only for studing purposes

1. Cardiomyopathies
GEEWAN KAMAL UDAWATTAGE
GROUP 21

2. Special group of myocardial dysfunctions in
which their defect primarily within the
myocardium.
which is not due to,
Increased BP
Coronary artery disease
Valvular heart disease
Congenital heart disease
Inflammation

3. Cardiomyopathies
1. Dilated
cardiomyopathy/congestive
cardiomyopathy
2. Hypertropied
cardiomyopathy/asymmetric
hypertropied
cardiomyopathy/hypertropi
ed obstructive
cardiomyopathy
3. Restricted cardiomyopathy
4. Arrhythmogenic right
ventricular cardiomyopathy
5. Obliterative cardiomyopathy

4. Dilated Cardiomyopathy
• Most common type of CM up to 90% of cases.
• Systolic failure
• About 30% cases are genetic defects
• Progressive cardiac dilation with impaired contractility and
hypokinetic myocardium.
• Due dilated ventricles fibrous capsules of mitral and tricuspid
valves also dilate this cause functional mitral regurgitation or
functional tricuspid valve regurgitaion
• Due to dilation of ventricles and volume overload thrombus
may be formed in LV or RV
• One of the causes of congestive cardiac failure.

5. • Due to backward
failure of LV
1. Cough
2. Dsypnea
3. Parosysmal
nocturnal dspnea
4. Orthopnea
5. Hemoptysis
• Due to backward
failure of RV
1. Hepatomegaly
2. Raised JVP
3. Generalized
edema

6. Causes
• Alcohol toxicity
• Beri beri(thiamin deficiency)
• Coxsacki virus(myocyte death and fibrosis)
• Cocaine
• Chagas disease
• Haemochromatosis.
• Pregnancy(peripartum or after few weeks of
delivery-unexpalined)
• Lot is idiopathic

7. Latest discoveries
• Dystropin ,a transmembrane protein of cardiac muscle
stablizes the sarcomeres from attaching the sarcomeres
with extracellular basement membrane so these
molecules are mutated (specially in idiopathic cases).
• Lapse law

8. • Functional defects in force generating
mechanisms(mitochondrial enzymes)
• Enzymes concerning with beta oxidation of fatty
acids are defective
This leads to hypocontractile and hypokinetic
myocardium

9. Diagnosis
• Since dilated venricles are overfilled,rapid ventricullar filling causes a S3 sound
CXR -enlarged cardiac silhouette,
• vascular redistribution interstitial edema,
• pleural effusions
EKG –normal
• tachycardia, atrial and ventricular
• enlargement, LBBB, RBBB, Q-waves
Echocardiography
• LV size, wall thickness function
Cardiac Catheterization
• hemodynamics
• LV ejection fraction
• angiography
Endomyocardial Biopsy

10. Complications
• Thromboembolism
• Arrythmias
• Functional regurgitation

11. Treatment
• Mainly to reduce preload
• Angiotensin-converting enzyme (ACE) inhibitors
• Angiotensin II receptor blockers (ARBs)
• Beta-blockers
• Aldosterone antagonists
• Cardiac glycosides
• Diuretics
• Vasodilators
• Antiarrhythmics
• Human B-type natriuretic peptide
• Inotropic agents

12. Hypertopied Cardiomyopathy
• Most common cause of
sudden cardiac death of
young atheletics during
vigorous exercise.
• Hypertropy of upper
part of interventricular
septum and cavity
becomes banana
shaped.
ventricular septal
thickness is 1.3 times
the free wall thickness

13. 3 types
1. Hypertropic obstructive
2. Hypertropic
nonobstructive
3. Hypertropic obliterative
apical

14. Causes
• Mutations in heavy chain beta
myosins,actins,troponins and tropomyosin causes
defects in contractions.
• So these myocardial cells with abnormal sarcomeres
produce growth factors and leads to overgrowth of
myocardial cells

15. Histologically,
• Disarray of bundles of
myocytes
• Disarray of individual
myocytes
• Disarray of sarcomere
arrangement within
myocytes
• Interstial fibrosis can
be seen

16. • Anterior leaflet of mitral valve and hypertopied septal
wall rub against each other and become fibrotic and
thickened so this produces a jerky pulse.

17. These patients develop
1. Anginal pain.
2. Trachyarrythmias.
3. Due to diastolic failure congestive pulmonery
disease so exertional dsypnea.
4. Hash systolic murmer.

18. How to diffenciate the murmer between aortic
stenosis and hypertopied cardiomyopathy??

19. HCM AORTIC STENOSIS
•Hash systolic murmer
•Dynamic obstruction
•When squatting or lying,
•EDV increase so ventricles dilates so gap
between anterior leaflet and wall of
hypertopied septum rises so the murmer will
decrease
•When standing or sitting,
EDV decrease so the gap increase and the
murmer increase
•Hash systolic murmer
•Fixed obstruction
•When squatting or lying,
EDV increases so murmer will increase
•When standing or sitting,
EDV decreases so murmer decreases

20. Complications
• Fatal arrythmias
• Infective endocarditis
• Left atrial fibrillation(when atria becomes
hypertopied)
• S4 sound (when hypertopied atrium contracts
against a stiffened ventricle)
• Thrombosis
• Left ventricular failure

21. Diagnosis
Diagnosis can be done by,
• EKG
NSR
Septal q waves
LVH
• ECHOCARDOGRAPY
0
2
4
6
8
10
12
14
16
18
20
< 15 16-19 20-24 25-29 > 30
Maximun left ventricular wall thickness
(mm)
Incidence of sudden
death(per 1000per
yr)

22. Dagger like q waves

23. Management
• Beta blockers to increase EDV
to make heart hypokinetic.
• Surgical excision of
hypertropied myocardium
• By using a cathetor alcohol is
injected to hypertopied
septum to induce
infarction.so infarction heals
by fibrosis so it decreases in
thickness(latest treatment)

24. Restrictive cardiomyopathy
• Ventricular muscle is firm and not relaxed well
due to in filtration.
• Diastolic failure.
• Ventricular filling is restricted

25. Causes
a) Idiopathic
b) Post radiation fibrosis (radiation of mediastinal
tumours and lung tumours)
c) amyloidosis(modified transthyretine deposited
extracellularly stained by congo red)
d) Sarcoidosis(multisystemic granulomatous
disesase)
e) Glycogen storage diseases
f) metastasis

26. amyloidosis
sarcoidosis

27. Conditions associated with restrictive
cardiomyopathy
• Endomyocardial fibrosis
More common in african adults complicated by mural thrombus.
• Loefflers endomyocarditis.
Develops in eosinophllic leukemic patients when eosinophills release
their major basic protein into myocardium which causes inflammation
and fibrosis.in their blood degranulated eosinophylls can be
found.complicated by thrombus formation.one of the latest treatment
is endomyocardial stripping(removes endocardial fibrosis and mural
thrombi)
• Endocardial fibroelastosis
Problem of children 2-3 yr old.may be focal or diffuse fibro elastic tissue
deposited in myocardium or endocardium.thrombi may form in
LV.combined with other congenital diseases.

28. Endomyocardial
fibrosis

29. Treatment
• Diuretics
• For congestive symptoms, but  LV/RV filling
  CO
• Digoxin (avoid in amyloidosis)
• Antiarrhythmics for afib
• amiodorone
• Pacemaker for conduction system disease
• Anticoagulation for thrombus (esp in atrial
appendages)

30. How can you differenciate between restrictive
cardiomyopathy and constrictive pericarditis?
Pericardium undergone massive fibrosis and
shrinked.

31. • It can be only done by echocardiograpy and endomyocardial biopsy.

32. Prognosis
• Prognosis depends on etiology

33. References
• http://www.mayoclinic.org/diseases-
conditions/cardiomyopathy/basics/complications/con-20026819
• http://en.wikipedia.org/wiki/Cardiomyopathy
• http://www.nhlbi.nih.gov/health/health-topics/topics/cm/
• http://www.cardiomyopathy.org/
• http://www.heart.org/HEARTORG/Conditions/More/Cardiomyopathy/Symptoms-
and-Diagnosis-of-Cardiomyopathy_UCM_444175_Article.jsp

34. Thank you!!!