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Cardiovascular Module > Cardiomyopathy > Flashcards

Cardiomyopathy Flashcards

1
Q

Cardiomyopathy definition

A

Disease of heart muscle of unknown cause

Heterogenous group of chronic myocardial dysfunction which results in cardiac failure

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2
Q

How do you diagnose a Cardiomyopathy

A

By excluding identifiable causes of myocardial dysfunction

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3
Q

Three subtypes of cardio myopathy

A

Dilated
hypertrophic
restrictive/constrictive

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4
Q

Second name of dilated cardiomyopathy

A

Congestive cardiomyopathy

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5
Q

Characteristics of dilated cardiomyopathy

A

Poor systolic function
considerable biventricular dilatation
Increased mass
Chronic progressive cardiac failure

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6
Q

Most common type of cardiomyopathy

A

Dilated cardiomyopathy(90%)

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7
Q

Associations to dilated cardiomyopathy

A

Chronic alcoholism (10-20%)
pregnancy
post viral myocarditis

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8
Q

Most common association to dilated cardiomyopathy

A

Alcoholism

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9
Q

In which trimester of pregnancy can you find cardiac failure

A

3rd

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10
Q

Risk factor associated to pregnancy cardiomyopathy

A 
Multiparous
Eclampsia
Twin gestation
Maternal age more than 30 years 
black race 
postpartum hypertension
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11
Q

Prognosis of pregnancy cardiomyopathy

A

50% spontaneous recovery

50% persistent left ventricular dysfunction or progressed to chronic cardiac failure and death

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12
Q

Dilated cardiomyopathy pathogenesis

A

Reduced left ventricular systole ejection fraction (less than 40%)

Left ventricle global hypokinesis, regional wall motion abnormalities

Cardiac dilatation

Atrioventricular valve incompetence due to ring dilatation

Low cardiac output

Promotion of inracardiac mural thrombi and systemic or pulmonary thromboembolism

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13
Q

Alcoholic cardiomyopathy cause of toxicity

A

Acetaldehyde which impairs calcium homeostasis, inhibits mitochondrial respiration, reduces protein synthesis

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14
Q

Dilated cardiomyopathy gross morphology

A 
Rounded , globular heart 
Dilatation of chambers 
Increase total heart weight 
Not all the time hypertrophy 
Mural thrombi
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15
Q

Arrythmogenic right ventricular dysplasia morphology

A

Thinned wall

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16
Q

Histology of dilated cardiomyopathy

A

Interstitial fibrosis
Subendocardial dense scarring in long standing case
Myocytes hypertrophy, atrophy or degeneration

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17
Q

Arryhtmogenic right Histology

A

Fatty infiltration
Loss of myocytes
Interstitial fibrosis

18
Q

Arryhtmogenic right ventricle dysplasia clinical presentation

A 
Dysphonies 
Fatigue
Exercise intolerance 
Life threatening arrythmias 
Severe acute cardiac failure 
Chest pain 
Systemic or pulmonary embolism 
Rarely sudden death
19
Q

What drug can exacerbate arryhtmkgenjc right ventricle dysplasia to heart failure

A

Digoxin

20
Q

Differential diagnosis of DCM

A 
Chronic IHD
myocarditis 
Haemochromatosis
Kwashiorkor 
Severe anemia 
Hyper/ hypo thyroïdism 
Heavy metal poisoning 
Adriamycin toxicity
21
Q

Hypertrophic cardiomyopathy definition

A

Diastolic disorder
Idiopathic cardiac hypertrophy
Restriction to ventricle filling

22
Q

Hypertrophic cardiomyopathy distinctive features

A

Idiopathic hypertrophic subaortic stenosis

Hypertrophic obstructive cardiomyopathy

Muscular subaortic stenosis

23
Q

Etiology of hypertrophic cardiomyopathy

A

Gene mutations encoding sarcomeric proteins in contractile units - Chr 14

Troponin T and myosin binding protein C ( MYBP-c)

Sometimes a-tropomyosin

24
Q

Parhogenesis of HCM

A

Unknown

25
Q

HCM parhogenesis HCM

A

Decreased ventricular compliance
Pronounced hypertrophy of LV
Systolic anterior motion of anterior mitral valve
Myofibre disarray

Cause
increase resistance to ventricular filling
Left atrial hypertrophy
Reduced stroke volume

Increased pulmonary venous pressure ->Exertion dyspnoea

Abnormal contraction over coronary artery may cause myocardial ischemia and frank infarction

26
Q

HCM gross morphology

A

Massive left ventricular hypertrophy
External usually normal
Left ventricular cavity reduced
Septal thickness greater than posterior free wall (S/P ratio over 1.6 at autopsy good index for diagnosis)

Patch of endocardium thickening
Mirror image of anterior mitral leaflet

Thickened valve leaflets
Chronic mitral incompetence-> Dilated left atrium
Poor left ventricular relaxation
Mural thrombus in mostly left atrium

Myocardial fibers in random directions and intertwine about small vessels

Hypertrophied myocytes

27
Q

HCM clinical features

A 
Few or any symptoms in general 
Arrythmias 
Angina pectoris 
CCF 
Infective endocarditis 
Obstruction to aortic outflow 
Syncope
28
Q

People at risk of HCM

A

Young adults

29
Q

What drug can improve the left ventricular relaxation during diastole in HCM

A

B adrénergiques blockers

Calcium Channel blockers

30
Q

Restrictive cardiomyopathy

A

Idiopathic diastolic disorder with reduced ventricular compliance , impaired ventricular filling , normal ventricular function

31
Q

Least common cardiomyopathy

A

Restrictive cardiomyopathy

32
Q

Restrictive cardiomyopathy characteristics

A

Gross endocardial thickening
Abnormal rigidity of ventricles
Progressive diastolic cardiac failure
Normal contractility

33
Q

2 conditions of restrictive cardiomyopathy

A

Endomyocardial fibrosis

Endocardial fibroelastosis

34
Q

Endomyocardial fibrosis characteristics

A

Severe scarring of one or both ventricles

Endemic in tropical Africa
Non endemic in tropical South America

Thought to be caused by hypereosinophilia

Endomyocardial necrosis with eosinophil infiltrate
Scarring of necrotic foci
Layering of endocardium by thrombus

35
Q

Endomyocardial fibrosis morphology

A

Scarring of endocardium and subendocardium mostly in apices and inflow tracts

Scarring of papillary muscles and chordae tendinae
Shortening with distortion of valves
Mitral / tricuspid incompetence 
Fused chordae 
Mural thrombus 
Obliterated apex by thrombus

Pericardial effusion

36
Q

Characteristics of right sided EMF

A

External malformation of the heart

Right atrium is dilated and hypertrophied

Marked sulcus between apices of ventricles

Prominent trabeculation of atrium

Patchy endocardial sclerosis

2/3 of right ventricle cavity obliterated with fusion of opposing walls

37
Q

Microscopy of right sided EMF

A

Scarred areas of original endocardium

Hyalinization of superficial layer

Elastosis minimal or absent in fibrous layer

Calcification of scars

38
Q

Right sided EMF clinical feature

A

Disease of children and young adults

Congestive cardiac failure

Mitral or tricuspid incompetence

Chronic venous congestion of liver

Ascites

Absence of peripheral edema

High venous pressure

Stunting of growth

Finger clubbing

39
Q

Endocardial fibroelastosis characteristics

A

Obscure origin
Infants and children affected

Interference with proper contraction and relaxation by thickened endocardium
Decreased diastolic filling
Cardiac failure

Possible causes :
Intrauterine endocardial anoxia
Viral infection

40
Q

Gross Morphology of endocardial fibroelastosis

A

Diffuse Thickening of mural endocardium By Dense white avascular tissue with elastic fibers (LV or LA)

Trabecular pattern of papillary muscles and chordae tendinae

Thickening of mitral and aortic valve cusps

41
Q

Microscopic Morphology of endocardial fibroelastosis

A

Thickened endocardium

Collagen and elastic fibers parallel to surface

Fibrosis of myocardium

42
Q

Clinical features of endocardial fibroelastosis

A

Sudden or gradual onset of cardiac failure

Death within minutes or hours of birth
Or
Heart failure weeks or months later

Adult form with chronic CCF , embolic phenomena

Cardiovascular Module (21 decks)

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