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Cardiology (Fall 2023) > Lecture 12: Cardiomyopathy > Flashcards

Lecture 12: Cardiomyopathy Flashcards

1
Q

Define cardiomyopathy.

A
  • disorders characterized by morphologically and functionally abnormal myocardium in absence of any other disease that could cause the observed “phenotype” (presentation i assume).
  • Excludes cardiac dysfunction caused by structural heart disease.

Structural HD: CAD, primary valve disease, and HTN

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2
Q

What are the 3 classifications of cardiomyopathy?

A
  • Dilated
  • Hypertrophic
  • Restrictive
  • Arrhythmogenic RV CM/dysplasia (ARVC/D)
  • unclassified CM

can be further broken into: intrinstic v extrinsic. primary v. secondary. ischemic v nonischemic.

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3
Q

What part of the heart is primarily affected in cardiomyopathy?

A

LV function

causes dysfunction in systole/diastole or both. presentation varies from asymptomatic to CHF and cardiac arrest

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4
Q

What is the initial imaging modality for cardiomyopathy?

A

Echocardiography

can also get nuclear imaging, coronary angiography w left ventriculography, and cardiac MRI.

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5
Q

What does systolic dysfunction eventually lead to?

A
  1. Decease in myocardial contractility and reduction in LVEF
  2. Compensatory LV enlargement
  3. compensatory Higher stroke volume
  4. HF develops once this fails
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6
Q

What characterizes systolic dysfunction?

A
  • Decreases in myocardial contractility
  • Reduction in LVEF
  • Heart will compensate by enlarging LV.
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7
Q

What characterizes diastolic dysfunction?

A
  • Abnormal LV relaxation and filling
  • Elevated filling pressures
  • Can occur with/without systolic dysfunction.
  • However, if there is systolic dysfunction, it will always occur.

difficult to quantify on echo therefore often missed or underestimated

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8
Q

What is myocarditis?

A
  • Inflammatory, infiltrative process of myocardium due to both infectious and non-infectious causes.
  • results in Necrosis and/or degeneration of myocytes
  • Leads to myocardial dysfunction and dilated cardiomyopathy.

may be acute, subacute, or chronic

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9
Q

What are the two main mechanisms by which myocarditis occurs?

A
  • Host-mediated: direct cytotoxic effect of the causative agent.
  • Autoimmune-mediated: secondary immune response.
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10
Q

What occurs in the acute phase of myocarditis?

A
  • First 2 weeks
  • Myocyte death due to causative agent.
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11
Q

What occurs in the chronic phase of myocarditis?

A
  • After 2 weeks
  • Result of inappropriate, overactive immune response.
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12
Q

What are the primary viral causes of myocarditis?

A
  • Adenovirus
  • Coxsackie B virus
  • CMV
  • COVID-19
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13
Q

What are the primary toxins that cause myocarditis?

A
  • Alcohol
  • Anthracyclines
  • Cocaine
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14
Q

Who is myocarditis MC in?

A

20-50 y/o men

men have higher mortality rate with this too. frequency of myocarditis is poorly defined d/t variability of clinical presentation

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15
Q

What typically precipitates infective myocarditis?

A
  • Acute febrile illness/respiratory infection

Will develop a few days or weeks after.

no known underlying cardiac pathology

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16
Q

What are the classic symptoms associated with infective myocarditis?

A
  • SOB
  • Pleural/pericardial chest pain
  • Fever/chills
  • HF
  • Arrhythmias (palps, syncope, sudden death)

can be gradual or abrupt with decreased CO, shock and severely depressed LV systolic fxn

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17
Q

What PE findings are we looking for in infective myocarditis?

A
  • Pericardial friction rub
  • S3/S4
  • Mitral or tricuspid regurg murmur
  • Volume overload
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18
Q

What are the important initial diagnostic tests to order for suspected infective myocarditis?

A
  • EKG - sinus tach, dysrhythmias, PVCs, ST-T changes
  • Cardiac Biomarkers (elevated troponins)
  • CXR (non-specific but could see cardiomeg,pulm edema, pleural effusion)
  • Transthoracic echo - CRITICAL
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19
Q

What labs are appropriate to order for suspected infective myocarditis?

A
  • CRP
  • ^ESR
  • CBC (eosinophilia)
  • +/- Rheumatology workup
  • Serum viral antibody titers
  • BNP (probs >100)
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20
Q

what other imaging can be used in suspected infective myocarditis

A

cardiac MRI
helps assess extent od inflammation, myocyte necrosis and scarring. also shows ventricular size/shape, wall motion abnormalities, and pericardial effusion

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

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21
Q

How is infective myocarditis concretely diagnosed?

A

Histologic evidence via endomyocardial biopsy.

Only do this if there is a high probability it will change management.

rarely used because seldom changes management

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22
Q

according to the AHA/ACC when is a EMB reccomended and when is it suggested

A
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23
Q

What is the primary treatment for myocarditis?

A
  1. Consult cardiology
  2. LVEF <40% = ACEI and BB
  3. NSAIDS for pain (colchicine)
  4. arrhythmic management

trials using immunosuppression, steroids, IVIG and antivirals have shown no benefit

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24
Q

what are typical causes of noninfective myocarditis

A
  • medications
  • illicit drugs
  • toxic substances
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25
Q

What is the primary treatment for noninfectious myocarditis?

A

If LVEF >40%, Monitor until HF symptoms start occurring.
Once HF symptoms occur, refer to cardiology.

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26
Q

What is the #1 reason for heart transplant?

A

Dilated cardiomyopathy

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27
Q

What is dilated cardiomyopathy?

A
  • LVEF < 40%
  • No CAD or valvular disease
  • Dilation and impaired contraction of one or both ventricles, predominantly the LV

Regurgitation may occur due to the stretch of mitral/aortic valves.

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28
Q

Who is dilated cardiomyopathy MC in?

A

3x more common in Black patients

once symptoms manifest, mortality rate is 50% at 5 years

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29
Q

What is the primary cause of dilated cardiomyopathy?

A

Idiopathic!!
could also be:
* infectious
* excessive alcohol
* genetic
* systemic disorders
* peripartum
* endocrinopathies
* tachycardia induced
* arrhythmia associated

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30
Q

what viruses can be infectious causes of dilated cardiomyopathy

A
  • Parvo B19
  • herpes
  • coxsackievirus
  • influenza
  • adenovirus
  • CMV
  • HIV
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31
Q

What are the two main diseases that can lead to DCM?

A
  • Chagas disease (protozoan infection. leading cause of DCM in central and south america)
  • Lyme disease (manifests as conduction abnormality. may cause myocardial dysfunction d/t myocarditis)

Trypanosoma cruzi

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32
Q

What are the 3 main bacterial causes of DCM?

A
  • TB
  • Meningococcal
  • Pneumococcal
33
Q

What is the main genetic inheritance of DCM?

A
  • Autosomal Dominant
  • involves antibodies to a variety of cardiac proteins
34
Q

what systemic disorders can lead to DCM

A
  • sarcoidosis
  • SLE
  • Celiac
  • Scleroderma
  • RA
35
Q

what endocrine disorders can lead to DCM

A
  • thryoid dysfunction
  • pheochromocytoma
  • cushings
  • GH excess/deficiency
36
Q

what are the arrhythmia associated etiologies for DCM

A
  • PVC-mediated (>15% burden)
  • RV-paced rhythm
37
Q

When specifically does peripartum CM occur?

A

Late pregnancy or early postpartum.

Early treatment can resolve it within 2-3 months.

presents as SCA or CHF

38
Q

what are tachycardia induced etiologies for DCM? how does this cause DCM?

A
  • afib, SVT, AVNRT
  • HR correlates with LV dysfunction
  • Causes reduced myocyte contractility
  • LV dysfunction can occur w/o dialtion
39
Q

What is the DCM etiology mnemonic?

A
  • Alcohol Abuse
  • Beriberi (wet) Vit B1 (thiamine) deficiency
  • Coxsackie B myocarditis
  • Chronic cocaine use
  • Chagas’ disease
  • Doxorubicin toxicity

ABCCCD

A Bunch of stuff Can Cause Cardiac Disease

40
Q

what would the PE show in DCM

A
  • rales
  • elevated JVP
  • S3 gallop
  • murmur o mitral or tricuspid regurg
  • peripheral edema
  • ascites
41
Q

What EKG findings can be seen in DCM?

A
  • Tachycardia
  • Pulsus alternans
  • LBBB
42
Q

What symptom would prompt us to order a BNP or NT-proBNP for DCM? Why do we order these?

A

Dyspnea

tests are ordered for prognosis and disease severity

43
Q

what is the purpose of an echo in DCM diagnosis

A

excludes valvular disease and confirms ventricular dilation, LVsystolic fxn or pulm HTN

44
Q

what is the treatment plan for dilated cardiomyopathy

A
  • treat underlying source (commonly resolves LV dysfunction)
  • CHF management
  • Prevention of SCA
  • Heart transplant
45
Q

What characterizes restrictive cardiomyopathy?

A
  • Nondilated ventricle with impaired filling
  • Fibrosis or infiltration of the ventricular wall
  • Diastolic dysfunction
  • Biatrial enlargement

Most uncommon type.

46
Q

What are the primary causes of restrictive cardiomyopathy?

A
  • Infiltrative disorders (amyloidosis, sarcoidosis, fatty infiltration)
  • Storage Disease (Hemochromatosis, Fabry’s)
  • Radiation, chemo, carcinoid heart disease, hypereosinophilic syndrome

MCC is chemo in the US.

47
Q

How is restrictive cardiomopathy diagnosed

A

echo or cardiac MRI

Endomyocardial biopsy may be considered

48
Q

How do we treat restrictive cardiomyopathy?

A
  • Treat underlying causes
  • Diuretics (edema and congestion)

Most likely bc it backs up into the lungs since filling gets back up due to poor diastolic function.

49
Q

What characterizes hypertrophic cardiomyopathy?

A
  • LV hypertrophy (occasionally RV involved)
  • Interventricular septum dysfunction most commonly involved as myocytes build up.
  • NOT due to HTN or valvular issues.
  • Diastolic dysfunction
50
Q

How is HCM typically inherited? what does this genetic mutation cause?

A

Autosomal dominant - this genetic mutation causes mutations of sarcomere genes

51
Q

What valve is at risk in HCM?

A

Aortic valve, since it sits above the interventricular septum.

52
Q

What does Hypertrophic cardiomyopathy lead to

A

can lead to LV outflow obstruction, MI, and/or mitral regurg

53
Q

How does HCM typically present?

A
  • Fatigue
  • CP
  • CHF
  • Syncope
  • SCA
  • Carotid pulses bisferiens d/t mimicked AS
  • increased risk for arrhythmias
54
Q

What murmur may occur in HCM?

A
  • Mid-systolic, harsh, 3rd-4th intercostal that is louder with valsalva
  • Quieter on squatting.
55
Q

What is the usual EKG change seen in HCM?

56
Q

What is the diagnostic modality of choice for HCM?

A

Echocardiogram - this will show LV wall >1.5 cm thick

57
Q

What medications are we advised to AVOID in HCM treatment?

A
  • Diuretics
  • Vasodilators

We do not want lowering of preload.

58
Q

What medications are indicated for HCM?

A
  • BBs
  • Verapamil specifically (relaxes contractility)
59
Q

What procedures may help with HCM?

A
  • Septal myectomy
  • Alcohol septal ablation
60
Q

What screening is recommended for any 1st degree relative with HCM?

A
  • Annual echo until 20
  • Q5years afterwards
61
Q

What is the MCC of HF in the US?

A

Ischemic cardiomyopathy

results from death/damage/hibernation of myocardium d/t reduced O2.

62
Q

What is the typical cause of ischemic cardiomyopathy?

A

CAD

but can be from any source of ischemia such as cocaine, vasospasm, thrombus ect.

63
Q

What characterizes ischemic cardiomyopathy?

A
  • Systolic dysfunction
  • LV involvement primarily but can involve RV or both ventricles
  • presents as CHF (edema, dyspnea, JVD)
64
Q

What would an EKG and CXR show in ischemic cardiomyopathy

A
  • EKG - Possible Q waves
  • CXR - may see pulm edema
65
Q

What should appear on echo for ischemic cardiomyopathy?

A
  • Decreased LVEF
  • Regional wall motion abnormality
66
Q

What diagnostic study after an echo may help with ischemic cardiomyopathy?

A

Coronary angiography - especially if LV dysfunction cause is unknown

67
Q

How do we manage ischemic cardiomyopathy?

A
  • Revascularization via PCI or CABG
  • Nuclear viability study to check to determine if myocardial dysfunction is d/t hibernation or scarring
  • CHF management
68
Q

How do we prevent SCA?

A
  • External wearable defibrillator (Lifevest)
  • ICD
69
Q

What characterizes arrhythmogenic RVCM?

A
  • Ventricular arrhythmias + specific myocardial pathology
  • RV free wal myocardium replaced by fibrous/fatty tissue leading to RV dilation and abnormal function of RV
  • Causes sudden death in young adults in Europe primarily
70
Q

How does arrhythmogenic right ventricular cardiomyopathy present

A
  • Chest pain
  • Palpitations
  • Syncope
  • SCA
71
Q

How do we diagnose ARVCM

A

Echo and Cardiac MRI

72
Q

How do we manage arrhythmogenic RVCM?

A
  • Diuretics for systemic congestion
  • Anitarrhythmics, ablation, or ICD for ventricular arrhythmias.
73
Q

What is Left ventricular noncompaction cardiomyopathy?

A
  • rare Congenital cardiomyopathy
  • Altered myocardial wall d/t intrauterine arrest of compaction of loose interwoven meshwork
74
Q

How does LV concompaction present

A
  • CHF
  • Thromboembolism
  • Ventricular arrhythmias

DX CONFIRMED W CARDIAC MRI

75
Q

How do we treat Left ventricular noncompaction cardiomyopathy?

A

Heart transplant

76
Q

What is takotsubo cardiomyopathy?

A
  • Stress-induced/broken heart syndrome
  • Causes ACS due to high catecholamine surge

MC in postmenopausal women.

Result of intense psychological or physical stress

77
Q

How is takotsubo diagnosed?

A

LV apical ballooning on Echo or LV angiography

78
Q

How is takotsubo’s treated?

A

BBs for a year

Cardiology (Fall 2023) (45 decks)

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