CARDIOMYOPATHY

1

Definition

• Disorders of the cardiac muscle of unknown aetiology

• “A primary disorder of the heart muscle that causes abnormal myocardial performance and is not the result of disease or dysfunction of other cardiac structures … myocardial infarction, systemic hypertension, valvular stenosis or regurgitation”

2

Classifications

Braunwald 8th ed, 2008.3

Functional Classification• Dilatated (congestive, DCM, IDC)

– ventricular enlargement and syst dysfunction

• Hypertrophic (IHSS, HCM, HOCM)– inappropriate myocardial hypertrophy

in the absence of HTN or aortic stenosis

• Restrictive (infiltrative)– abnormal filling and diastolic function

4

IDIOPATHIC DILATED CARDIOMYPATHYEPIDEMIOLOGY

• ANNUAL INCIDENCE 5-8/100,000

• PREVELANCE 36/ 100,000

• INCREASED RISK ASSOCIATED WITH:– MALE GENDER– BLACK RACE– HYPERTENSION– CHRONIC BETA-AGONIST USE

5

ETIOLOGIES OF DILATED CARDIOMYOPATHY

0

5

10

15

20

25

30

35

40

45

50

Disorder

IDCM

Myocarditis

Ischmic CM

InfiltrativediseasePeripartum CM

Hypertension

HIV

CTD

Substanceabuse

Felker et al NEJM 20006

IDIOPATHIC DILATED CARDIOMYOPATHYPATHOLOGY

• Four chamber dilatation• Mild to moderate ventricular

hypertrophy• Varying degrees of interstitial

fibrosis and myocyte hypertrophy

• “Functional” atrioventricular regurgitation is common

• Normal epicardial coronary arteries

7

IDIOPATHIC DILATED CARDIOMYOPATHYPATHOGENESIS

• Familial/genetic factors• Viral myocarditis and cytotoxic insults• Immunologic abnormalities• Beta-receptor auto-antibodies

– Abnormal T-cell function

• Metabolic, energetic, and contractile abnormalities– Ca2+-ATPase– Myofibrillar ATPase– Creatine Kinase

8

MOLECULAR DEFECTS IN DILATED CARDIOMYOPATHY

Fatkin D, et al. NEJM 1999;341Fatkin D, et al. NEJM 1999;341

GENESLamin A/Cδ-sarcoglycanDystrophinDesminVinculinTitinTroponin-Tα-tropomyosinß-myosin heavy chainActin

Mitochondrial DNA mutations

9

IDIOPATHIC DILATED CARDIOMYPATHYCLINICAL PRESENTATIONS

• Heart failure symptoms 75%-85%• Anginal chest pain 8%-20%

• Emboli (systemic or pulmonary) 1%-4%• Syncope <1%• Sudden cardiac death <1%

10

IDIOPATHIC DILATED CARDIOMYPATHYCARDIAC IMAGING

• CXR : enlargement of cardiac silhoutte• ECG : evidence of old MCI, conduction abnormalities e.g

LBBB, LV hypertrophy, AF or VT• 24-hour ambulatory ECG (Holter)

– lightheadedness, palpitation, syncope

• Two-dimensional echocardiogram or Radionuclide ventriculography to assess : LV ejection fraction, end-diastolic volume, diastolic function

• Cardiac catheterization– age >40, ischemic history, high risk profile, abnormal ECG

11

IDCM:PROGNOSTIC FEATURES

• VENTRICULOGRAPHIC FINDINGS– Degree of impairment in LVEF– Extent of left ventricular enlargement– Coexistent right ventricular dysfunction– Ventricular mass/volume ratio– Global wall motion abnormalities– Left ventricular sphericity

• CLINICAL FINDINGS– Favorable prognosis: NYHA < IV, younger age, female

sex

– Poor prognosis: Syncope, persistent S3 gallop, right-sided heart failure, AV or bundle branch block, hyponatremia, troponin elevation, increased BNP, maximum oxygen uptake < 12 mg/kg/min

12

IDIOPATHIC DILATED CARDIOMYPATHYPREDICTING PROGNOSIS

Predictive Possible Not PredictiveClinical factors symptoms alcoholism age

peripartum durationfamily history viral illness

Hemodynamics LVEF LV sizeCardiac index atrial pressure

Dysarrhythmia LV cond delay AV block simple VPCcomplex VPC atrial fibrillation

Histology myofibril volume other findings

Neuroendocrine hyponatremiaplasma norepinephrineatrial natriuretic factor 13

RIGHT VENTRICULAR BIOPSY TECHNIQUE

ENDOMYOCARDIAL BIOPSY IN DILATED CARDIOMYOPATHY

14

INDICATIONS FOR ENDOMYOCARDIAL BIOPSY

• Acute dilated cardiomyopathy with refractory heart failure symptoms

• Rapidly progressive ventricular dysfunction in an unexplained cardiomyopathy of recent onset

• New onset cardiomyopathy with recurrent ventricular tachycardia or high grade heart block

• Heart failure in the setting of fever, rash, and peripheral eosinophilia

• Dilated cardiomyopathy in setting of systemic diseases known to affect the myocardium (systemic lupus erythematosus, polymyositis, sarcoidosis)

Wu LA, et al. Mayo Clin Proc 2001;76:1030-815

IDIOPATHIC DILATED CARDIOMYOPATHYMANAGEMENT

• Limit activity based on functional status• salt restriction of a 2-g Na+ (5g NaCl) diet• fluid restriction for significant low Na+• initiate medical therapy

– ACE inhibitors, diuretics– digoxin, carvedilol– hydralazine / nitrate combination

16

IDIOPATHIC DILATED CARDIOMYOPATHYMANAGEMENT

• consider adding ß-blocking agents if symptoms persists

• anticoagulation for EF <30%, history of thromboemoli, presence of mural thrombi

• intravenous dopamine, dobutamine and/or phosphodiesterase inhibitors

• cardiac transplantation

17

HYPERTROPHIC CARDIOMYOPATHY

• First described by the French and Germans around 1900

• uncommon with occurrence of 0.02 to 0.2%• a hypertrophied and non-dilated left ventricle in

the absence of another disease

• small LV cavity, asymmetrical septal hypertrophy (ASH), systolic anterior motion of the mitral valve leaflet (SAM)

18

HYPERTROPHIC CARDIOMYOPATHY EPIDEMIOLOGY

• Prevalence : 1 per 500 in US

• Higher in Black individual• Inherited in an

autosomal dominant pattern

• Predominance in younger Male

• Commonest cause of sudden death during exertion in young people

19

HYPERTROPHIC CARDIOMYOPATHY PATHOGENESIS

• Misconception that outflow tract obstruction• Impaired ventricular compliance as

consequence of inappropriate myocardial hypertrophy

20

HYPERTROPHIC CARDIOMYOPATHY PATOPHYSIOLOGY

• Systole– dynamic outflow tract gradient

• Diastole– impaired diastolic filling, filling pressure

• Myocardial ischemia– muscle mass, filling pressure, O2 demand– vasodilator reserve, capillary density– abnormal intramural coronary arteries– systolic compression of arteries

21

HYPERTROPHIC CARDIOMYOPATHY MYOCARDIAL DISARRAY

Normal Muscle Structure Myocardial Disarray

(Parallel alignment) (Disorganised alignment)22

HYPERTROPHIC CARDIOMYOPATHY TYPES

• HCM or HOCM• Asymmetric septal (ASH) - without obstruction• Asymmetric septal (ASH) - with obstruction• Symmetric hypertrophy - concentric• Apical hypertrophy

23

65% 35%

10%

www.kanter.com/hcm24

HCM

Mitral Valve in normal position

25

HOCM

Mitral valve presses against septum

MR

26

SYMMETRIC

symmetric or

concentric27

APICAL

Small cavity

remains

Apical Hypertrophy28

HYPERTROPHIC CARDIOMYOPATHY CLINICAL PRESENTATIONS

• Some patients asymptomatic• Symptomatic

– dyspnea in 90%– angina pectoris in 75%– fatigue, pre-syncope, syncope

risk of SCD in children and adolescents– palpitation, PND, CHF, dizziness less frequent

• Costello syndrome• ECG : giant negative T waves in the precordial leads• Spade like appearence

29

HYPERTROPHIC CARDIOMYOPATHY PHYSICAL EXAMINATIONS

• Carotid impulse• Prominent a waves of JV pulse• Outflow murmur• Mitral regurgitation• Atrial fibrilation• Embolic phenomena• Heart Failure symptoms

30

HYPERTROPHIC CARDIOMYOPATHY DIAGNOSTIC APPROACH

ECG :• Abnormalities of ST segment and T waves• LVH• QRS complex tallest at mid precordial leads

Echo :• Concentric caused by pressure overload e.g. AS• Eccentric caused by volume overload e.g MR, AR• Septal thickening wall• Mitral valve anterior leafleat may be enlarged• MR

31

32

HYPERTROPHIC CARDIOMYOPATHY DIFFERENTIAL DIANOSIS

• Left Ventricular hypertrophy• Outflow obstruction secondary to valvular

heart disease e.g AS, coarctation of aorta and infiltrative disorder of myocardium

• Pattern hypertrophy in hypertension is concentric meanwhile in HCM is distinctive

33

HCM vs Aortic Stenosis

HCM Fixed Obstruction

carotid pulse spike and dome parvus et tardusmurmur radiate to carotids

valsalva, standing squatting, handgrip passive leg elevation

systolic thrill 4th left interspace 2nd right interspacesystolic click absent present

34

Hypertrophic CardiomyopathyTreatment algorithm

35

HYPERTROPHIC CARDIOMYOPATHY MANAGEMENT

Medical management:• B-blockers• CCB, verapamil• Disopyramide in decreasing outflow gradient• Endocarditis prophylaxisPermanent PacingI C D Alcohol ablation of the septum• Injected 1-4 ml absolute alcohol into the septal

perforator branch of LAD

36

HYPERTROPHIC CARDIOMYOPATHY MANAGEMENT

Surgical therapy• Subaortic ventricular myotomy• Resection myocardium from proximal septum to

beyond mitral leafleatAdvantage :• Low mortality• Reduced symptoms• Improved functional capacityHeart transplantation

37

Restrictive Cardiomyopathy

38

RESTRICTIVE CARDIOMYOPATHY

• Hallmark: abnormal diastolic function• rigid ventricular wall with impaired

ventricular filling• importance lies in its differentiation from

operable constrictive pericarditis • Characteristics by : Abnormal compliance of

the left ventricle and short relaxation time

39

RESTRICTIVE CARDIOMYOPATHY ETIOLOGY

•Non infiltrative cause Associated with patchy endomyocardial fibrosis, increased cardiac mass and enlarged atria•Infiltrative cause

Amyloidisis, primary caused by the deposition of amyloid protein. Secondary caused by production nonimunoglobulin protein and termed AA

•Sarcoidosis•Endomyocardial fibrosis

40

RESTRICTIVE CARDIOMYOPATHY EXCLUSION “GUIDELINES”

• LV end-diastolic dimensions 7 cm• Myocardial wall thickness 1.7 cm• LV end-diastolic volume 150 mL/m2• LV ejection fraction < 20%

41

RESTRICTIVE CARDIOMYOPATHY CLASSIFICATION

• Idiopathic• Myocardial

1. Noninfiltrative– Idiopathic– Scleroderma

2. Infiltrative– Amyloid– Sarcoid– Gaucher disease– Hurler disease

3. Storage Disease– Hemochromatosis– Fabry disease– Glycogen storage

• Endomyocardial– endomyocardial fibrosis– Hyperesinophilic synd– Carcinoid– metastatic malignancies– radiation, anthracycline

42

RESTRICTIVE CARDIOMYOPATHY CLINICAL PRESENTATIONS

• Symptoms of right and left heart failure• Angina if CAD involve• Jugular Venous Pulse

– prominent x and y descents• Echo-Doppler

– abnormal mitral inflow pattern– prominent E wave (rapid diastolic filling)– reduced deceleration time ( LA pressure)

43

RESTRICTIVE CARDIOMYOPATHY DIAGNOSTIC APPROACH

ECG :• Low voltage• Poor R wave progression• Pseudoinfarction pattern in the inferior leads• P pulmonal If pulmonary hipertension present• Atrial arrhythmia esp fibrillation• Sick sinus syndrome is common• Ventricular Tachiarrhytmia

44

RESTRICTIVE CARDIOMYOPATHY DIAGNOSTIC APPROACH

Chest X-ray• Normal CTR and enlarged atria• Enlarged right ventricle may be seen if pulmonary

hypertension presentEcho• Severe biatrial enlargement• Thickened LV wallsCardiac nuclear imagingCT scan and MRICardiac catheterization and endomyocardial biopsy

45

• No satisfactory medical therapy• Drug therapy must be used with caution

– diuretics for extremely high filling prssures– vasodilators may decrease filling pressure– ? Calcium channel blockers to improve diastolic

compliance– digitalis and other inotropic agents are not

indicated

RESTRICTIVE CARDIOMYOPATHY MANAGEMENT

46

RESTRICTIVE CARDIOMYOPATHY DIFFERENTIAL DIAGNOSIS

• Constrictive pericarditis• Chronic RV infarction• RV dysfunction from RV pressure or RV

volume overload• Instrinsic RV myocardial disease• Tricuspid valve disease

47

Constrictive - Restrictive Pattern“Square-Root Sign” or “Dip-and-Plateau”

48

49

RESTRICTIVE CARDIOMYOPATHY Restriction vs Constriction

History provide can important clues• Constrictive pericarditis

– history of TB, trauma, pericarditis, sollagen vascular disorders

• Restrictive cardiomyopathy– amyloidosis, hemochromatosis

• Mixed– mediastinal radiation, cardiac surgery

50

MYOCARDITIS

• Inflammation of myocardium• Can be result of systemic disorder or

infectious agent• Viral-Coxsackie B, echovirus, influenza,

parainfluenza, Epstein-Bar, and HIV• Bacterial-C. Diptheria, N. meningitidis, M.

pneumonia, and beta-hemolytic strep• Frequently accompanied with pericarditis

51

MYOCARDITISClinical Presentations

• Fever, tachycardia out of proportion to fever, myalgias, headache,rigors

• Chest pain due to coexisting pericarditis• Pericardial friction rub• Severe cases may have CHF symptoms

52

MYOCARDITISCLINICAL PRESENTATIONS

• EKG-nonspecific changes, av block, prolonged QRS suration, or ST elevation(with pericarditis)

• CXR-Normal• Cardiac Enzymes- may be elevated• Differentail-ischemia or infarct, valvular

disease, and sepsis

53

MYOCARDITIS

MANAGEMENT • Supportive care• Blood cultures• Antibiotics for bacterial cause• Watch for signs of progressive heart failure

54

Pericardial disease

55

• Fibrous sac surrounding heart-dense network of collagen fibres

• Serous membrane – two continuous layers

separated by a small amount of fluid lubricant (10-20mls )

• Layers are :– Visceral is inner layer

(epicardium)– Parietal is continuous with

diaphragm and outer walls of great arteries

56

ACUTE PERICARDITIS

• Loose visceral pericardium and dense parietal pericardium surround heart

• Pericardial space may contain up to 50ml normally

• Etiologies of acute pericarditis-viral, bacterial, fungal, malignancy, drugs, radiation, connective tissue disorder, uremia, myxedema, post-MI, or idiopathic

57

ACUTE PERICARDITIS ETIOLOGY

Idiopathic – 86%• Infective (viral or bacterial) – 7%• Following a myocardial infarction or cardiac surgery

(Dressler’s syndrome)• Radiation therapy• Neoplastic disease (commonly lung or breast) – 6%• Connective tissue disease

Figures from Permanyer-Miralda et al 198558

ACUTE PERICARDITIS CLINICAL PRESENTATIONS

• Retrosternal chest pain – sharp or stabbing pain worse on insp and lying flat

• Friction rub (high pitched scratching noise)

• Raised jugular venous pressure

59

ACUTE PERICARDITIS DIAGNOSIS APPROACH

• EKG-changes in four stages• 1-ST elevation in I, V5 and V6, PR

depression in II, aVF and V4-V6• 2-ST segment normalizes, T wave

decreases• 3-Inverted T waves in leads with

previous ST elevation• 4-Return to normal ECG• In I, V5, or V6 ST:Twave ratio

>0.25 most likely acute pericarditis

60

ACUTE PERICARDITIS DIAGNOSIS APPROACH

• Chest Xray-normal and can help r/o other disease

• Other tests of value-CBC, bun and cr, streptococcal serology, viral serologies, antinuclear/anti-DNA abs, thyroid function, ESR, Cardiac Enzymes

61

ACUTE PERICARDITIS MANAGEMENT

• Search for the underlying disease• No good evidence from randomised controlled trials• Patients require bed rest• NSAID (aspirin, indomethacin) are generally accepted

as effective for relieving symptoms of chest pain• NSAID ketorolac tromethamine rapid results• Colchicine may be a useful adjunct in those who do

not respond to NSAIDs alone

62

ACUTE PERICARDITIS PROGNOSIS

• Pericarditis is usually a benign disorder• Diagnosis relates to underlying cause• But any cause can lead to an effusion and

tamponade which can lead to death• Pericarditis can also progress to pericardial

constriction and heart failure

63

CONSTRICTIVE PERICARDITIS

• Occurs when fibrous thickening and loss of elasticity interfere with diastolic filling

• Cardiac trauma, pericardiotomy, intrapericardial hemmorhage, fungal or bacterial pericarditis, uremic pericarditis are most common causes

64

CONSTRICTIVE PERICARDITIS CLINICAL PRESENTATIONS

• Sx’s gradually develop-mimics restrictive CM- CHF, DOE, and decreased exercise tolerance

• Chest pain, orthopnea and pnd are uncommon

• Exam-Pedal edema, hepatomegaly, ascites, jvd, and Kussmaul’s sign.

• Pericardial “knock”-early diastolic sound may be heard at apex

65

CONSTRICTIVE PERICARDITIS DIAGNOSIS APPROACH

• EKG-not very helpful-may show low voltage QRS and inverted T waves

• CXR-pericardial calcifications seen in 50% on lateral view(not diagnostic)

• ECHO, CT, MRI are diagnostic

66

CONSTRICTIVE PERICARDITIS DIFFERENTIAL DIAGNOSIS

• Consider acute pericarditis, myocarditis, exacerbation of chronic ventricular dysfunction, or systemic process resulting in decreased cardiac performance(sepsis)

67

CONSTRICTIVE PERICARDITIS MANAGEMENT

• Supportive care• Symptomatic patients require admission and

pericardiectomy

68

PERICARDIAL EFFUSION

• Major causes are post cardiac surgery and • Neoplastic disease• Gradual accumulation of fluid (chronic) permits

progressive stretching of pericardium• Patient may develop a substantial fluid without

significant increase in intrapericardial pressure

69

PERICARDIAL EFFUSION PATOPHYSIOLOGY

• Significantly increased intrapericardial pressure impedes diastolic filling of the ventricles

• Therefore in order for the ventricles to fill the end-diastolic pressure must exceed the pericardial pressure

• Global effusion – pericardial pressure is equal around heart

• Therefore both ventricles have to increase EDP to same amount for ventricles to fill

70

PERICARDIAL EFFUSION DIAGNOSTIC APPROACH

• Clinical examination – SOB, orthopnoea, tachycardia (varying degrees)

• Auscultation – may have muffled heart sounds• ECG may show low amplitude QRS complexes and

alternating axis• CXR – globular appearance to heart and therefore

increased cardiothoracic ratio• Echo – size of effusion and haemodynamic effect of it

71

C X R ECHO

72

PERICARDIAL EFFUSION TREATMENT

• Depends on the cause and nature• If acute the cause is treated and the patient

monitored• If persistent problem or life threatening more

dramatic action is called for

73

CARDIAC TAMPONADE

• Occurs when the fluid accumulation around the heart impairs filling to such an extent that there is haemodynamic compromise.

• It is a medical emergency and must be treated promptly.

• Risk of death depends upon speed of diagnosis, treatment and underlying cause of the tamponade.

74

CARDIAC TAMPONADE CLINICAL PRESENTATIONS

• Dyspnea and decreased exercise tolerance-wt loss, pedal edema, ascites

• Tachycardia, Narrow pulse pressure• Pulsus paradoxus• JVD, Muffled heart sounds, Hypotension

75

CARDIAC TAMPONADE DIAGNOSTIC APPROACH

• EKG-low voltage QRS with ST elevation and PR depression possible

• Electrical Alternans-classic finding—P and R wave beat to beat variability

• CXR-+/- enlarged cardiac silhoutte “globular heart”• ECHO :

- Size and location of effusion- Any evidence of diastolic collapse- ‘Swinging’ of the heart- Decrease of insp. flow across MV

76

CARDIAC TAMPONADE DIAGNOSTIC APPROACH

• IV Fluid Bolus-improves RV filling and improves hemodynamics

• Pericardiocentesis-therapeutic and diagnostic

• Admission to ICU or monitored setting

77