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Cardio > Cardiomyopathy - Exam 2 > Flashcards

Cardiomyopathy - Exam 2 Flashcards

1
Q

What is cardiomyopathy? What are the 5 different types?

A

Disorders characterized by morphologically and functionally abnormal myocardium in the absence of any other disease that is sufficient, by itself, to cause the observed phenotype.

Restrictive CM
Dilated CM
Hypertrophic CM
Arrhythmogenic RV CM and dysplasia
“Unclassified” Cardiomyopathies

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2
Q

What are the classic 3 types of cardiomyopathy?

A

Restrictive CM
Dilated CM
Hypertrophic CM

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3
Q

What is systolic dysfunction? What happens next? Eventually, what will develop?

A

decrease in myocardial contractility and reduction in LVEF

at the beginning the heart will compensate to maintain cardiac output but eventually the process fails and heart failure develops

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4
Q

What is the Frank-Starling relationship? When is it commonly seen?

A

Frank-Starling relationship (↑stretch = ↑contractility)

when the Left ventricle enlarges resulting in a higher stroke volume

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5
Q

What is diastolic dysfunction a result of?

A

cardiac dysfunction d/t abnormal LV relaxation and filling accompanied by elevated filling pressures

systolic dysfunction may also be present but it does NOT have to be present

diastolic dysfunction is often underestimated/missed

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6
Q

However, if systolic dysfunction comes first, _____ dysfunction follows

A

diastolic

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7
Q

What is myocarditis? What are 2 different causes? What does it result in?

A

An inflammatory, infiltrative process involving the myocardium

caused by infectious and noninfectious conditions

Results in necrosis and/or degeneration of myocytes
May lead to myocardial dysfunction and dilated cardiomyopathy

aka long term inflammation leads to scarring, which leads to necrosis

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8
Q

What is the MC pt population? **What is the MC cause in North America?

A

men aged 20-50

**viral: coxsackie B

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9
Q

What are the 3 common non-infectious causes of myocardititis? At what point do these patients need to be sent to cardiology?

A

medications, illicit drugs, and toxic substances

once LVEF drops below 40% or s/s of HF present

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10
Q

What are the 2 main mechanisms for myocarditis?

A

Host-mediated: direct cytotoxic effect of the causative agent

Autoimmune-mediated: secondary immune response

think mass inflammation

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11
Q

What are the 2 different phases of myocarditis?

A

acute and chronic

acute: first 2 weeks of disease

chronic: after 2 weeks

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12
Q

What is happening in the acute phase of myocarditis?

A

Myocyte death is a direct result of the causative agent, leading to cell-mediated cell toxicity

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13
Q

What is happening in the chronic phase of myocarditis?

A

A result of an inappropriate, overactive immune response

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14
Q

What are the top 3 infectious causes of myocarditis?

A

adenovirus -> COVID 19
coxsackie B virus
cytomegalovirus

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15
Q

What are the top 3 cardiotoxins that lead to myocarditis?

A

alcohol

anthracyclines (type of abx Daunorubicin, doxorubicin, and epirubicin)

cocaine

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16
Q

What is a typical myocarditis presentation?

A

Typically develops days to a few weeks after onset of acute febrile illness / respiratory infection if infectious cause with no known underlying cardiac pathology present

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17
Q

What are the classic symptoms of myocarditis?

A

SOB, pleural/pericardial chest pain that can be positional, +/- fever, chills

can happen gradual or abrupt with decreased CO, shock and severe LV systolic function

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18
Q

What will you hear on heart auscultation on a pt that has myocarditis?

A

pericardial friction rub, tachycardia, S3 or S4, murmur of mitral or tricuspid regur if ventricular dilation is severe

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19
Q

What 5 things would you want to order for a pt with suspected myocarditis?

A

EKG
cardiac biomarkers
labs
CXR
Echo: ALWAYS DONE!!!!

Bx: but not always done because it is VERY risky

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20
Q

When would you order a myocardial bx?

A

if the cause is unknown, other treatments have been tried and nothing is working AND it would change the outcome

should only be obtained if there is a high probability that results will change patient management

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21
Q

According to the AHA/ACC, when is a biopsy recommended?

A
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22
Q

What would a Cardiac MRI (CMR) tell you? Will it confirm the dx of myocarditis?

A

Cardiac MRI (CMR): helps assess extent of inflammation, myocyte necrosis and scarring, ventricular size / shape changes, wall motion abnormalities, and pericardial effusion

Can SUGGEST myocarditis, but sensitivity and specificity are limited and time-dependent

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23
Q

What is the tx for myocarditis? What is the ideal HR for these pts?

A

LVEF <40% → ACE-I, BB

Myopericardial Chest pain → NSAIDs (+/- colchicine)

HR less than 70

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24
Q

**______ is the MC type of cardiomyopathy. What is the MC type of pt? What is the mortality?

A

Dilated Cardiomyopathy

black pts

mortality is 50% at 5 years

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25
Q

What is dilated cardiomyopathy characterized by? What is their LVEF?

A

Characterized by dilation and impaired contraction of one or both ventricles, predominantly the LV

Defined by LVEF <40% without CAD or valvular disease

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26
Q

_____ is the #1 reason for heart transplant. What is the underlying cause?

A

dilated cardiomyopathy

cause is unknown

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27
Q

What is Chagas disease?

A

Caused by protozoan infection, Trypanosoma cruzi

Leading cause of DCM in Central and South America

28
Q

How does Lyme Disease manifest in cardiomyopathy?

A

Usually manifests as conduction abnormality

May cause myocardial dysfunction due to myocarditis

29
Q

dilated cardiomyopathy is genetic, how is it inherited?

A

Autosomal dominant predominantly

Inherited syndromes, such as muscular dystrophies, hemochromatosis, thalassemias

30
Q

How will dilated cardiomyopathy present?

A

s/s of right and left HF

rales, elevated JVP, S3 gallop, murmur of mitral or tricuspid regurg, peripheral edema, ascites

arrhythmias: pulsus alternans

31
Q

What diagnostics do you want to order if you suspect dilated cardiomyopathy? Which one is gold standard?

A

BNP or NT-proBNP

**Echocardiogram- gold standard

consider radionuclide ventriculography (MUGA) and cardiac MRI

32
Q

Why do you need to order a BNP or NT-proBNP? What does it tell you?

A

are necessary to determine prognosis and disease severity - done if SOB/DOE is present

BNP gets released when the body has too much fluid, so it correlates with how much fluid is in the body

33
Q

What is the tx for dilated cardiomyopathy?

A

treat underlying source

CHF managment

prevent sudden cardiac arrest

heart transplant

implant defibrillator if EF is less than 35%

34
Q

When do you need to implant a defibrillator?

A

when EF is less than 35%

defibrillator will shock the heart to keep it in a normal rhythm

35
Q

What is restrictive cardiomyopathy characterized by? What is it caused by? more likely to affect diastolic or systolic? atrial or ventricle?

A

nondilated ventricles with impaired filling

Caused fibrosis or infiltration of the ventricular wall

Causes diastolic dysfunction

lead to moderate to severe biatrial enlargement

aka crunchy and thick, atria are trying to push into a stiff ventricle

36
Q

What are some main etiologies of restrictive cardiomyopathy?

A

Infiltrative disorders: Amyloidosis, sarcoidosis, fatty infiltration

storage diseases: Hemochromatosis, Fabry disease

radiation, chemo, carcinoid heart disease, hypereosinophilic syndrome

37
Q

How will restrictive cardiomyopathy present?

A

RIGHT sided HF (diastolic, thinks backs up to the body)

JVD
Edema
Ascites
Hepatic enlargement

38
Q

How do you dx restrictive cardiomyopathy?

A

Echo or cardiac MRI will assist in diagnosis
Endomyocardial biopsy may be considered

39
Q

What is the tx for restrictive cardiomyopathy?

A

treat underlying cause if known

reduce pulm and systemic congestion with diuretics

40
Q

What are the 4 underlying processes that need to be controlled for heart failure/dilated/restrictive cardiomyopathy management? How do you control each process?

A
41
Q

What kind of pt is most likely to present with hypertrophic cardiomyopathy? Why is it a major problem?

A

young athlete that presents with cardiac arrest

thick wall blocks the aortic valve

42
Q

What is the underlying root cause of hypertrophic cardiomyopathy? What is it characterized by? What does it lead to?

A

Caused by mutations of sarcomere genes

Characterized by LV hypertrophy NOT caused by pathologic loading conditions like HTN or AS

Leads to LV outflow tract obstruction and impaired filling leading to decreased systemic blood flow!!

43
Q

How is Hypertrophic Cardiomyopathy inherited? What septum is more likely to be involved?

A

Autosomal-dominant trait with variable penetrance

Interventricular septum is commonly more prominently involved

44
Q

What are the 3 key symptoms for hypertrophic cardiomyopathy? ______ due to mimicked aortic stenosis

A

Syncope, Angina, Dyspne

Carotid pulsus bisferiens : a double pulse in the carotid artery that’s characterized by two peaks separated by a dip in the middle

45
Q

What murmur is associated with Hypertrophic Cardiomyopathy? Where do you need to listen to it?

A

Mid-systolic, harsh, louder with valsalva, quieter with squatting

3rd and 4th intercostals

46
Q

Why is the mid-systolic harsh murmur louder with valsalva and quieter with squatting?

A

louder with valsalva due to the decreased blood flow

quieter with squatting because the heart is stretching out and getting the larger septum out of the way

47
Q

What is noted on the EKG for hypertrophic cardiomyopathy? What is the diagnostic modality of choice? How thick does the LV wall need to be?

A

EKG may demonstrate LVH pattern

Echocardiogram is the diagnostic modality of choice

LV wall >1.5 cm thick

48
Q

**What is the management for hypertrophic cardiomyopathy?

A

Avoid volume depletion: LOTS of water

Avoid diuretics and vasodilators

**Activity restriction

**Beta-blockers or Verapamil (helps relax contractility)

Septal myectomy or alcohol septal ablation

Screening of 1st degree relatives

49
Q

How often due to first degree relatives need to be screened for hypertrophic cardiomyopathy?

A

Annual echo until age 20 and then Q5 yrs

50
Q

______ is the MC cause of HF in the US. What are 4 common causes?

A

Ischemic Cardiomyopathy

CAD
cocaine
vasospams
thrombus

51
Q

Ischemic Cardiomyopathy is characterized by _______. Mainly affects the ____ but can involve ____. How does it present?

A

systolic dysfunction

LEFT ventricle but can involve the right or both ventricles

presents like CHF

52
Q

What are the diagnostic things you would need to order in ischemic cardiomyopathy? What will each show?

A

EKG: possible Q waves
CXR: may have pulm edema
Echo: decreased LVEF and/or regional wall motion abnormalities
coronary angiography: recommended!! especially if LV dysfunction cause is unknown

53
Q

What is ischemic cardiomyopathy management?

A

Revascularization (PCI or CABG)! For acute ischemia/infarction

Consider nuclear viability study to determine if myocardial dysfunction is due to scarring or hibernating myocardium

manage the CHF

54
Q

How do you prevent sudden cardiac arrest?

A

External wearable defibrillator

or implanted defibrillator

55
Q

What is Arrhythmogenic Right Ventricular Cardiomyopathy characterized by? What is the underlying cause?

A

Characterized by ventricular arrhythmias and a specific myocardial pathology

inherited, autosomal dominant

56
Q

What is happening in Arrhythmogenic Right Ventricular Cardiomyopathy? What does it lead to?

A

RV free wall myocardium is replaced by fibrous/fatty tissue, producing RV dilation and myocardial thinning

Leads to abnormal RV function and possible LV dysfunction later on

57
Q

Arrhythmogenic Right Ventricular Cardiomyopathy can lead to ____ in young adults. What is the mean age of presentation?

A

can cause sudden death in young adults

Mean age of presentation is 30yo

58
Q

What is the presentation of Arrhythmogenic Right Ventricular Cardiomyopathy? What is the highlighted symptom? What is the management?

A

palpitations syncope, SCA, chest pain, dyspnea

management:
manage CHF symptoms

antiarrhythmics, ablation, or ICD for vent arrhythmias

heart transplant

59
Q

What is the MC presenting arrhythmia in Arrhythmogenic Right Ventricular Cardiomyopathy? What diagnostic tests should you order?

A

MC presenting arrhythmia is VT with LBBB pattern

echo and cardiac MRI

60
Q

What is Left Ventricular Noncompaction? How does it present?

A

Rare congenital cardiomyopathy that results from altered myocardial wall due to intrauterine arrest of compaction of the loose interwoven meshwork

CHF, thromboembolism, and ventricular arrhythmias occur

61
Q

How do you diagnose Left Ventricular Noncompaction? What is the tx?

A

Echo to start; Cardiac MRI to confirm

Consider cardiac transplant

62
Q

What is Stressed Induced Cardiomyopathy? What is the MC pt?

A

Causes an ACS (even STEMI) in the absence of critical CAD, due to a high catecholamine surge a result of intense psychological or physical stress

Primarily occurs in postmenopausal women

63
Q

How do you diagnosis stress induced cardiomyopathy? What is the tx?

A

Characterized by LV apical ballooning on echo or LV angiography (systolic dysfunction of the apex and/or mid segments)

Almost all patients recover in a few weeks
Treat with beta blockers for at LEAST 1 YEAR to try and prevent CV events

64
Q

What are 2 other names for Stressed Induced Cardiomyopathy?

A

broken heart syndrome and Takotsubo cardiomyopathy

65
Q

At what point is stenosis in heart vessels considered significant?

A

70% and greater is considered significant stenosis

66
Q

What is this? What cardiomyopathy is it associated with?

A

LV apical ballooning on echo or LV angiography

Stressed Induced Cardiomyopathy

67
Q
A

Cardio (24 decks)

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