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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
This cardiomyopathy is the leading cause of sudden death in pre adolescent and adolescent children
Hypertrophic cardiomyopathy
26
Hallmark of hypertrophic cardiomyopathy
Inappropriate , asymmetric myocardial hypertrophy Abscess of obvious inciting stimulus
27
Main area of the heart affected by hypertrophic cardiomyopathy
Interventricular septum with LVOT obstruction
28
Thickness of wall in hypertrophic cardiomyopathy
More than 15mm
29
2 types of hypertrophic cardiomyopathy
Obstructive (mid systolic obstruction of flow) Non obstructive
30
Symptoms of HCM
Asymptomatic Sudden cardiac death Dyspnea Syncope Presyncope Chest pain Palpitations Fatigue
31
Signs of HCM
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
Complication of HCM
Heart failure Ventricular arrhythmia Infective endocarditis of mitral valve Atrial fibrillation with mural thrombus formation Sudden death
33
Investigation of HCM
2D echo Chest MRI in selected cases CxR Cardiac catheterization Electrophysiologic studies Genetic testing
34
Management of HCM
BBlockers NDHP CCBs (verapamil, diltiazem ) Disopyramide Amiodarone Antitussives Diuretic
35
Management to avoid in HCM
Inotropic drugs Nitrates Vasodilators Digitalis
36
Surgical options in HCM
Surgical myectomy Catheter alcohol septal ablation Mitral valve replacement ICD placement Heart transplant
37
What is Restrictive cardiomyopathy
Non compliant ventricular walls that resist diastolic filling
38
Part of the heart most commonly affected with RCM
Left ventricle
39
RCM types
Obliterative due to fibrosis of endocardium and subendocardium Non obliterative due to MI by abnormal substance such as amyloid
40
Possible etiology of RCM
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
Différent possible consequence of RCM
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
Investigations done in RCM
Echocardiogram MRI Cardiac CT scan ECG Chest X ray L and R heart catheterization Cardiac biopsy Lab test Biopsy
43
What do you see on echo of RCM
Both atria Dilated LVEF may be normal Elevated filling pressure on Doppler Myocardial hypertrophy
44
Findings on echo of amyloidosis with RCM
Bright echo pattern from myocardium
45
Treatment of RCM
Diuretics Bblockers Anticoagulation Avoid nitrates
46
People at risk of Endomyocardial fibrosis
Children and adolescents in tropics
47
Age at risk of Endomyocardial fibrosis
10 and 30 yo
48
Clinical manifestation of Endomyocardial fibrosis
Right or left heart failure

2 - Cardiovascular Medicine (16 decks)

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