Approach to patient with Dilated Cardiomyopathy

1. DCMDCM
An approach to Diagnosis andAn approach to Diagnosis and
ManagementManagement
DR. MD. Saiful IslamDR. MD. Saiful Islam
MD (cardiology) Final part studentMD (cardiology) Final part student
Department of CardiologyDepartment of Cardiology
DMCHDMCH

2. CardiomyopathiesCardiomyopathies
Definition:Definition:
 It is a heterogenous group of diseaseIt is a heterogenous group of disease
of myocardium,of myocardium,
 associated with mechanical orassociated with mechanical or
electrical dysfunction,electrical dysfunction,
 which is usually but not invariablywhich is usually but not invariably
exhibits inappropriate ventricularexhibits inappropriate ventricular
hypertrophy or dilationhypertrophy or dilation
 & are due to variety of etiology that& are due to variety of etiology that
frequently are genetic.frequently are genetic.

3. WHO ClassificationWHO Classification
 PrimaryPrimary
(those resulting from(those resulting from
genetic abnormalitiesgenetic abnormalities
of cardiac muscle)of cardiac muscle)
– DilatedDilated
– HypertrophicHypertrophic
– RestrictiveRestrictive
 SecondarySecondary
(those resulting from(those resulting from
infections, metabolicinfections, metabolic
and nutritional diseases,and nutritional diseases,
endocrine disorders,endocrine disorders,
neuromuscular diseases,neuromuscular diseases,
blood diseases, tumors)blood diseases, tumors)
Br Heart J 1980; 44:672-673
Cardiomyopathies

4. CardiomyopathyCardiomyopathy
WHO Classification
1. Dilated
• Enlarged
• Systolic dysfunction
2. Hypertrophic
• Thickened
• Diastolic dysfunction
3. Restrictive
• Diastolic dysfunction
3. Arrhythmogenic RV dysplasia
• Fibrofatty replacement
3. Unclassified
• Fibroelastosis
• LV noncompaction
Circ 93:841, 1996

5. ACC/AHA classificationACC/AHA classification

6. Dilated CardiomyopathyDilated Cardiomyopathy
•Dilation and impaired contraction of ventricles:
•Reduced systolic function with or without heart failure
•Characterized by myocyte damage
•Multiple etiologies with similar resultant pathophysiology
•Majority of cases are idiopathic
•In 20-50 % of cases, the disease is recognized as familial.
•Autosomal dominant inheritance is most commonly
encountered and mutations in several cardiac structural or
metabolic genes have been identified.

7. DCMDCM
Main features:
dilatation of both
ventricles and atria
Mural thrombi are
frequently present
in LV
Scarring of the
mitral
and tricuspid
leaflets
and secondary
dilatation of their
Annuli
Reduced systolic
function

8. DCM: EtiologyDCM: Etiology
Idiopathic
Myocarditis
Ischemic
Infiltrative
Peripartum
Hypertension
Valvular
Familial
Inflammatory
Infectious
Viral – picornovirus, Cox B, CMV, HIV
Ricketsial - Lyme Disease
Parasitic - Chagas’ Disease, Toxoplasmosis
Non-infectious
Collagen Vascular Disease (SLE, RA)
Toxic
Alcohol, Anthracyclins (adriamycin), Cocaine
Metabolic
Endocrine –thyroid dz, pheochromocytoma, DM, acromegaly,
Nutritional
Thiamine, selenium, carnitine
Neuromuscular (Duchene’s Muscular Dystrophy--x-linked)

9. ETIOLOGIES OF DILATED CARDIOMYOPATHYETIOLOGIES OF DILATED CARDIOMYOPATHY
0
5
10
15
20
25
30
35
40
45
50
Disorder
IDCM
Myocarditis
Ischmic CM
Infiltrative
disease
Peripartum CM
Hypertension
HIV
CTD
Substance
abuse
Felker et al NEJM 2000

10. GeneticsGenetics
Some cardiomyopathies are monogenic disorders
Primary genetic – HCM, ARVC, LVNC
Mixed etiology – DCM (20-40 %), RCM (rare)
Great variability of genotype and phenotype
Hundreds and thousands of mutations
Many genes
Various types of inheritence
Different phenotypes in identical mutations

11. MOLECULAR DEFECTS IN DILATEDMOLECULAR DEFECTS IN DILATED
CARDIOMYOPATHYCARDIOMYOPATHY
Fatkin D, et al. NEJM 1999;341Fatkin D, et al. NEJM 1999;341
GENES
Dystrophin
Desmin
Vinculin
Titin
Troponin-T
α-tropomyosin
ß-myosin heavy chain
Actin
Mitochondrial DNA
mutations

12.  Pathogenisis :DCMPathogenisis :DCM

13. PathophysiologyPathophysiology
Mutation Exogenous
insult
Contraction and relaxation
disorder
Ineffective energy utilization
Altered Ca ions handling
Activation of compensatory
neurohumoral mechanisms
Apoptosis
Fibrosis
Hypertrophy
Heart
failure
Arrhythmia, sudden
death
Thromboembolic
complication

14. PathophysiologyPathophysiology
•Initial Compensation for impaired myocyte contractility:
•Frank-Starling mechanism
•Neurohumoral activation
∀↑ intravascular volume
•Eventual decompensation
•ventricular remodeling
•myocyte death/apoptosis
•valvular regurgitation

15. PhysiologicPhysiologic
consequencesconsequences

16. • Failure of the LV causes an increase in end-diastolicFailure of the LV causes an increase in end-diastolic
volume, which results in increase in LA, pulmonaryvolume, which results in increase in LA, pulmonary
venous and pulmonary capillary pressure.venous and pulmonary capillary pressure.
Mitral valve regurgitation may result from papillary muscleMitral valve regurgitation may result from papillary muscle
dysfunction or severe dilatation of the valve annulus.dysfunction or severe dilatation of the valve annulus.
Idiopathic
Dilated
Cardiomyopathy

17. IDCMIDCM
EPIDEMIOLOGYEPIDEMIOLOGY
 ANNUAL INCIDENCEANNUAL INCIDENCE 5- 8/100,0005- 8/100,000
 INCREASED RISK ASSOCIATED WITH:INCREASED RISK ASSOCIATED WITH:
– MALE GENDERMALE GENDER
– BLACK RACEBLACK RACE
– HYPERTENSIONHYPERTENSION
– CHRONIC BETA-AGONIST USECHRONIC BETA-AGONIST USE

18. IDCMIDCM
PATHOGENESISPATHOGENESIS
 Familial/genetic factorsFamilial/genetic factors
 Viral myocarditis and cytotoxic insultsViral myocarditis and cytotoxic insults
 Immunologic abnormalitiesImmunologic abnormalities
– Beta-receptor auto-antibodiesBeta-receptor auto-antibodies
– Abnormal T-cell functionAbnormal T-cell function
 Metabolic, energetic, and contractileMetabolic, energetic, and contractile
abnormalitiesabnormalities
CaCa2+2+
-ATPase-ATPase
Myofibrillar ATPaseMyofibrillar ATPase
Creatine KinaseCreatine Kinase

19. IDCMIDCM
Pathological consequencePathological consequence
 Four chamber dilatationFour chamber dilatation
 Varying degrees of interstitial fibrosis andVarying degrees of interstitial fibrosis and
myocyte hypertrophymyocyte hypertrophy
 ““Functional” atrioventricular regurgitation isFunctional” atrioventricular regurgitation is
commoncommon
 Normal epicardial coronary arteriesNormal epicardial coronary arteries

20. IDCMIDCM
PATHOLOGIC FINDINGSPATHOLOGIC FINDINGS

21. IDCMIDCM
CLINICAL PRESENTATIONSCLINICAL PRESENTATIONS
 Heart failure symptomsHeart failure symptoms 75%-85%75%-85%
 Anginal chest painAnginal chest pain 8%-20%8%-20%
 EmboliEmboli (systemic or pulmonary)(systemic or pulmonary) 1%-4%1%-4%
 SyncopeSyncope <1%<1%
 Sudden cardiac deathSudden cardiac death <1%<1%

22. DCM: FamilialDCM: Familial
 30% of ‘idiopathic’30% of ‘idiopathic’
 Inheritance patternsInheritance patterns
– Autosommal dom/rec, x-linked, mitochondrialAutosommal dom/rec, x-linked, mitochondrial
mutationmutation
 DiagnosisDiagnosis
– Three generation family historyThree generation family history is essential foris essential for
diagnosisdiagnosis
– Screening of family member & genetic testingScreening of family member & genetic testing
 Mechanism:Mechanism:
– Abnormalities in:Abnormalities in:
 Energy productionEnergy production
 Contractile force generationContractile force generation
– Specific genes coding for:Specific genes coding for:


23. DCM : IschemicDCM : Ischemic
 Defined as -cardiomyopathy in theDefined as -cardiomyopathy in the
presence of:presence of:
– Prior extensive myocardial infractionPrior extensive myocardial infraction
– Severe coronary artery diseaseSevere coronary artery disease
 ICM-ICM- causes 60% to 70% cases of systoliccauses 60% to 70% cases of systolic
heart failure in industrilized countriesheart failure in industrilized countries

24. DCM: PeripartumDCM: Peripartum
Diagnostic CriteriaDiagnostic Criteria
 1 mo pre, 5 mos post1 mo pre, 5 mos post
 Echo: LV dysfunctionEcho: LV dysfunction
– LVEF < 45%LVEF < 45%
– FS < 30%FS < 30%
 EpidemiologyEpidemiology
 1:4000 women1:4000 women
– JAMA 2000;283:1183JAMA 2000;283:1183
 Proposed mechanisms:Proposed mechanisms:
– Inflammatory Cytokines:Inflammatory Cytokines:

25. DCM: toxicDCM: toxic
Alcoholic cardiomyopathyAlcoholic cardiomyopathy
 Chronic useChronic use
 Reversible with abstinenceReversible with abstinence
 Continued use is associated with a 3Continued use is associated with a 3
to 6 year mortility excedding 50%to 6 year mortility excedding 50%
 Mechanism?:Mechanism?:
– Directly inhibits:Directly inhibits:
 mitochondrial oxidative phosphorylationmitochondrial oxidative phosphorylation
 Fatty acid oxidationFatty acid oxidation
– Myocyte cell death and fibrosisMyocyte cell death and fibrosis

26. DCM: InfectiousDCM: Infectious
Acute viral myocarditisAcute viral myocarditis
 Coxasackie B or echovirusCoxasackie B or echovirus
 Self-limited infection in young peopleSelf-limited infection in young people
 Mechanism?:Mechanism?:
– Myocyte cell death and fibrosisMyocyte cell death and fibrosis
– Immune mediated cell injuryImmune mediated cell injury

27. Difference betweenDifference between
DCM and ICMDCM and ICM
 DCMDCM
1.1. Global hypokinesiaGlobal hypokinesia
2.2. RWMA and theRWMA and the
coronary territorycoronary territory
do not conformdo not conform
3.3. Dyskinesia notDyskinesia not
seenseen
4.4. RV involvement isRV involvement is
oftenoften
 ICMICM
1.1. RegionalRegional
hypokinesiahypokinesia
2.2. RWMA followsRWMA follows
coronary territorycoronary territory
3.3. Dyskinesia is seenDyskinesia is seen
4.4. RV involvement isRV involvement is
rarerare

28. Diagnosis of DCMDiagnosis of DCM
 HistoryHistory
 Clinical examinationClinical examination
 Chest x-rayChest x-ray
 12-lead ECG12-lead ECG
 EchocardiographyEchocardiography
 Blood testsBlood tests
 Family screening and genetic testingFamily screening and genetic testing
 Endomyocardial biopsyEndomyocardial biopsy
 Cardiac CT ScanCardiac CT Scan //
MRI (magnetic resonance imaging)MRI (magnetic resonance imaging)
 Cardiac catheterizationCardiac catheterization

29. Associated history withAssociated history with
DCMDCM
 Alcohol consumptionAlcohol consumption
 Peripartum statusPeripartum status
 IHDIHD
 MalnutritionMalnutrition
 Hereditary involvement-3 generationHereditary involvement-3 generation
of family history is essentialof family history is essential
 Diabetes mellitusDiabetes mellitus
 Endocrine disorder(thyroid)Endocrine disorder(thyroid)
 Valvular disorder( eg. AR , MR)Valvular disorder( eg. AR , MR)

30. Clinical ManifestationsClinical Manifestations
Symptoms:Symptoms:
 All age group may be affectedAll age group may be affected
 May be asymptomaticMay be asymptomatic
 The onset is insidious, but sometimes symptoms of heartThe onset is insidious, but sometimes symptoms of heart
failure occur suddenlyfailure occur suddenly
 DyspneaDyspnea
 PalpitationPalpitation
 Chest painChest pain
 Dizziness and syncopeDizziness and syncope
 CVA,Hemiplegia,ParaplegiaCVA,Hemiplegia,Paraplegia
 Systemic embolismSystemic embolism
 Infants and younger children haveInfants and younger children have respiratory symptomsrespiratory symptoms
and failure to thriveand failure to thrive..

31. Clinical ManifestationsClinical Manifestations
Signs:Signs:
 Pulse : Sinus tachycardia, Irregular in AF or inPulse : Sinus tachycardia, Irregular in AF or in
extrasystol, Bradycardia in CHBextrasystol, Bradycardia in CHB
 BP : Low BP , Narrow pulse pressureBP : Low BP , Narrow pulse pressure
 JVP : ElevatedJVP : Elevated
 Precordium:Precordium:
– Apex shifted, forceful and ill sustainedApex shifted, forceful and ill sustained
– S2 may be palpable due to PHS2 may be palpable due to PH
– May be parasternal liftMay be parasternal lift
– On auscultation:S1 and S2 soft, S2 loud from PHOn auscultation:S1 and S2 soft, S2 loud from PH
, Pansystolic murmur at mitral and tricuspid, Pansystolic murmur at mitral and tricuspid
area,S3 and S4 are commonarea,S3 and S4 are common

32. Other peripheral signs:Other peripheral signs:
 Liver : Enlarged , tender and pulsatileLiver : Enlarged , tender and pulsatile
 Edama : at dependent partEdama : at dependent part
 Lung base: Basal crepitationLung base: Basal crepitation
 Others: Signs of systemic andOthers: Signs of systemic and
pulmonary embolism may be foundpulmonary embolism may be found

33. ACC/AHA HEART FAILURE EVALUATION GUIDELINESACC/AHA HEART FAILURE EVALUATION GUIDELINES
CLASS I & II RECOMMENDATIONSCLASS I & II RECOMMENDATIONS
 Laboratory StudiesLaboratory Studies
– Blood count, urinalysis, electrolytes, renal function,Blood count, urinalysis, electrolytes, renal function,
glucose, LFTsglucose, LFTs (class I; level C)(class I; level C)
– Thyroid stimulating hormoneThyroid stimulating hormone (class I; level C)(class I; level C)
– Fe/TIBC, ferritinFe/TIBC, ferritin (class IIa, level C)(class IIa, level C)
– Urinary screening for hemochromatosisUrinary screening for hemochromatosis (class IIa;(class IIa;
level C)level C)
– Measurement of ANA, rheumatoid factor, urinaryMeasurement of ANA, rheumatoid factor, urinary
VMA and metanepherines in selected patientsVMA and metanepherines in selected patients
(class IIa; level C)(class IIa; level C)
– HIV testingHIV testing (class IIb; level C)(class IIb; level C)
 ElectrocardiogramElectrocardiogram (class I; level C)(class I; level C)
 Chest x-rayChest x-ray (class I; level C)(class I; level C)
 Echocardiogram/Doppler or radioventriculogramEchocardiogram/Doppler or radioventriculogram (class(class
I;level C)I;level C) -Adapted from Hunt SA et al. Circulation 2001;104:2996-3007

34. ECG Finding:ECG Finding:
 Sinus tachycardia, BradycardiaSinus tachycardia, Bradycardia
 Low voltage ECGLow voltage ECG
 LVHLVH
 Rt or Lt Atrial hypertrophyRt or Lt Atrial hypertrophy
 Atrial or Ventricular arrythmiaAtrial or Ventricular arrythmia
 Pseudo infraction pattern(Deep but narrow ‘q’Pseudo infraction pattern(Deep but narrow ‘q’
suggestive of antero-septal MI)suggestive of antero-septal MI)
 Shallow invesion of ‘T’ waveShallow invesion of ‘T’ wave
 LBBB, RBBB ,LAHB, LPHB, Bifascicular orLBBB, RBBB ,LAHB, LPHB, Bifascicular or
Trifascicular blockTrifascicular block
 AV block( 1’or 2’ or 3’degree)AV block( 1’or 2’ or 3’degree)

35. ECGECG

36. X-rayX-ray
 Cardiomegally ,simulting pericardial effusionCardiomegally ,simulting pericardial effusion
or multi vaivular disease or Ischemicor multi vaivular disease or Ischemic
cardiomyupathycardiomyupathy
 Pulmonary edema with Kerley’ B linePulmonary edema with Kerley’ B line
 Pleural effusion may be presentPleural effusion may be present
 Pulmonary infraction is not uncommonPulmonary infraction is not uncommon
 Usually no LA enlargement so single rightUsually no LA enlargement so single right
border. No calcified valve and normal aortaborder. No calcified valve and normal aorta

37. X-rayX-ray

38. EchocardiographyEchocardiography
Typical FeaturesTypical Features
 2D Echo of DCM2D Echo of DCM
1.1. All four chambers are dilatedAll four chambers are dilated
2.2. LV wall thin with hypokinesiaLV wall thin with hypokinesia
 M mode echo of DCMM mode echo of DCM
1.1. Valve-Normal in morphology with reduced excursionValve-Normal in morphology with reduced excursion
2.2. EPSS is increasedEPSS is increased
3.3. Mitral valve shows Penguin appearance in the sea shoreMitral valve shows Penguin appearance in the sea shore
 Color dopplerColor doppler
1.1. Functional MR and TR with trivial ARFunctional MR and TR with trivial AR
 Comment : DCMComment : DCM

39. EchocardiographyEchocardiography

40. INDICATIONS FOR ENDOMYOCARDIALINDICATIONS FOR ENDOMYOCARDIAL
BIOPSYBIOPSY
 AcuteAcute dilated cardiomyopathy withdilated cardiomyopathy with refractory heartrefractory heart
failurefailure symptomssymptoms
 Rapidly progressiveRapidly progressive ventricular dysfunction in anventricular dysfunction in an
unexplainedunexplained cardiomyopathy ofcardiomyopathy of recent onsetrecent onset
 New onsetNew onset cardiomyopathy withcardiomyopathy with recurrentrecurrent ventricularventricular
tachycardia or high grade heart blocktachycardia or high grade heart block
 Heart failure in the setting of fever, rash, and peripheralHeart failure in the setting of fever, rash, and peripheral
eosinophiliaeosinophilia
 Dilated cardiomyopathy in setting ofDilated cardiomyopathy in setting of systemic diseasessystemic diseases
known to affect the myocardiumknown to affect the myocardium (systemic lupus(systemic lupus
erythematosus, polymyositis, sarcoidosis)erythematosus, polymyositis, sarcoidosis)
Wu LA, et al. Mayo Clin Proc 2001;76:1030-8

41. RIGHT
VENTRICULAR
BIOPSY
ENDOMYOCARDIAL BIOPSY IN DILATED
CARDIOMYOPATHY

42. NON-INVASIVE EVALUATION OF MYOCARDITISNON-INVASIVE EVALUATION OF MYOCARDITIS
MRI IMAGINGMRI IMAGING
Friedrich MG et al. Circulation 1998;97:1802-9.
EnhancedEnhanced

43. INDICATIONS FORINDICATIONS FOR CORONARY ANGIOGRAPHY INCORONARY ANGIOGRAPHY IN
NEW ONSET CARDIOMYOPATHYNEW ONSET CARDIOMYOPATHY
ACC/AHA CONSENSUS GUIDELINESACC/AHA CONSENSUS GUIDELINES
 Patients with Known Coronary Artery Disease/Angina PectorisPatients with Known Coronary Artery Disease/Angina Pectoris
– Revascularization recommended in vast majority of suchRevascularization recommended in vast majority of such
individuals with multivessel disease. Little role for non-invasiveindividuals with multivessel disease. Little role for non-invasive
testing.testing.
– Coronary angiography consideredCoronary angiography considered Class IClass I RecommendationRecommendation
(Level of evidence: B)(Level of evidence: B)
 Patients with Known Coronary Artery Disease Who Lack AnginaPatients with Known Coronary Artery Disease Who Lack Angina
– No controlled trials have examined whether coronaryNo controlled trials have examined whether coronary
revascularization can improve outcomes in this populationrevascularization can improve outcomes in this population
– Many centers first evaluate patient for myocardial hibernationMany centers first evaluate patient for myocardial hibernation
– Coronary angiography consideredCoronary angiography considered Class IIaClass IIa RecommendationRecommendation
(Level of Evidence:C)(Level of Evidence:C)
 Patients with or without Chest Pain in Whom Coronary ArteryPatients with or without Chest Pain in Whom Coronary Artery
Disease has Not Been EvaluatedDisease has Not Been Evaluated
– Approximately 35% of patients with IDCM will report angina-likeApproximately 35% of patients with IDCM will report angina-like
painpain
– Coronary angiography should be consideredCoronary angiography should be considered Class IIaClass IIa
recommendation (Level of Evidence: C)recommendation (Level of Evidence: C) Hunt SA,et al. Circulation 2001;104:2996

44. Aim of ManagementAim of Management
•Heart failure therapy:
1. Drug therapy
2. Mechanical assist devices
3. Heart transplant
•Prevention of sudden cardiac death
•Prevention of thromboembolic complications

45. Treatment of DCMTreatment of DCM
 No specific treatmentNo specific treatment
 Modification of contributory factor:Modification of contributory factor:
– Avoid alcoholAvoid alcohol
– Avoid pregnancyAvoid pregnancy
– Genetic counsellingGenetic counselling
– Limit exerciseLimit exercise
– Weight reductionWeight reduction
– Control of blood pressureControl of blood pressure
– Promt control of InfectionPromt control of Infection
– Correction of anaemia and malnutritionCorrection of anaemia and malnutrition

46. SupportiveSupportive
medical treatment:medical treatment:
 Treatment of HF:Treatment of HF:
– RestRest
– Salt and water restrictionSalt and water restriction
– Diuretics (eg. Frusemide)Diuretics (eg. Frusemide)
– DigoxinDigoxin
– Vasodilators (eg.ACE inhibitor , Nitrates,Vasodilators (eg.ACE inhibitor , Nitrates,
Prazocin )Prazocin )
-Potassium supplements-Potassium supplements
-Spironolactone-Spironolactone
-Vitamin: B1-Vitamin: B1
-Levocarnitine-Levocarnitine

47. Con..Con..
 Anti arrythmic drugs:Anti arrythmic drugs:
– 90% patient has complex ventricular90% patient has complex ventricular
arrythmiaarrythmia
– IF AF: DigoxinIF AF: Digoxin
– If VT or VF :AmiodoroneIf VT or VF :Amiodorone
 Long term anticoagulant therapy:Long term anticoagulant therapy:
– To prevent systemic and pulmonaryTo prevent systemic and pulmonary
embolismembolism
 Consider adding beta blocker if symptomsConsider adding beta blocker if symptoms
persistpersist


48. Con..Con..
• Intervension
• ICD: Patients with documented arrhythmias or a
history of unexplained syncope should be treated
aggressively, usually with an implantable
cardioverter-defibrillator (ICD). Prevention of
sudden death with implantable ICD is efficacious
• CRT: For heart failure in DCM-Using special
pacing technique (Bi ventricular)
• Surgery treatment
– ventricular septal myotomy (disabling angina, syncope
associated with LV outflow obstruction)
– mitral valve replacement (if obstruction cannot be
alleviated)
Cardiac transplantation

49. Implantation ofImplantation of cardiovertercardioverter--
defibrillatordefibrillator
Patients with severely
depressed myocardial
function should be
monitored for
arrhythmias and, if
present, treated
aggressively with
antiarrhythmic agents
or an
implantable
cardioverter-
defibrillator
(ICD).

50. CRT: CardiacCRT: Cardiac
Resynchronization TherapyResynchronization Therapy
1. Improved hemodynamics1. Improved hemodynamics
– Increased COIncreased CO
– Reduced LV fillingReduced LV filling
pressurespressures
– Reduced sympatheticReduced sympathetic
activityactivity
– Increased systolic functionIncreased systolic function
2. Reverse LV2. Reverse LV
remodeling/architectureremodeling/architecture
– Decreased LVES/EDDecreased LVES/ED
volumesvolumes
– Increased LVEFIncreased LVEF
– Circ ’02, JACC ’02,Circ ’02, JACC ’02,
JACC ’02, NEJM’02JACC ’02, NEJM’02

51. Newly emerging treatment for DCMNewly emerging treatment for DCM
 Immunosuppressive andImmunosuppressive and
immunomodulation therapyimmunomodulation therapy
 Gene therapyGene therapy
 Cellular transplantationCellular transplantation
– Fetal cardiomyocytesFetal cardiomyocytes
– Skeletal myoblastsSkeletal myoblasts
– Adult stem cellsAdult stem cells
– Embryonic stem cellsEmbryonic stem cells

52. Complication of DCMComplication of DCM
 Heart failureHeart failure
 Arrythmias : AF ,SVT,PVCs ,VT VFArrythmias : AF ,SVT,PVCs ,VT VF
 AV blocks:1’ 2’ and CHBAV blocks:1’ 2’ and CHB
 BBB : RBBB ,LBBB, LAHB ,LPHBBBB : RBBB ,LBBB, LAHB ,LPHB
 Systolic or pulmonary embolismSystolic or pulmonary embolism
 SyncopeSyncope
 Sudden cardiac deathSudden cardiac death

53. PrognosisPrognosis
 The course of disease is progressively downhill,The course of disease is progressively downhill,
although some patients may remain stable foralthough some patients may remain stable for
years.years.
 Treatment of HF may result in temporaryTreatment of HF may result in temporary
remission, but relapses are common and in timeremission, but relapses are common and in time
– patients become resistant to therapy.– patients become resistant to therapy.
 Prognosis for survival beyond a year is poor.Prognosis for survival beyond a year is poor.
 50% die within 2 years of diagnosis50% die within 2 years of diagnosis
 Nearly all die in 5 yearsNearly all die in 5 years
 10% may recover10% may recover
 25-40% survives for 10 years25-40% survives for 10 years

54. IDCM:PROGNOSTIC FEATURESIDCM:PROGNOSTIC FEATURES
 VENTRICULOGRAPHIC FINDINGSVENTRICULOGRAPHIC FINDINGS
– Degree of impairment in LVEFDegree of impairment in LVEF
– Extent of left ventricular enlargementExtent of left ventricular enlargement
– Coexistent right ventricular dysfunctionCoexistent right ventricular dysfunction
– Ventricular mass/volume ratioVentricular mass/volume ratio
– Global wall motion abnormalitiesGlobal wall motion abnormalities
– Left ventricular sphericityLeft ventricular sphericity
 CLINICAL FINDINGSCLINICAL FINDINGS
– Favorable prognosisFavorable prognosis:: NYHA < IV, younger age, femaleNYHA < IV, younger age, female
sexsex
– Poor prognosisPoor prognosis:: Syncope, persistent S3 gallop, right-Syncope, persistent S3 gallop, right-
sided heart failure, AV or bundle branch block,sided heart failure, AV or bundle branch block,
hyponatremia, troponin elevation, increased BNP,hyponatremia, troponin elevation, increased BNP,
maximum oxygen uptake < 12 mg/kg/minmaximum oxygen uptake < 12 mg/kg/min

55. SummarySummary
 Cardiomyopathies are diseases of the heartCardiomyopathies are diseases of the heart
muscle and are classified based on theirmuscle and are classified based on their
structural and functional phenotypestructural and functional phenotype
 Disorders are frequently geneticDisorders are frequently genetic
 Accurate differentiation is needed in order toAccurate differentiation is needed in order to
guide treatment and managementguide treatment and management

56. Thanks for payingThanks for paying
attention!attention!