to TR and there is high HF is resistant to TR and there is high risk of thromboembolism & risk of thromboembolism &

progression to deathprogression to death . .

Hypertrophic cardiomyopathyHypertrophic cardiomyopathy11 - -In HCM there is variableIn HCM there is variable muscle muscle

hypertrophyhypertrophy commonly commonly involving involving IVS,IVS, unrelated to any unrelated to any pressure or volume overload, pressure or volume overload, restricting LV filling during restricting LV filling during diastole.diastole. In 25% there is In 25% there is LV outflowLV outflow obstructionobstruction due to combined effect due to combined effect of hypertrophy & SAM of za of hypertrophy & SAM of za anterior Mitral valve leaflet (anterior Mitral valve leaflet (HOCM)HOCM) or idiopathic hypertrophic or idiopathic hypertrophic

subaortic stenosis (subaortic stenosis (IHSS)IHSS) . .

22 --The abn. muscle is a focus of The abn. muscle is a focus of dangerous arrhythmiasdangerous arrhythmias, sp VT which , sp VT which may convert to VF & HCM is may convert to VF & HCM is potentially fatal & pat must potentially fatal & pat must avoid avoid severe exercisesevere exercise which may provoke which may provoke arrhythmiasarrhythmias..

33--The The obst. is worsenedobst. is worsened by factors that by factors that increase myocardial contractility or increase myocardial contractility or

decreased LV filling and size decreased LV filling and size 44 - -Obst. is decreasedObst. is decreased by by factors that factors that

increasincreas venous return venous return..55 - -In 50 % it is familial-AD inheritanceIn 50 % it is familial-AD inheritance. .

Sporadic cases occur. Presentation Sporadic cases occur. Presentation in in early adulthoodearly adulthood

66 - -HFHF results from stiff non compliant LV results from stiff non compliant LV with impaired diastolic filling with impaired diastolic filling

77 - -MRMR results from SAM results from SAM88 - -Systolic LV function is preserved till Systolic LV function is preserved till

latelate when progressive dilatation when progressive dilatation my occurmy occur..

ClinicallyClinically-:-: 11 - -AsymptomaticAsymptomatic

22 - -Chest pain, dyspnoea & syncopeChest pain, dyspnoea & syncope 33 - -Cardiac arrhythmias & sudden deathCardiac arrhythmias & sudden death

44--Jerky carotid pulse because of rapidJerky carotid pulse because of rapid ejectionejection..

55 - -Double apical pulsationDouble apical pulsation due to forceful due to forceful atrial contraction producing T4 atrial contraction producing T4

66 - -Ejection syst. murmurEjection syst. murmur in za Lt sternal in za Lt sternal border due to LV outflow obst. border due to LV outflow obst. Intensity isIntensity is increasedincreased by factors by factors that decrease LV size (e.g. that decrease LV size (e.g. Valsalva,s, amyl nitrate, standing & Valsalva,s, amyl nitrate, standing & digoxin) by bringing up za ant. digoxin) by bringing up za ant. Leaflet of MV close to the septum. Leaflet of MV close to the septum. Situations that increase Situations that increase cardiac size by increasing venous cardiac size by increasing venous return return decrease za intensitydecrease za intensity of z of z murmur e.g. BB, lying down and murmur e.g. BB, lying down and squatting squatting..

77--Pansyst. murmur of MR secondary to Pansyst. murmur of MR secondary to SAMSAM

88 - -S4S4

99 - -Sudden deathSudden death sp. those who present sp. those who present early, FH of sudden death, early, FH of sudden death, history of syncope , those with history of syncope , those with VT on ambulatory ECG & abn BP VT on ambulatory ECG & abn BP response during exerciseresponse during exercise . .

InvestigationsInvestigations:: 11 - -CXRCXR: Usually unremarkable: Usually unremarkable

22 - -ECG:ECG: LV hypertrophy, ST-Tw LV hypertrophy, ST-Tw changes, Q waves in inferolat. changes, Q waves in inferolat. leads in up to 25 % resulting from leads in up to 25 % resulting from hypertrophy of IVS 3hypertrophy of IVS 3- Echo is - Echo is diagnostic: diagnostic: ASH, SAM & small ASH, SAM & small hyper contractile LVhyper contractile LV

44 - -Ambulatory ECG: Ambulatory ECG: prognostic prognostic informationinformation

55 - -Pedigree analysisPedigree analysis

ManagementManagement-:-: Aim is to prevent sudden death & Aim is to prevent sudden death &

relieve anginarelieve angina..

11 - -Amidarone & implantable Amidarone & implantable defibrillatorsdefibrillators for za arrhythmias for za arrhythmias..

22 - -Chest pain & dyspnoea are treated Chest pain & dyspnoea are treated by by BBBB and and verapamilverapamil or or disopyramidedisopyramide. . 33- Avoid digoxin - Avoid digoxin & vasodilators& vasodilators as these may as these may increase outflow obstincrease outflow obst..

44 - -In severe obst. In severe obst. surgical resection of surgical resection of muscles below Ao valve muscles below Ao valve..

55 - -Non surgical septaL alcohol Non surgical septaL alcohol injectioninjection in some in some..

66 - -Screen family membersScreen family members by echo for by echo for early diagnosis & TR of early diagnosis & TR of complicationscomplications

Restrictive CMRestrictive CM11 - -Characterized by Characterized by impaired diastolic impaired diastolic

fillingfilling symptom & signs of HF.symptom & signs of HF. 2 2-The contractile function of za -The contractile function of za ventricles is preserved. ventricles is preserved. 3-Dilatation of za atria with 3-Dilatation of za atria with thrombus formation is commonthrombus formation is common..

44 - -Conditions associated with RCMConditions associated with RCM include include amyloidosis, sarcoidosis, amyloidosis, sarcoidosis, Lofflers endocarditis, radiation, Lofflers endocarditis, radiation, EMF, post surg. myocardial fibrosis, EMF, post surg. myocardial fibrosis, haemochromatosis, carcinoid haemochromatosis, carcinoid

syndrome & sclerodermasyndrome & scleroderma. .

55--Idiopathic form of RCM may be familialIdiopathic form of RCM may be familial..

ClinicallyClinically-:-:11 - -Dyspnoea, fatigue & embolic symptomsDyspnoea, fatigue & embolic symptoms

are za presenting features are za presenting features..

22 - -persistently elevated JVP, persistently elevated JVP, hepatomegaly, ascites & dependent hepatomegaly, ascites & dependent edemaedema due to restricted RV filling due to restricted RV filling-.-.

33 - -Kussmaul,s sign, S4 & cardiac Kussmaul,s sign, S4 & cardiac enlargement with S3 in advanced enlargement with S3 in advanced cases cases. Cardiac size may remain normal. Cardiac size may remain normal..

44 - -Should be diff from constrictiveShould be diff from constrictive pericarditispericarditis.. Normal LV function & Normal LV function & thick pericardium suggest CPthick pericardium suggest CP..

InvestigationsInvestigations-:-:11 - -CXRCXR: Normal or slightly enlarged : Normal or slightly enlarged

cardiac size with atrial enlargement cardiac size with atrial enlargement and pulm congestionand pulm congestion..

22 - -ECG: ECG: Low voltage, ST segment & T Low voltage, ST segment & T wave abn wave abn..

33 - -EchoEcho: myocardial thickening , normal : myocardial thickening , normal systolic EF with impaired diast. systolic EF with impaired diast. FillingFilling..

44 - -Endomyocardial biopsyEndomyocardial biopsy

( amyloidosis) ( amyloidosis) TreatmentTreatment-:-: 11 - -No specific TRNo specific TR

22--TR HF & embolic manifestationsTR HF & embolic manifestations

33--TR underlying causeTR underlying cause e. g. e. g. amyloidosis with melphalan, amyloidosis with melphalan, prednisolone +/- colchicine. prednisolone +/- colchicine. Sarcoidosis with Sarcoidosis with prednisoloneprednisolone..

44 - -Cardiac transplantationCardiac transplantation in severe in severe cases sp idiopathiccases sp idiopathic..

Arrhythmogenic RV CMArrhythmogenic RV CM-Characterized by : Characterized by :

1- Progressive fibro fatty replacement 1- Progressive fibro fatty replacement of RV myocardiumof RV myocardium

- 22 - -Familial in 50% with ADFamilial in 50% with AD- 33--The condition is often The condition is often

asymptomaticasymptomatic 4- Risk of vent 4- Risk of vent arrhythmia & suddenarrhythmia & sudden

- deathdeath.++.++- 55 - - RT HF or CHFRT HF or CHF later with ventricular later with ventricular - dilatation and paradoxical decrease dilatation and paradoxical decrease

inin - arrhythmiasarrhythmias . .

66 - -CXR-CXR- Unremarkable except in Unremarkable except in advanced casesadvanced cases..

77 - -ECG-ECG- T wave inversion in V1-3,epsilon T wave inversion in V1-3,epsilon waves at z end of QRS & RBBB waves at z end of QRS & RBBB..

88 - -EchoEcho– Normal early—Dilated RV later– Normal early—Dilated RV later..

99 - -RV biopsy-RV biopsy-=Fibro fatty tissue=Fibro fatty tissue..

1010 - -BBBB 1 1stst line TR for non life line TR for non life threatening arrhythmias. threatening arrhythmias. Amiodarone & sotalolAmiodarone & sotalol for for symptomatic refractory symptomatic refractory

arrhythmiasarrhythmias . .

1111 - -Cardiac transplantationCardiac transplantation..