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Cardiomyopathy Flashcards

1
Q

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

A

Cardiomyopathy

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2
Q

What does cardiomyopathy exclude?

A

Cardiac dysfunction caused by structural heart disease such as CAD, primary valve disease, HTN

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3
Q

What are the traditional classifications of cardiomyopathy?

A
  • Dilated cardiomyopathy
  • Restrictive cardiomyopathy
  • Hypertrophic cardiomyopathy
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4
Q

What are the WHO/ISFC classifications of cardiomyopathy?

A
  • Dilated, Hypertrophic, Restrictive
    AND
  • Arrhythmogenic right ventricular cardiomyopathy/dysplasia
  • Unclassified cardiomyopathies
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5
Q

In addition to the traditional classification and WHO/ISFC classification, cardiomyopathy can be classified as what?

A
  • Intrinsic vs extrinsic
  • Primary vs secondary
  • Ischemic vs nonischemic
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6
Q

What are types of familial/genetic cardiomyopathy?

A
  • Unidentified gene defect
  • Disease sub-type
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7
Q

What are types of non-familial/non-genetic cardiomyopathy?

A
  • Idiopathic
  • Disease sub-type
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8
Q

What does cardiomyopathy predominantly involve?

A
  • LV
  • Dysfunction of systole, diastole, or both
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9
Q

How can the presentation of cardiomyopathy range?

A
  • Asymptomatic to decompensated CHF to cardiac arrest
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10
Q

What are diagnostic modalities for cardiomyopathy?

A
  • Echocardiography
  • nuclear imaging
  • coronary angiography w/ left ventriculography
  • Cardiac MRI

Dysfunction can be transient or permanent

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11
Q

What does systolic dysfunction cause?

A
  • Decrease in myocardial contractility and reduction in LVEF
  • Compensatory mechanisms of LV enlargement resulting in higher stroke volume
  • Frank-Starling relationship (increased stretch= increased contractility
  • Eventually will lead to HF
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12
Q

What does diastolic dysfunction cause?

A
  • Cardiac dysfunction d/t abnormal LV relaxation and filling, accompanied by elevated filling pressures
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13
Q

What is the relationship between diastolic dysfunction and systolic dysfunction?

A

May occur with or without systolic dysfunction but always present if systolic dysfunction occurs

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14
Q

What is one problem with diastolic dysfunction diagnosis?

A

More difficult to quantify on echo so often underestimated or missed

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15
Q

What is myocarditis?

A
  • Inflammatory, infiltrative process involving the myocardium caused by infectious and noninfectious conditions
  • May be acute, subacute, or chronic
  • Results in necrosis and/or degeneration of myocytes
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16
Q

What can myocarditis lead to?

A
  • Myocardial dysfunction
  • Dilated cardiomyopathy
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17
Q

What is the pathogenesis of myocarditis?

A
  • Varies depending on underlying cause (often undetermined)
  • 2 main mechanisms: host-mediated and autoimmune mediated
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18
Q

What causes host-mediated myocarditis?

A

direct cytotoxic effect of the causative agent

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19
Q

What causes autoimmune-mediated myocarditis?

A

secondary immune response

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20
Q

What are the 2 main phases of myocardial damage in myocarditis?

A
  • Acute phase and chronic phase
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21
Q

What are characteristics of the acute phase of myocarditis?

A
  • First 2 weeks
  • Myocyte death is a direct result of the causative agent, leading to cell-mediated cell toxicity
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22
Q

What are characteristics of the chronic phase of myocarditis?

A
  • Post 2 weeks
  • Result of inappropriate, overactive immune response
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23
Q

What are 3 infectious causes of myocarditis to know?

A
  • Adenovirus (COVID-19)
  • Coxsackie B virus
  • Cytomegalovirus
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24
Q

What are 3 noninfectious causes of myocarditis to be aware of?

A

Cardiotoxins:

  • Alcohol
  • Anthracyclines
  • Cocaine
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25
Q

Which group has a slightly higher mortality rate due to myocarditis?

A

Men

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26
Q

What is the epidemiology of myocarditis?

A
  • Frequency poorly defined due to variability of clinical presentation
  • MC in patients 20-50 y/o
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27
Q

What is the presentation of myocarditis?

A
  • Days to few weeks after onset of acute febrile illness/respiratory infection
  • No known underlying cardiac pathology***
  • SOB, pleural/pericardial chest pain, +/- fever, chills
  • Could present with HF
  • Palpitations, syncope, or sudden death may occur due to arrhythmias
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28
Q

What are the classic symptoms of myocarditis?

A
  • SOB, pleural/pericardial chest pain, +/- fever, chills
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29
Q

If a patient with myocarditis has HF, what presentation may be present?

A

Gradual or abrupt
* Decreased cardiac output
* shock
* Severely depressed LV systolic function

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30
Q

What will be present on physical exam of myocarditis?

A

Heart auscultation:
* Pericardial friction rub
* Tachycardia
* S3 or S4
* Murmur of mitral or tricuspid regurgitation if ventricular dilation is severe

Heart failure: Volume overload

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31
Q

What initial testing should be performed for myocarditis?

A
  • EKG
  • Cardiac biomarkers
  • CXR
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32
Q

What will be findings of the EKG, cardiac biomarkers, and CXR in myocarditis?

A
  • EKG: sinus tachycardia, dysrhythmias, ventricular ectopy (PVCs), ST-T changes
  • Cardiac biomarkers: elevated troponin levels common (esp. in rapid onset myocarditis)
  • CXR: typically nonspecific, but may see cardiomegaly, pulmonary edema, pleural effusion
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33
Q

What additional testing can be performed for myocarditis?

A
  • Labs: CRP, ESR (elevated), CBC (eosinophilia), +/- rheumatologic workup, serum viral antibody titers, BNP (in setting of HF)
  • Transthoracic echo
  • Cardiac MRI
  • Endomyocardial biopsy
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34
Q

Does a transthoracic echo need to be performed in myocarditis? Why or why not?

A
  • Yes! It is critical
  • Allows visualization of myocardium, assessment of ventricular function, helps to r/o other pathology
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35
Q

Why would a cardiac MRI be performed?

A
  • 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

Cardiology will decide whether or not to perform cardiac MRI

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36
Q
A
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37
Q

What does concrete confirmation of myocarditis require?

A

Histological evidence

Most cases are presumed

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38
Q

When would endomyocardial biopsy be obtained?

A

If there is a high probability that results will change patient management

Not our call (cardiology) and has limited sensitivity and specificity

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39
Q

When is endomyocardial biopsy recommended by the AHA/ACC?

A
  • Fulminant, unexplained HF (new onset <2 weeks w/hemodynamic compromise)
  • Unexplained new onset HF 2 weeks-3 months w/dilated LV, new ventricular arrhythmia, Mobitz II 2nd degree AV block, 3rd degree block or failure to respond to usual care in 1-2 weeks
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40
Q

When is EMB suggested by AHA/ACC?

A
  • HF >3 months with dilated LV, new ventricular arrhythmia, Mobitz II 2nd degree AV block, 3rd degree block or failure to respond to usual care in 1-2 weeks
  • HF with dilated cardiomyopathy with allergic reaction and/or eosinophilia
41
Q

How is myocarditis treated?

A
  • Consult cardiology
  • Treatment directed towards patient presentation
  • LVEF <40% –> ACEI, BB
  • NSAIDs for myopericardial chest pain
  • Arrhythmia management
  • Trials using immunosuppressive therapies, corticosteroids, IVIG, and antivirals have not shown benefit
42
Q

What is most noninfectious myocarditis caused by?

A
  • Medications
  • Illicit drugs
  • Toxic substances
43
Q

How can patients with myocardial injury from toxic agents be managed?

A
  • Monitored if LVEF (>40%) is preserved and no HF symptoms present
  • If HF or LVEF ensues, managed by cardiology
44
Q

What type of cardiomyopathy accounts for the majority of cases?

A

Dilated cardiomyopathy

45
Q

Which population more commonly gets dilated cardiomyopathy?

A

Black patients

46
Q

What is the prognosis of dilated cardiomyopathy once symptoms manifest?

A

mortality of 50% at 5 years

47
Q

What are characteristics of dilated cardiomyopathy?

A
  • Dilation and impaired contraction of one or both ventricles, predominantly the LV
  • LVEF <40% without CAD or valvular disease
48
Q

What is the etiology of dilated cardiomyopathy?

A
  • Idiopathic
  • Excessive alcohol consumption
  • Infectious
  • Genetic
  • Systemic disorders
  • Peripartum
  • Endocrinopathies
  • Tachycardia induced
  • Arrhythmia associated
49
Q

What are infectious causes of dilated cardiomyopathy?

A

Viral
* Parvo B19
* Herpes
* Coxsackievirus
* Influenza
* Adenovirus
* CMV
* HIV

Bacterial
* TB, meningococcal, pneumococcal

Chagas disease, lyme disease

50
Q

What is chagas disease (causes dilated cardiomyopathy)?

A
  • Protozoan infection
  • Leading cause of DCM in central and south america
51
Q

How does lyme disease usually manifest in dilated cardiomyopathy?

A
  • conduction abnormality
  • May cause myocardial dysfunction due to myocarditis
52
Q

What are genetic causes of dilated cardiomyopathy?

A
  • Autosomal dominant predominantly involves antibodies to cardiac proteins
  • Inherited syndromes such as muscular dystrophies, hemochromotosis, thalassemias
53
Q

What are systemic disorders that can cause dilated cardiomyopathies?

A
  • Sarcoidosis
  • SLE
  • Celiac
  • Scleroderma
  • RA
54
Q

What endocrine disorders can cause dilated cardiomyopathy?

A
  • Thyroid dysfunction
  • Pheochromocytoma
  • Cushing’s
  • GH excess or deficiency
55
Q

What arrhythmias can cause dilated cardiomyopathy?

A
  • PVC mediated
  • RV paced rhythm
56
Q

What is the etiology of peripartum CM and how does it present?

A
  • Unclear etiology
  • Late in pregnancy or early postpartum
  • Presents as CHF or SCA (sudden cardiac arrest)
  • typically normalizes after 2 to 3 months of therapy
57
Q

What are causes of tachycardia induced dilated cardiomyopathy?

A
  • Afib, SVT, AVNRT
58
Q

What is the pathophysiology of tachycardia induced dilated cardiomyopathy?

A
  • HR correlates with LV dysfunction
  • Reduced myocyte contractility
  • LV dysfunction can occur without dilatation
59
Q

What is the mneumonic for dilated cardiomyopathy etiology?

A
  • A- alcohol
  • Bunch of stuff: beriberi (wet)
  • Can: Coxsackie B myocarditis
  • Cause: Chronic cocaine use
  • Cardiac: Chaga’s disease
  • Dilation: doxorubicin toxicity

beriberi heart disease is indistinguishable from DCM

60
Q

What is the presentation of dilated cardiomyopathies?

A
  • Gradual HF development
  • Arrhythmias
  • Sudden death possible
61
Q

What is present on PE of dilated cardiomyopathy due to HF?

A
  • Rales
  • Elevated JVP
  • S3 gallop
  • Murmur of mitral or tricuspid regurg
  • Peripheral edema
  • Ascites
62
Q

What arrhythmias can be caused by dilated cardiomyopathy?

A
  • Tachycardia
  • Pulsus alternans
  • LBBB
63
Q

How is dilated cardiomyopathy diagnosed?

A
  • If dyspnea present, BNP or NT-proBNP to determine prognosis and severity
  • Echo
  • Possibly radionuclide ventriculography (MUGA), cardiac MRI
64
Q

Why would a echo be helpful in dilated cardiomyopathy?

A
  • Excludes valvular disease
  • Confirms ventricular dilation, reduced LV systolic function (and RV systolic dysfunction if applicable), or pulmonary HTN
65
Q

How is dilated cardiomyopathy treated?

A
  • Treat underlying source if known, which commonly resolves LV dysfunction
  • CHF management
  • Prevention of SCA
  • Heart transplant
66
Q

What are characteristics of restrictive cardiomyopathy?

A
  • Nondilated ventricle with impaired filling
  • Caused by fibrosis or infiltration of ventricular wall
  • Causes diastolic dysfunction
67
Q

How does restrictive cardiomyopathy present on echo?

A
  • Non-hypertrophied with moderate to severe biatrial enlargement
68
Q

What are causes of restrictive cardiomyopathy?

A
  • Infiltrative disorders: amyloidosis, sarcoidosis, fatty infiltration
  • Storage diseases: hemochromatosis, fabry disease
  • Radiation/chemo
  • Carcinoid heart disease
  • Hypereosinophilic syndrome
69
Q

How is restrictive cardiomyopathy diagnosed?

A
  • Echo or cardiac MRI
  • Endomyocardial biopsy may be considered
70
Q

How is restrictive cardiomyopathy treated?

A
  • Treat underlying cause
  • Reduce pulmonary and systemic congestion with diuretics
71
Q

What causes hypertrophic cardiomyopathy?

A

mutations of sarcomere genes
autosomal dominant trait with variable penetrance

72
Q

What are characteristics of hypertrophic cardiomyopathy?

A
  • LV hypertrophy
  • Occassionally RV involved
  • Not caused by pathologic loading conditions, such as HTN and valvular disease
  • Interventricular septum commonly prominently involved
  • Leads to diastolic dysfunction
73
Q

What can hypertrophic cardiomyopathy lead to?

A
  • LV outflow obstruction, myocardial ischemia, and/or mitral regurgitation
74
Q

What is the presentation of hypertrophic cardiomyopathy?

A
  • Fatigue
  • Chest pain
  • CHF
  • Syncope
  • SCA
  • Carotid pulsus bisferiens d/t mimicked aortic stenosis
  • Increased risk for arrhythmias (afib, SVT, and VT/VF) and SCA

LISTEN FOR THIS MURMUR ON SPORTS PHYSICALS

75
Q

What will hypertrophic cardiomyopathy murmur sound like?

A
  • Mid-systolic
  • Harsh
  • 3rd and 4th intercostals
  • Louder with valsalva
  • Quieter with squatting
76
Q

How is hypertrophic cardiomyopathy diagnosed?

A
  • EKG with LVH pattern
  • Echo = diagnostic of choice with LV wall > 1.5 cm thick
77
Q

How is hypertrophic cardiomyopathy managed?

A
  • Avoid volume depletion
  • Activity restriction
  • Beta-blockers or verapamil (helps relax contractility)
  • Avoid diuretics and vasodilators (would decrease preload)
  • Septal myectomy or alcohol septal ablation
  • Screening of 1st degree relatives
78
Q

How do you screen 1st degree relatives for hypertrophic cardiomyopathy?

A
  • Annual echo until age 20 and then Q5 yrs
79
Q

What is the most common cause of HF in the US?

A

Ischemic cardiomyopathy

80
Q

What is the cause of ischemic cardiomyopathy?

A
  • death/damage/hibernation of myocardium d/t reduced oxygen
  • typically from CAD, but any ischemia can cause
  • Cocaine, vasospasm, thrombus can also cause
81
Q

What are characteristics of ischemic cardiomyopathy?

A
  • transient or permanent systolic dysfunction
  • predominantly affects LV, but can involve RV or both ventricles
82
Q

What is the presentation of ischemic cardiomyopathy?

A
  • CHF
  • (edema, dyspnea, JVD)
83
Q

How is ischemic cardiomyopathy diagnosed?

A
  • Q waves on EKG possible
  • CXR may reveal pulmonary edema
  • Echo–> decreased LVEF, regional wall motion abnormality
  • Coronary angiography recommended, especially if LV dysfunction cause unknown
84
Q

How is ischemic cardiomyopathy managed?

A
  • Revascularization (PCI or CABG) for acute ischemia/infarction
  • Consider nuclear viability study to determine if myocardial dysfunction due to scarring or hibernating myocardium
  • CHF management
  • Prevention of SCA
  • Cardiac rehab
85
Q

How is SCA prevented in ischemic cardiomyopathy?

A
  • External wearable defibrillator or implanted cardioverter-defibrillator
86
Q

What are characteristics of arrhythmogenic right ventricular cardiomyopathy?

A
  • Ventricular arrhythmias and specific myocardial pathology
  • RV free wall myocardium replaced by fibrous/fatty tissue, producing RV dilation (leads to abnormal RV function)
87
Q

Arrhythmogenic right ventricular cardiomyopathy is a important cause of death in which population?

A

Young adults

88
Q

Where is arrhythmogenic right ventricular cardiomyopathy more prevalent?

A

Europe, rare in US

89
Q

What is the presentation of arrhythmogenic right ventricular cardiomyopathy?

A
  • Chest pain
  • Palpitations
  • Syncope
  • SCA
90
Q

How is arrhythmogenic right ventricular cardiomyopathy diagnosed?

A
  • Echo and cardiac MRI
91
Q

How is arrhythmogenic right ventricular cardiomyopathy managed?

A
  • Manage systemic congestion with diuretics
  • Manage ventricular arrhythmias with antiarrhythmics, ablation or ICD
92
Q

What are the unclassified cardiomyopathys and their causes?

A

Left ventricular noncompaction
* Congenital
* Altered myocardial wall due to intrauterine arrest of compaction of loose interwoven meshwork

Stress-induced cardiomyopathy
* ACS (even STEMI) in absence of critical CAD, due to catecholamine surge bc of intense psychological or physical stress

93
Q

What is the presentation of unclassified cardiomyopathy (left ventricular noncompaction)?

A
  • CHF
  • Thromboembolism
  • Ventricular arrhythmias
94
Q

How is left ventricular noncompaction diagnosed?

A

Cardiac MRI to confirm

95
Q

How is left ventricular noncompaction treated?

A

Cardiac transplant

96
Q

Which population more commonly gets stress-induced cardiomyopathy?

A

Postmenopausal women

97
Q

How is stress-induced cardiomyopathy diagnosed?

A

LV apical ballooning on echo or LV angiography

98
Q

How is stress-induced cardiomyopathy treated?

A

Almost all patients recover in a few weeks
* Treat with beta blockers for at least a year to try to prevent CV events

Cardiovascular (17 decks)

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