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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.
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
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
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
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
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)
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
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
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
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).
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
Initial Compensation for impaired myocyte contractility includes:
Frank-Starling mechanism
EDV (ventricular dilation) to stroke work
Neurohumoral ( sympathetic activation)
HR x SV (contractility) to maintain C.O.
renal blood flow
renin: Angiotensin II PVR
Aldosterone & intravascular volume
Eventual decompensation
PVR and intravascular volume overburden ventricles
pulmonary and systemic congestion
Tricuspid and mitral regurgitation from annular dilatation
The threshold of left ventricular enlargement and dysfunction necessary to diagnose peripartum cardiomyopathy has not been precisely defined. The following definition, based upon a 1992 NHLBI workshop definition for idiopathic dilated cardiomyopathy, has been proposed [34,35]:
Left ventricular ejection fraction (LVEF) less than 45 percent AND/OR M-mode fractional shortening less than 30 percent PLUS Left ventricular end-diastolic dimension greater than 2.7 cm/m2