CARDIOMYOPATHIES

1. Cardiomyopathies

2. Objectives
 Discuss definition, etiologies and classification of
CMP
 Discuss dilated, hypertrophic, and restrictive
cardiomyopathy
 Review current medical management of patients
with cardiomyopathy

3. Definition
A group of diseases that primarily affect the
heart muscle and are not the result of disease or
dysfunction of other cardiac structures
(congenital/acquired valvular, hypertensive,
coronary arterial, or pericardial abnormalities).

4. Etiology
1. Cardiovascular disease (remodeling)
2. Infectious
3. Toxins
4. Systemic connective tissue diseases
5. Infiltrative and proliferative diseases
6. Nutritional deficiencies
7. Idiopathic

5. Classification on an etiologic basis
 Primary type
- Heart muscle disease of unknown cause
 Secondary type
– Myocardial disease of known cause
– Associated with a disease involving other
organ system
– Specific cardiomyopathy by WHO

6. Classif. From Harris

7. Peripartum heart disease(D)

8. Peripartum heart disease(D)

11. WHO Classification
 Unknown cause
(primary)
 Dilated
 Hypertrophic
 Restrictive
 unclassified
 Specific heart muscle
disease (secondary)
 Infective
 Metabolic
 Systemic disease
 Heredofamilial
 Sensitivity
 Toxic

12. Idiopathic Dilated
Cardiomyopathy

13. IDC - Definition
 a disease of unknown etiology that principally
affects the myocardium
 pathology
 increased heart size and weight
 ventricular dilatation, normal wall thickness
 heart dysfunction out of portion to fibrosis

14. IDC cont…
 About one in three cases of CHF is due to DCM.
 LV and/or right ventricular (RV) systolic pump
function is impaired, leading to progressive
cardiac dilatation (remodeling).
 Symptoms of heart failure (HF) typically appear
only after remodeling has been ongoing for some
time (months or even years).

15. Secondary DCM
 End result of myocardial damage produced by a variety
of known or unknown infectious, metabolic, or toxic
agents.
 ? Immunologic: DCM may be the late consequence of
acute viral myocarditis.
 A reversible form of DCM may be found with alcohol
abuse, pregnancy, thyroid disease, cocaine use, and
chronic uncontrolled tachycardia.

16. Incidence and Prognosis
 3-10 cases per 100,000
 Age: may occur at any age
 most commonly becomes apparent clinically in the 3rd or 4th decades.
 Blacks 2x more frequent than whites
 Men 3x more frequent than women
 Symptoms may be gradual in onset
 death from progressive pump failure
1-year 25%
2-year 35-40%
5-year 40-80%
 stabilization observed in 20-50% of patient
 complete recovery is rare

17. History and Physical Examination
 Symptoms of heart failure
 pulmonary congestion (left HF)
dyspnea (rest, exertional, nocturnal), orthpnea
 systemic congestion (right HF)
edema, nausea, abdominal pain, nocturia
 low cardiac output
fatigue and weakness
 Hypotension, narrow pulse pressure, tachycardia,
tachypnea, JVD, S3,S4, MR/TR

18. Cardiac Imaging
 Chest radiogram:
 enlargement of the cardiac silhouette due to LV dilatation, although
generalized cardiomegaly is often seen.
 Pulmonary vascular redistribution and interstitial or, in advanced cases,
alveolar edema.
 Electrocardiogram:
 sinus tachycardia or atrial fibrillation, ventricular arrhythmias, left atrial
abnormality, low voltage, diffuse nonspecific ST-T-wave abnormalities, and
sometimes intraventricular and/or AV conduction defects.
 Echocardiography, computed tomographic imaging (CTI), and
cardiac magnetic resonance imaging (CMRI)
 LV dilatation, with normal, minimally thickened, or thinned walls, and
systolic dysfunction.
 Radionuclide ventriculography
 Cardiac catheterization and coronary angiography
 age >40, ischemic history, high risk profile, abnormal ECG
 Circulating levels of brain natriuretic peptide are usually elevated.

19. Management of DCM
 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
 ß-blocking agents

20. Management of DCM
 anticoagulation for EF <30%, history of
thromboemoli, presence of mural thrombi
 intravenous dopamine, dobutamine and/or
phosphodiesterase inhibitors
 cardiac transplantation

21. Hypertrophic
Cardiomyopathy

22. Hypertrophic Cardiomyopathy
 A disorder caused by mutations in the genes that code for
various proteins within the sarcomere.
 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)

23. 65% 35%
10%
www.kanter.com/hcm

24. Pathophysiology
 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

25. Clinical Manifestation
 About half of all patients with HCM have a positive
family history compatible with autosomal dominant
transmission.
 Asymptomatic, echocardiographic finding
 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

26. Physical Examination
 Most patients demonstrate a double or triple
apical precordial impulse and a fourth heart
sound.
 a rapidly rising arterial pulse: Those with
intraventricular pressure gradients.
 systolic murmur:
 the hallmark of obstructive HCM, which is typically
harsh, diamond-shaped, & usually begins well after
1st heart sound.

27. Laboratory Evaluation
 ECG: LV hypertrophy and widespread deep, broad Q
waves, T wave inversions.
 Chest X-ray: may be normal, although a mild to
moderate increase in the cardiac silhouette is common.
 Echocardiogram: The mainstay of the diagnosis of HCM
 LV hypertrophy, often with the septum 1.3 times the
thickness of the posterior LV free wall.
 CMRI is superior to echocardiography in providing
accurate measurements of regional hypertrophy and in
identifying sites of regional fibrosis.

28. Natural History
 annual mortality 3% in referral centers probably closer to
1% for all patients
 risk of SCD higher in children may be as high as 6% per
year majority have progressive hypertrophy
 clinical deterioration usually is slow
 progression to DCM occurs in 10-15%

29. Recommendations for Athletic
Activity
 Avoid most competitive sports (whether or not symptoms
and/or outflow gradient are present)
 Low-risk older patients (>30 yrs) may participate in athletic
activity if all of the following are absent
 ventricular tachycardia on Holter monitoring
 family history of sudden death due to HCM
 history of syncope or episode of impaired consciousness
 severe hemdynamic abnormalities, gradient 50 mmHg
 exercise induced hypotension
 moderate or sever mitral regurgitation
 enlarged left atrium (50 mm)
 paroxysmal atrial fibrillation
 abnormal myocardial perfusion

30. Management
 beta-adrenergic blockers
 calcium antagonist
 disopyramide
 amiodarone, sotalol
 Pacing
 myotomy-myectomy
 plication of the anterior mitral leaflet

31. Restrictive
Cardiomyopathy

32. Restrictive Cardiomyopathies
 Hallmark: abnormal diastolic function
 rigid ventricular wall with impaired ventricular
filling
 bear some functional resemblance to
constrictive pericarditis
 importance lies in its differentiation from
operable constrictive pericarditis

33. 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%

34. 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

35. Clinical Manifestations
 Symptoms of right and left heart failure
 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)

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

37. Restriction vs Constriction
History provide important clues
 Constrictive pericarditis
 history of TB, trauma, pericarditis, collagen
vascular disorders
 Restrictive cardiomyopathy
 amyloidosis, hemochromatosis
 Mixed
 mediastinal radiation, cardiac surgery

38. Laboratory Examinations
 ECG: low-voltage, nonspecific ST-T-wave abnormalities and
various arrhythmias.
 x-ray: Pericardial calcification which occurs in constrictive
pericarditis, is absent.
 Echocardiography, CTI, and CMRI typically reveal symmetrically
thickened LV walls and normal or slightly reduced ventricular
volumes and systolic function; the atria are usually dilated.
 Doppler echocardiography typically shows diastolic dysfunction.
 Cardiac catheterization shows a reduced cardiac output, elevation
of the RV and LV end-diastolic pressures, and a dip-and-plateau
configuration of the diastolic portion of the ventricular pressure
pulses resembling constrictive pericarditis.

39. Treatment
 No satisfactory medical therapy
 Chronic anticoagulation is often recommended to
reduce the risk of embolization from the heart.
 Drug therapy must be used with caution
 diuretics for extremely high filling pressures
 vasodilators may decrease filling pressure
 ? Calcium channel blockers to improve diastolic compliance
 digitalis and other inotropic agents are not indicated

40. Summary

41. Clinical/Functional Classification of
Cardiomyopathies
1. Dilated (congestive, DCM, IDC)
 Left and/or right ventricular enlargement, impaired systolic function,
congestive heart failure, arrhythmias, emboli
2. Hypertrophic ( HCM, HOCM)
 Disproportionate left ventricular hypertrophy, typically involving septum
more than free wall, with or without an intraventricular systolic pressure
gradient; usually of a nondilated left ventricular cavity
 inappropriate myocardial hypertrophy in the absence of HTN or aortic
stenosis
3. Restrictive (infiltrative)
 Endomyocardial scarring or myocardial infiltration resulting in restriction
to left and/or right ventricular filling and diastolic function

46. Cardiomyopathy in Africa
 Endomyocardial Fibrosis
 This is a progressive disease that presents as a RCM.
 Dilated Cardiomyopathy
 DCM is a common cause of heart failure in Africa
and has been reported in up to one-half of patients
hospitalized with CHF.