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Clin Med I- CARDIO > Cardiomyopathies > Flashcards

Cardiomyopathies Flashcards

1
Q

Types of cardiomyopathy

A

Dilated, hypertrophic, and restrictive cardiomyopathy

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2
Q

Most common type of cardiomyopathy

A

DCM

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3
Q

What is DCM characterized by?

A

Ventricular contraction is reduced (contractile dysfunction), left ventricle dilation. RV may also be dilated.

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4
Q

African American male, aged 40 years old presents with symptoms of low output HF including dyspnea upon exertion, orthopnea, S3 heart sound, peripheral edema. Also has conduction disturbance. Diagnosis?

A

DCM

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5
Q

Etiologies of DCM

A

Idiopathic, Ischemic, Stress-induced, infectious, genetic, LV non-compaction, peripartum, toxic causes, inflammatory diseases

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6
Q

Infectious causes of DCM

A

Viral, HIV, Chagas, and Lyme disease

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7
Q

Toxic causes of DCM

A

Alcohol, cocaine, meds, trace elements

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8
Q

Stress-induced DCM aka

A

Broken heart syndrome, Transient left ventricular apical ballooning, and Takotsubo Cardiomyopathy

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9
Q

What is stress induced DCM triggered by

A

Acute medical illness or intense emotional or physical stress

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10
Q

What is the most common cause of myocarditis?

A

Viral DCM

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11
Q

What organisms are associated with causing viral DCM?

A

Parovirus B19, human herpesvirus 6, influenza virus, adenovirus, echovirus, ctyamegalovirus, HIV, cosackieirus

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12
Q

What is the leading cause of DCM in Central and South America?

A

Chagas Disease

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13
Q

Chagas disease occurs due to..

A

protozoan infection due to Trypanosoma cruzi

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14
Q

Of Lyme Disease and Chagas disease, which one is more chronic vs. self limited and mild?

A

Chagas- chronic symptoms. Lyme- self limited and mild

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15
Q

What kind of abnormality is present in Lyme disease?

A

Usually manifests as a conduction abnormality in which the cardiac muscle is dysfunctional.

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16
Q

Mode of inheritance in DCM

A

Autosomal dominant usually. Autosomal recessive, X-linked, mitochondrial inheritance have also been described.

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17
Q

Autoimmune cause of DCM in…..

A

30% of patients

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18
Q

Left-ventricular Non-compaction aka

A

Isolated Ventricular Non-Compaction, Left Ventricular Hypertrabeculation, Spongy Myocardium

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19
Q

How might alcohol cause DCM?

A

Alcohol causes oxygen free radical damage and defects in cardiac protein synthesis which is toxic to cardiac myocytes

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20
Q

What are the mechanisms by which cocaine might cause DCM?

A

Direct toxic effect, cocaine-induced hyperadrenergic state, and parenteral cocaine abusers- might cause infection

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21
Q

How might trace elements cause DCM?

A

Accumulation of cobalt, arsenic or deficiency of selenium

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22
Q

Which DCM related etiology is associated with pregnancy?

A

Peripartum- occurs late pregnancy and early postpartum. 4 criteria- hx failure in last month of pregnancy or within 5 months of delivery, absence of other identifiable cause of HF, absence of recognizable heart disease prior to last, and LV systolic dysfunction less than 45% ejection fraction

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23
Q

How does Angiotensin II affect preload and afterload?

A

Causes aldosterone secretion which causes increased preload, and constriction of vascular smooth muscle which causes increased afterload

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24
Q

What is the gold standard for DCM?

A

Endomyocardial biopsy

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25
Q

Diagnosis of DCM

A

Elevated cardiac markers, serological tests for viral, parasitic etiologies, markers of inflammation, Echocardiography, endomyocardial biopsy, cardiac cathetirization-r/o ischemic disease, and nuclear and magnetic resonance imaging

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26
Q

Management of DCM

A

Education, Meds include ACE-Inhibitors, Aldosterone inhibitors, anticoagulation, BB, and Diuretic Therapy, and mechanical- implantable cardiac defibrillator or biventricular pacemaker

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27
Q

How often should first degree relatives of DCM patients be screened?

A

every 3-5 years

28
Q

Cardinal feature of HCM

A

Myocyte-myofibril disarray

29
Q

Hypertrophic cardiomyopathy aka

A

I-HAM. Idiopathic hypertrophic subaortic stenosis, Hypertrophic obstructive cardiomyopathy, Asymmetric septal hypertrophy, and Muscular subaortic stenosis

30
Q

Dyspnea, atrial fibrillation, and HTN. Patient presents with massive asymmetric hypertrophy, diastolic left ventricular dysfunction, small internal diamteter of left ventricle. S2 split due to aortic valve closing early, harsh crescendo systolic murmur. Aortic outflow obstruction and MR present. SAM also present. What do you suspect?

A

HCM

31
Q

Pattern of LVH in HCM

A

TYpe I- classic septal hypertrophy (Anterior segment of the ventricular septum). Type II- Hypertrophy of anterior and posterior segments of the septum. Type III- extensive thickening. Type IV- Isolated apical hypertrophy.

32
Q

HOCM

A

Hypertrophic Obstructive Cardiomyopathy- HCM with SAM- Hypertrophic Cardiomyopathy with systolic anterior motion of the mitral valve

33
Q

Three ages of presentation in this disorder- adolescents, like young athletes wtih syncope during athletic event No PE abnormalities. Also ages 40 and 60.

A

HCM

34
Q

DDX of hypertrophic Cardiomyopathy

A

Hypertensive heart disease- hypertrophy pattern is different, and valvular heart disease

35
Q

Clinical presentation of HCM

A

Dyspnea, effort-related chest discomfort, syncope, sudden cardiac death, atrial fibrillatin, hypertension

36
Q

Imaging study of choice for diagnosis of HCM

A

Echocardiography

37
Q

Diagnosis of HCM

A

Electrocardiography, Cardiac catheterization, echocardiography- LV hypertrophy, LA enlargement, mitral regurgitation, septal hypertrophy

38
Q

Types of HCM Tx

A

Avoidance of volume depletion, activity restriction, Meds, Mechanical and surgical

39
Q

Why do you want to avoid volume depletion in HCM?

A

Tends to decrease SV, and increases the left ventricular outflow gradient. May lead to hypotension, lightheadedness, or syncope

40
Q

Meds in Tx of HCM

A

BB (decreases chronotropy and inotropy), Calcium Antagonists (Improves diastolic filling, decreases chronotropy), Anti-arrythmic drugs

41
Q

Most commonly used CCB in HCM

A

Verapamil

42
Q

Most commonly used anti-arrythmic drug in HCM

A

Amiodarone

43
Q

Mechanical tx in HCM

A

Implantable Cardioverter Defibrillator Therapy to prevent sudden cardiac death

44
Q

Surgical tx of HCM

A

Alcohol ablation of the septum, myectomy, and heart transplant

45
Q

HCM may be caused by mutations such as-

A

encoding proteins of the cardiac sarcomere- MYH7 (Beta myosin heavy chain), MYBPC3 (cardiac myosin-binding protein C), TNNT2 (Cardiac troponin T)

46
Q

Least common CM outside of tropic area

A

Restrictive CM

47
Q

What are RCM characterized by?

A

Walls of ventricles become stiff, but not necessarily thickened. They are non-dilated, with impaired ventricular filling. Also, bi-atrial enlargement. Normal systolic function.

48
Q

RCM general classification

A

Secondary restrictive physiology, nonfiltrative, infiltrative, storage diseases, endomyocardial disease, radiation/drug toxicity

49
Q

Noninfiltrative causes of RCM

A

Idiopathic, familial, hypertrophic, and diabetic

50
Q

Infiltrative causes of RCM

A

Amyloidosis, Sarcoidosis, Fatty infiltration, Gaucher disease, and Hurler Syndrome

51
Q

Storage disease causes of RCM

A

Hemochromatosis, Fabry Disease, GLycogen Storage disease

52
Q

Endomyocardial disease causes of RCM

A

Endomyocardial fibrosis and hypereosinophilic syndrome, Churg-Strauss Syndrome, radiation, and toxic drugs

53
Q

What age group is more affected by Idiopathic RCM?

A

older adults

54
Q

Test of choice for amyloidosis RCM?

A

Echocardiography

55
Q

What toxic drugs may cause RCM?

A

Methysergin (Vascular headaches), Ergotamine (Migraine headaches)

56
Q

Diagnosis of RCM

A

Labs, CXR- enlarged RV, EKG- RV hypertrophy, atrial arrhythmias, echo- ventricular doppler filling patterns, cardiac catheterization

57
Q

Gold standard to diagnose RCM

A

Cardiac catheterization- R and L heart pressure during inspiration and endomyocardial biopsy

58
Q

Tx for RCM

A

To slow heart rate- BB
To control atrial fibrillation- antiarryhthmics, and anticoagulation
To improve diastolic relaxation- CCB, BB, ACE-I
Diuretics, spironolactone, control systemic BP, avoid digitalis
Cardiac Transplantation

59
Q

Patient presents with fatigue and weakness, pulmonary hypertension, dyspnea, peripheral edema, ascites, arrhythmias. Echo reveals that ventricles are not dilated, but the atria are enlarged. There is a diastolic filling problem in the ventricles due to the rigid walls, leading to decreased preload and EDV. What do you suspect?

A

RCM

60
Q

Ground glass appearance on echo would make you suspect what cause for Restrictive CM?

A

Amyloidosis RCM

61
Q

Patient comes in with dyspnea, atrial fibrillation, and HTN. He is a young athletic patient that had a syncopal episode. ECG shows you LV hypertrophy, echo shows you abnormal systolic anterior leaflet motion of the mitral valve which is causing obstruction of blood from the LV to aorta. So you have aortic outflow obstruction and also MR. You are suspicious of HCM. What maneuvers could you do in the office to help with your diagnosis?

A

Decrease volume in LV would cause increased obsturction–>increased murmur intensity. Decreased venous return to heart caused by upright position to sitting, squatting, supine position, valsalva, nitroglycerin or other vasodilator. To decrease intensity, increase volume in LV by going from standing to squatting, handgrip, after passive elevation of legs.

62
Q

Are ACE-I or diuretics indicated in HCM patients?

A

NO. AVOID volume depletion! Already have decreased volumes in LV due to hypertrophy. Stick to BB, CCB, and anti-arrhythmia drugs in tx of HTN

63
Q

HCM patient in ER who is at risk of sudden cardiac death most likely due to…

A

abnormal arrhythmai- often degenerate into VF (atrial arrhythmia, SVT, WPW syndrome). That’s why often treated wtih amiodarone (along with BB and CCB)

64
Q

Which cardiomyopathy cause diastolic vs. systolic problem?

A

HCM and RCM- diastolic filling problem. DCM- systolic contraction problem

65
Q

Which sides of the heart are most effected in CM?

A

DCM and HCM- left side of heart. RCM- right side more affected

Clin Med I- CARDIO (10 decks)

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