The American Heart Association (AHA) expert consensus panel proposed definition of cardiomyopathies is as follows: “Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction, which usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation, due to a variety of etiologies that frequently are genetic. Cardiomyopathies are either confined to the heart or are part of generalized systemic disorders, and often lead to cardiovascular death or progressive heart failure–related disability.”

1. Cardiomyopathy
• Dr Ramachandra Barik

2. WHO,1980
Heart muscle diseases of unknown cause
But not from cardiac dysfunction due to known
cardiovascular entities
Hypertension
Ischemic heart disease
Valvular disease

3. WHO/International Society and Federation of Cardiology (ISFC) Task Force,1995
1. Dilated
• Enlarged
• Systolic dysfunction
2. Hypertrophic
• Thickened
• Diastolic dysfunction
3. Restrictive
• Diastolic dysfunction
4. Arrhythmogenic RV dysplasia
• Fibrofatty replacement
5. Unclassified
• Fibroelastosis
• LV noncompaction
Anatomy & physiology

4. European Working Group definition,2008

5. American Heart Association
• Featured by
• Mechanical dysfunction
• Electrical dysfunction
• Ventricular hypertrophy
• Ventricular dilation
• Mostly genetic
• Primary (In situ)
• Secondary (A part of systemic
disease)
EXCLUDED
• Ischemic cardiomyopathy
• Valvular disease
• Congenital heart disease
• Hypertension heart disease

6. American Heart Association

7. CMP but normal with looking heart

8. Pathophysiology
Mutation:Pathological and polymorphic Exogenous insult:Viral,toxins,alcohol
Contraction and relaxation disorder
Ineffective energy utilization
Altered Ca ions handling
Activation of compensatory
neurohumoral mechanisms
Apoptosis
Fibrosis
Hypertrophy
Heart failure:Systolic ,diastolic,both Arrhythmia, sudden death Thromboembolic complication

11. PATHOPHYSIOLOGY
DISEASED MYOCARDIUM CHANNELOPATHY

13. OUTFLOW OBSTRUCTION

14. Natural history

15. Risk of Sudden Death
(LVEF >35%)
Maggioni AP. GISSI-2 Trial Circulation. 1993;87:312-322.
EF<35%
(LVEF < 35%)
NO PVC
SOME PVC
MANY VPCo PVBs
1-10 PVBs/h
> 10 PVBs/h
0.86
A
0.88
0.90
0.92
0.94
0.96
0.98
1.00
0 30 60 90 120 150 180
Days
Survival p log-rank 0.002
0.88
0.90
0.92
0.94
0.96
0.98
1.00
0 30 60 90 120 150 180
Days
Survival
B
p log-rank
0.0001
0.86

16. Complications
• Heart failure
• Blood clots
• Valve problems
• Cardiac arrest /Sudden death:VT/VF

17. Symptoms
• SYSTOLIC HEART FAILURE
• DIASTOLIC HEART FAILURE
• OUTFLOW TRACT
OBSTRUCTION
• ARRYTHMIA
• SYSTEMIC FEAURES
• SYNDROMIC ASSOCIATION

18. Tests
• Chest X-ray
• Echocardiogram
• Electrocardiogram (ECG)
• Treadmill stress test
• Cardiac catheterization
• Cardiac magnetic resonance
imaging (MRI)
• Cardiac computerized
tomography (CT) scan
• Blood tests.
• ELECTROPHYSIOLOGY
• Myocardial or endocardial
biopsy
• Genetic testing or screening

19. TREATMENT
• Exogenous insult prevention
• Sudden death prevention
• Primary=ICD
• Secondary:ICD
• Heart failure therapy
• Symptomatic
• Life prolonging=MYECTOMY AND HEART TRANSPLANTATION,CRT
• Thromboembolic complications
• Gene therapy:GAUCHER’S AND FABRY’S DISEASE

20. SYMPTOMATIC :MM=Frank-Starling curve
• B=diuretic or venodilator
• B”=excess diuretic or venodilator
• C=inotrope
• D=arterial dilator
• E=Inotrope and vasodilator

22. Implantable Cardioverter Defibrillator

23. Cardiac resynchronization therapy

24. THE DILATED CARDIOMYOPATHIES
• Dilated left ventricle
• systolic dysfunction
• that is not caused by ischemic or
valvular heart disease
• The most common
cardiomyopathy

25. NATURAL HISTORY

26. Hypertrophic cardiomyopathy
1. Most common of the genetic
cardiovascular diseases
2. Compatible with normal
longevity in many patients
3. Most common cause of
sudden death in the young
4. 1 in 500 people

27. Wall thickness of 13-15 mm in males and 11-12
mm in females

30. PATHOPHYSIOLOGY

31. TYPES

33. RISK FACTOR FOR SCD

36. RESTRICTIVE CARDIOMYOPATHY
• INVRTED PEAR SIGN

37. INFILTRATION OF INTERSTITIUM

38. Cardiac Amyloidosis Based on Amyloid

39. SARCOID CARDIOMYOPATHY
• Hematoxylin-eosin staining:
Noncaseating granuloma typical
of sarcoid. The arrow points to
an “asteroid body” in the
cytoplasm of the giant cell

40. Peripartum cardiomyopathy
• LV systolic dysfunction
• Towards the end of pregnancy(last month) or in the
months(puriperium) following delivery
• When no other cause of heart failure is found
• The LV may not be dilated but the ejection fraction is nearly always
below 45%

41. PPCMP

43. Broken Heart

44. Arrythmogenic right ventricular dysplasia

48. Revised Task Force Criteria for the Diagnosis
of ARVD

50. Left Ventricular Noncompaction

51. Left Ventricular Noncompaction

52. Carcinoid Heart Disease
• a systemic disorder mediated by
elevated circulating levels of
vasoactive substances, including
serotonin (5-hydroxytryptamine
[5-HT]), 5-hydroxytryptophan,
histamine, bradykinin,
tachykinins, and prostaglandins
produced by a rare metastatic
neuroendocrine malignancy,
carcinoid
•
• Carcinoid syndrome is
characterized by a triad of
symptoms—flushing, diarrhea,
and bronchospasm—that occur
in association with hepatic
metastases

53. • The characteristic pathologic features of carcinoid heart disease are
right-sided valve thickening and retraction resulting from
myofibroblast proliferation along with deposition of collagen, smooth
muscle cells, and elastic tissue. Tricuspid annular and subvalvar
involvement and pulmonary root constriction also occur, thereby
adding to the valvular dysfunction. Very rarely the heart is involved
directly by carcinoid metastases

54. • Untreated, patients with carcinoid syndrome have a median survival
of 3 to 4 years, and the presence of carcinoid heart disease shortens
this to less than 1 year. Therapy is not generally curative and includes
debulking the hepatic metastases by embolization or partial hepatic
resection and by the use of octreotide, a somatostatin analogue that
binds to somatostatin receptors on the surface of carcinoid tumor
cells and inhibits the secretion of vasoactive substances

55. Endomyocardial Disease
• CARCINOID HD
• LOEFFLLER’S CMP
• ENDOCARDIAL FIBROSIS

56. • Elevation of urinary 5-HIAA levels is highly specific and moderately
sensitive for the diagnosis of carcinoid syndrome, and the
echocardiographic and cardiac MRI features of thickened immobile
tricuspid and pulmonary valves with combined stenosis and
regurgitant lesions are highly suggestive of carcinoid heart disease

57. Löffler (Eosinophilic) Endocarditis
• Hypereosinophilia has been defined as either a chronic absolute
eosinophil count higher than 1500 cells/mL for at least 1 month,
although hypereosinophilia persisting for 6 months or longer is
common, or pathologic evidence of hypereosinophilic tissue invasion
• Three stages:
• acute: Myocarditis
• Intermediate: Valvular and thrombus formation
• Fibrotic :RCM

58. Endomyocardial Fibrosis
• Fibrosis of the LV and RV apical endocardium
• Presents as RCM
• The south Asian subcontinent
• More males affected than females (23% versus 17%)
• Family clustering
• IDIOPATHIC /hypereosinophilic syndrome
• A bimodal peak in age :First decade and second to fourth decades

59. • Heart failure symptoms from left or right restrictive physiology
• Dyspnea on exertion
• Paroxysmal nocturnal dyspnea
• Edema/ Ascites
• Imaging
• apical fibrosis / atrial enlargement and MV and TV SVF
• TREAMENT
• surgical resection of the endocardial fibrosis with valve repair or replacement
can have a dramatic effect on symptoms and survival
• significant risk for morbidity and mortality.

60. Structurally normal heart

61. LONG QT
• Delayed repolarization of the
myocardium
• QT prolongation :QTc >480 msec
• syncope, seizures, and SCD
• Structurally normal heart

62. BRUGADA SYNDROME
BrS is an heritable arrhythmia syndrome characterized by an ECG
pattern consisting of coved-type ST-segment elevation (≥2 mm)
followed by a negative T wave in the right precordial leads V 1 through
V 3 (often referred to as a type 1 Brugada ECG pattern) and increased
risk for sudden death resulting from episodes of polymorphic
ventricular tachyarrhythmias

63. LOSS OF SCN5A CHANNEL FUNCTION

64. Catecholaminergic Polymorphic Ventricular
Tachycardia
• Gain-of-function mutations
in RyR2lead to leaky calcium
release channels, which results
in excessive release of calcium,
particularly during sympathetic
stimulation, that can precipitate
calcium overload, delayed
depolarizations, and ventricular
arrhythmias

65. Bidirectional ventricular tachycardia