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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

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

25
HCM parhogenesis HCM
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
HCM gross morphology
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
HCM clinical features
``` Few or any symptoms in general Arrythmias Angina pectoris CCF Infective endocarditis Obstruction to aortic outflow Syncope ```
28
People at risk of HCM
Young adults
29
What drug can improve the left ventricular relaxation during diastole in HCM
B adrénergiques blockers | Calcium Channel blockers
30
Restrictive cardiomyopathy
Idiopathic diastolic disorder with reduced ventricular compliance , impaired ventricular filling , normal ventricular function
31
Least common cardiomyopathy
Restrictive cardiomyopathy
32
Restrictive cardiomyopathy characteristics
Gross endocardial thickening Abnormal rigidity of ventricles Progressive diastolic cardiac failure Normal contractility
33
2 conditions of restrictive cardiomyopathy
Endomyocardial fibrosis | Endocardial fibroelastosis
34
Endomyocardial fibrosis characteristics
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
Endomyocardial fibrosis morphology
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
Characteristics of right sided EMF
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
Microscopy of right sided EMF
Scarred areas of original endocardium Hyalinization of superficial layer Elastosis minimal or absent in fibrous layer Calcification of scars
38
Right sided EMF clinical feature
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
Endocardial fibroelastosis characteristics
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
Gross Morphology of endocardial fibroelastosis
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
Microscopic Morphology of endocardial fibroelastosis
Thickened endocardium Collagen and elastic fibers parallel to surface Fibrosis of myocardium
42
Clinical features of endocardial fibroelastosis
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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