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2 - Cardiovascular Medicine > Cardiomyopathies > Flashcards

Cardiomyopathies Flashcards

1
Q

Definition cardiomyopathy

A

Heart muscle disease of unknown cause in which the heart muscle is structurally/ functionally abnormal leading to mechanical/ electrical dysfunction resulting in dilated, hypertrophic pathophysiology of heart

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2
Q

Classification of cardiomyopathies

A

1- dilated cardiomyopathy
2- hypertrophic cardiomyopathy
3- restrictive cardiomyopathy
4- Arrythmogenic right ventricular cardiomyopathy
5- unclassified cardiomyopathy

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3
Q

Categories of cardiomyopathy

A

Primary involving only the heart

Secondary involving other organs

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4
Q

What is the moges classification of cardiomyopathies

A

M- morpho functional phenotype
O- involved organs
G - Genetic
E- etiology
S- functional status

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5
Q

4 major types of primary cardiomyopathy

A

HCM
DCM
ARVD
RCM

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6
Q

cardiomyopathies with genetics as primary cause

A

HCM
ARVC
LVNC

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7
Q

cardiomyopathies with Mixed etiologies

A

DCM
RCM

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8
Q

Steps to diagnose cardiomyopathies

A

History
Examination
Echocardiographic evaluation (2Decho, Doppler echo)
MRI and CT scan In some cases

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9
Q

Cases when to further investigate wirh mri and Ct scan

A

Local fat
Iron
Amyloid infiltration
Scar
Fibrosis
Focal hypertrophy
Iv apical aneurysm
Fv structure / function

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10
Q

What characterizes DCM

A

Dilatation and impaired contraction of one or both ventricles

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11
Q

Pathophysiology of DCM

A

Dilated ventricles with impaired contraction
Increased ventricular mass with normal or reduced wall thickness
Can lead to valve incompetence

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12
Q

This modifiable risk factor is involved in many cases of dilated cardiomyopathy

A

Alcohol

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13
Q

25% cases of DCM are inherited as autosomal dominant trait, true or false

A

True

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14
Q

Common causes of DCM i

A

Idiopathic
Infections
Drugs
Alcohol
Inflammatory dx
Pregnancy
Poor nutrition
Hereditary / genetic
Toxic substances
Autoimmune disorders

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15
Q

Main presentation of DCM

A

Heart failure
Reduced ejection fraction
Arrhythmia
Thromboembolism
Sudden death
Sporadic chest pain

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16
Q

Main differential of DCM

A

Ventricular dysfunction due to CAD

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17
Q

Most useful investigations test in DCM

A

Cardiac MRI
Echocardiogram

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18
Q

Pharmacotherapy in DCM

A

RAAS blockers ( ACEIs, ARBs, ARNIs)
Bblockers
Mineralocorticoid receptor antagonists
SGLT2 inhibitors
Loop diuretics
Vasodilators

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19
Q

Devices and surgeries done in DCM

A

ICD - implantable cardioverter defribillator

CRT- cardiac resynchronization therapy
VAD- ventricular assist device
Heart transplant

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20
Q

What is peripartum cardiomyopathy PPCM

A

Pregnancy associated cardiomyopathy occurring in late pregnancy or early puerperium which presents with heart failure towards end of pregnancy or within 5 months after delivery

21
Q

Ejection fraction in peripartum cardiomyopathy

A

Less than 45 %

22
Q

Risk factors in peripartum cardiomyopathy

A

More than 30 yo
African descent
Pregnancy with multiple fetuses
Preeclampsia, eclampsia , postpartum hypertension history
Long term oral tocolytic more than 4 weeks with beta agonists
Maternal cocaine abuse
Selenium deficiency

23
Q

What is hypertrophic cardiomyopathy

A

Increase in LV wall thickness not explained solely by abnormal loading conditions

24
Q

Form of inheritance in hypertrophic cardiomyopathy

A

Autosomal dominant trait with variable expression

25
Q

This cardiomyopathy is the leading cause of sudden death in pre adolescent and adolescent children

A

Hypertrophic cardiomyopathy

26
Q

Hallmark of hypertrophic cardiomyopathy

A

Inappropriate , asymmetric myocardial hypertrophy
Abscess of obvious inciting stimulus

27
Q

Main area of the heart affected by hypertrophic cardiomyopathy

A

Interventricular septum with LVOT obstruction

28
Q

Thickness of wall in hypertrophic cardiomyopathy

A

More than 15mm

29
Q

2 types of hypertrophic cardiomyopathy

A

Obstructive (mid systolic obstruction of flow)
Non obstructive

30
Q

Symptoms of HCM

A

Asymptomatic
Sudden cardiac death
Dyspnea
Syncope
Presyncope
Chest pain
Palpitations
Fatigue

31
Q

Signs of HCM

A

Double carotid arterial pulse
Double apical impulse
S4 possibly heard
Systolic ejection crescendo decrescendo murmur due to LVOT obstruction
Holosystolic murmur at apex and axilla due to mitral regurgitation
Diastolic decrescendo murmur of AR

32
Q

Complication of HCM

A

Heart failure
Ventricular arrhythmia
Infective endocarditis of mitral valve
Atrial fibrillation with mural thrombus formation
Sudden death

33
Q

Investigation of HCM

A

2D echo
Chest MRI in selected cases
CxR
Cardiac catheterization
Electrophysiologic studies
Genetic testing

34
Q

Management of HCM

A

BBlockers
NDHP CCBs (verapamil, diltiazem )
Disopyramide
Amiodarone
Antitussives
Diuretic

35
Q

Management to avoid in HCM

A

Inotropic drugs
Nitrates
Vasodilators
Digitalis

36
Q

Surgical options in HCM

A

Surgical myectomy
Catheter alcohol septal ablation
Mitral valve replacement
ICD placement
Heart transplant

37
Q

What is Restrictive cardiomyopathy

A

Non compliant ventricular walls that resist diastolic filling

38
Q

Part of the heart most commonly affected with RCM

A

Left ventricle

39
Q

RCM types

A

Obliterative due to fibrosis of endocardium and subendocardium

Non obliterative due to MI by abnormal substance such as amyloid

40
Q

Possible etiology of RCM

A

Genetic or acquired
Systemic disease (amyloidosis, hemochromatosis)
Sarcoidosis
Fabry disease
Loiffler syndrome
Genetic abnormalities (fabry dx, gaucher dx, hemochromatosis )
Connective tissue dx (amyloidosis , systemic sclerosis, Endocardial fibroelastosis )
Carcinoid tumors
Endomyocardial fibrosis
Hypereosinophilic syndrome
Sarcoidosis

41
Q

Différent possible consequence of RCM

A

Endocardial thickening or myocardial/papillary infiltration with or without myocyte death

Hypertrophy and fibrosis in one or both ventricles

Mitral or tricuspid malfunction -> regurgitation

SA and AV nodes malfunction -> SA and AV block

Main hemodynamic consequence -> Diastolic dysfunction with rigid non compliant ventricle and impaired diastolic filling -> pulmonary venous hypertension

Mural thrombi possible -> systemic emboli

42
Q

Investigations done in RCM

A

Echocardiogram
MRI
Cardiac CT scan
ECG
Chest X ray
L and R heart catheterization
Cardiac biopsy
Lab test
Biopsy

43
Q

What do you see on echo of RCM

A

Both atria Dilated
LVEF may be normal
Elevated filling pressure on Doppler
Myocardial hypertrophy

44
Q

Findings on echo of amyloidosis with RCM

A

Bright echo pattern from myocardium

45
Q

Treatment of RCM

A

Diuretics
Bblockers
Anticoagulation
Avoid nitrates

46
Q

People at risk of Endomyocardial fibrosis

A

Children and adolescents in tropics

47
Q

Age at risk of Endomyocardial fibrosis

A

10 and 30 yo

48
Q

Clinical manifestation of Endomyocardial fibrosis

A

Right or left heart failure

2 - Cardiovascular Medicine (16 decks)

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