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Cardiovascular > Cardiomyopathy > Flashcards

Cardiomyopathy Flashcards

1
Q

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

A

Cardiomyopathy

Excludes cardiac dysfunction caused by structural heart disease: CAD, primary valve disease, HTN

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2
Q

Cardiomyopathy involves which part of the heart and causes dysfunction of systole, diastole, or both?

A

LV

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3
Q

diagnostic modalities of Cardiomyopathy include:

A
  1. echo
  2. nuclear imaging
  3. coronary angiography w/ L ventriculography
  4. Cardiac MRI

resulting dysfunction can be transient or permanent

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4
Q

how does Systolic dysfunction happen?

A

decrease in myocardial contractility nd reduction in LVEF

Eventually fails and HF develops

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5
Q

what Compensatory mechanisms in systolic dysfunction aim to maintain cardiac output:

A
  • LV enlargement resulting in higher stroke volume
  • Frank-Starling relationship (↑stretch = ↑contractility)
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6
Q

how does Diastolic dysfunction happen?

A

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

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7
Q

T/F: diastolic dysfunction can happen with or w/o systolic dysfunction

A

T

Always present if systolic dysfunction occurs

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8
Q

Why is diastolic dysfunction often underestimeated/missed?

A

more difficult to quantify on echo

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9
Q

An inflammatory, infiltrative process involving the myocardium caused by infectious and noninfectious conditions

A

Myocarditis

May be acute, subacute, or chronic

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10
Q

what can Myocarditis lead to?

A
  • necrosis and/or degeneration of myocytes
  • myocardial dysfunction and dilated cardiomyopathy
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11
Q

Pathogenesis of Myocarditis

A

Varies depending on the underlying cause (which is often undetermined)

  1. Host-mediated: direct cytotoxic effect of the causative agent
  2. Autoimmune-mediated: secondary immune response
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12
Q

Myocardial damage occurs in 2 main phases:

A
  1. Acute phase:
    - First 2 weeks
    - Myocyte death is a direct result of the causative agent, leading to cell-mediated cell toxicity
  2. Chronic phase:
    - After 2 weeks
    - A result of an inappropriate, overactive immune response
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13
Q

what are the MC infectious causes of myocarditis

A
  1. adenovirus
  2. coxsackie B virus
  3. cytomegalovirus
  4. COVID
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14
Q

what are the MC cardiotoxin causes of myocarditis

A
  1. alcohol
  2. anthracyclines
  3. cocaine
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15
Q

Epidemiology of Myocarditis

A
  • Frequency is poorly defined d/t variability of clinical presentation
  • MC 20-50 y/o
  • Men have a slightly higher mortality rate
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16
Q

myocarditis presentation

A
  1. days-weeks after acute febrile illness / respiratory infection - No known underlying cardiac pathology
  2. SOB, pleural/pericardial chest pain, +/- fever, chills—Could also present with HF
    - gradual or abrupt with decreased cardiac output, shock, and severely depressed LV systolic function
    - Palpitations, syncope, or sudden death may also occur d/t arrhythmias
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17
Q

PE of myocarditis

A
  • pericardial friction rub, tachycardia, S3 or S4, murmur of mitral or tricuspid regurg if ventricular dilation is severe
  • HF → volume overload
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18
Q

initial testing for myocarditis

A
  1. EKG → sinus tachycardia, dysrhythmias, ventricular ectopy (PVCs), ST-T changes
  2. Cardiac biomarkers → elevated troponin levels common (esp. in rapid onset myocarditis)
  3. CXR → typically nonspecific, but may see cardiomegaly, pulmonary edema, pleural effusion
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19
Q

other testing for myocarditis (not initial)

A
  1. Labs: CRP, ESR (elevated), CBC (eosinophilia), +/- rheumatologic workup, serum viral antibody titers, BNP (in setting of HF)
  2. TTE: critical!
  3. cardiac MRI (CMR)
    - suggests myocarditis, but sensitivity and specificity are limited and time-dependent
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20
Q

what testing allows visualization of the myocardium, assessment of ventricular function, helps r/o / assess other pathology (regurg)

A

TTE

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21
Q

what testing helps assess extent of inflammation, myocyte necrosis and scarring, ventricular size / shape changes, wall motion abnormalities, and pericardial effusion

A

cardiac MRI (CMR)

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22
Q

Endomyocardial biopsy (EMB) should only be obtained if ?

A

there is a high probability that results will change patient management

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23
Q

tx for infectious myocarditis

A
  1. Consult Cardiology!
  2. tx directed toward presentation
    - LVEF <40% → ACE-I, BB
    - NSAIDs (possibly Colchicine) for myopericardial CP
    - Arrhythmia management
    - Trials using immunosuppressive therapies, corticosteroids, IVIG, and antivirals have not shown benefit
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24
Q

noninfectious myocarditis MCC by?

A

medications, illicit drugs, and toxic substances

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25
Q

Majority of cardiomyopathies are what type?

A

dilated

26
Q

what is the #1 reason for heart transplant

A

dilated cardiomyopathy

27
Q

dilated cardiomyopathy is MC in what race/ethnicity

A

Black pts
3x than white pts

28
Q

Once sx of dilated cardiomyopathy manifest, mortality is ?% at 5 years

A

50%

29
Q

dilated cardiomyopathy is characterized by?

A
  • dilation and impaired contraction of one or both ventricles, predominantly the LV
  • Defined by LVEF <40% w/o CAD or valvular disease
30
Q

causes of dilated cardiomyopathy

A

idiopathic

  • Excessive alcohol consumption
  • Infectious
  • Genetic
  • Systemic Disorders
  • Peripartum
  • Endocrinopathies
  • Tachycardia induced
  • Arrhythmia associated
31
Q

infectious causes of dilated cardiomyopathy

A
  1. Viral - Parvo B19, herpes, coxsackievirus, influenza, adenovirus, CMV, HIV
  2. Bacterial - TB, Meningococcal, Pneumococcal
  3. Chagas Disease
    - Caused by protozoan infection, Trypanosoma cruzi
    - Leading cause of DCM in Central and South America
  4. Lyme Disease
    - Usually manifests as conduction abnormality
    - May cause myocardial dysfunction due to myocarditis
32
Q

Systemic Disorders that cause dilated cardiomyopathy

A

Sarcoidosis, SLE, Celiac, Scleroderma, RA

33
Q

how do genetics cause dilated cardiomyopathy?

A
  • Autosomal dominant predominantly
  • Involve antibodies to a variety of cardiac proteins
  • Inherited syndromes, such as muscular dystrophies, hemochromatosis, thalassemias
34
Q

Endocrine Disorders that cause dilated cardiomyopathy

A

Thyroid dysfunction, pheochromocytoma, Cushing’s or GH excess or deficiency

35
Q

Arrhythmia Associated that cause dilated cardiomyopathy

A

PVC-mediated (>15% burden)
RV-paced rhythm

36
Q

how does Peripartum CM cause dilated cardiomyopathy?

A
  • Rare cause
  • Unclear etiology
  • Occurs late in pregnancy or early postpartum
  • Presents as CHF or SCA
  • Typically normalizes after 2 to 3 months of therapy
37
Q

how does Tachycardia cause dilated cardiomyopathy?

A
  • Afib, SVT, AVNRT
  • HR correlates with LV dysfunction
  • Causes reduced myocyte contractility
  • LV dysfunction can occur without dilatation
38
Q

DCM etiology mnemonic

A

ABCCCD - a bunch (of stuff) can cause cardiac dilation

  1. alcohol abuse
  2. beriberi (wet)
  3. coxsackie B myocarditis
  4. chronic cocaine use
  5. chagas’ disease
  6. doxorubicin toxicity
39
Q

presentation of dilated cardiomyopathy

A
  1. Gradual HF development
    - PE: rales, elevated JVP, S3 gallop, murmur of mitral or tricuspid regurg, peripheral edema, ascites
  2. Arrhythmias
    - Tachycardia, pulsus alternans, LBBB
  3. Sudden death possible
40
Q

how to diagnose DCM

A
  1. If dyspnea is present, BNP or NT-proBNP are necessary to determine prognosis and disease severity
  2. Echo
    - Excludes valvular disease and confirms ventricular dilation, reduced LV systolic function (and RV systolic dysfunction if applicable), or pulmonary HTN
  3. Possibly radionuclide ventriculography (MUGA), cardiac MRI
41
Q

tx for DCM

A
  • Treat underlying source if known, which commonly resolves LV dysfunction
  • CHF management
  • Prevention of SCA
  • Heart transplant
42
Q
  • Characterized by nondilated ventricle with impaired filling
  • Caused fibrosis/infiltration of the ventricular wall
  • Causes diastolic dysfunction
  • Ventricles predominantly non-hypertrophied on echo, with moderate to severe biatrial enlargement

what type of cardiomyopathy?

A

Restrictive Cardiomyopathy (RCM)

43
Q

causes of restrictive cardiomyopathy

A
  1. Infiltrative disorders
    - Amyloidosis, sarcoidosis, fatty infiltration
  2. Storage diseases
    - Hemochromatosis, Fabry disease
  3. Radiation, chemo, carcinoid heart disease, hypereosinophilic syndrome
44
Q

how to diagnose RCM

A

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

45
Q

tx for RCM

A

Treat underlying cause if known
Reduce pulmonary and systemic congestion
with diuretics

46
Q
  • Caused by mutations of sarcomere genes; genetic disposition
  • Characterized by LV hypertrophy

which cardiomyopathy is this?

A

hypertrophic cardiomyopathy

47
Q

what parts of the heart are involved in hypertrophic cardiomyopathy?

A
  • Occasionally RV involved
  • NOT caused by pathologic loading conditions, such as HTN and valvular disease (AS)
  • Interventricular septum is MC involved
  • Leads to diastolic dysfunction
48
Q

Hypertrophic Cardiomyopathy
may lead to ?

A

LV outflow obstruction, myocardial ischemia and/or mitral regurgitation

49
Q

pt presents with
- Fatigue, chest pain, CHF, syncope, SCA
- Carotid pulsus bisferiens d/t mimicked aortic stenosis
- Mid-systolic, harsh, 3rd and 4th ICS, louder with valsalva, quieter with squatting

what does this pt have?

A

Hypertrophic Cardiomyopathy

Increased risk for arrhythmias (afib, SVT, and VT/VF) and SCA

50
Q

diagnostics for Hypertrophic Cardiomyopathy

A
  • EKG may demonstrate LVH pattern
  • Echo is the diagnostic modality of choice
    — LV wall >1.5 cm thick
51
Q

management for Hypertrophic Cardiomyopathy

A
  1. Avoid volume depletion
  2. Activity restriction
  3. BB or Verapamil (helps relax contractility)
    - Avoid diuretics and vasodilators
  4. Septal myectomy or alcohol septal ablation
  5. Screening of 1st degree relatives
    - Annual echo until age 20 and then Q5 yrs
52
Q

Most common cause of HF in the U.S

A

Ischemic Cardiomyopathy

Results from death/damage/hibernation of myocardium d/t reduced O₂

53
Q

Ischemic Cardiomyopathy is typically results from what condition, but can be from any source of ischemia?

A

CAD

Cocaine, vasospasm, thrombus

54
Q

what type of cardiomyopathy
- Characterized by systolic dysfunction - Can be transient or permanent
- Predominantly affects LV, but can involve the RV or both ventricles
- CHF (edema, dyspnea, JVD)

A

Ischemic Cardiomyopathy

55
Q

diagnostics for Ischemic Cardiomyopathy

A
  1. Possible Q waves on EKG
  2. CXR - pulmonary edema
  3. Echo → decreased LVEF, regional wall motion abnormality
  4. Coronary angiography recommended, esp LV dysfunction cause unknown
56
Q

management for Ischemic Cardiomyopathy

A
  1. Revascularization (PCI or CABG)! For acute ischemia/infarction
  2. Consider nuclear viability study to determine if myocardial dysfunction is due to scarring or hibernating myocardium
  3. CHF management
  4. Prevention of SCA:
    - External wearable defibrillator
    - Implanted cardioverter - defibrillator
  5. Cardiac rehab
57
Q
  • Characterized by ventricular arrhythmias and a specific myocardial pathology
  • RV free wall myocardium is replaced by fibrous/fatty tissue, producing RV dilation –> RV function abnormal

what type of cardiomyopathy?

A

Arrhythmogenic Right Ventricular Cardiomyopathy

young adults; More prevalent in Europe; rare in US

58
Q

presentation and management for Arrhythmogenic Right Ventricular Cardiomyopathy

A
  1. chest pain, palpitations, syncope, SCA
  2. Diagnose w/ echo and cardiac MRI
  3. Management
    - Manage systemic congestion with diuretics
    - Manage ventricular arrhythmias with antiarrhythmics, ablation or ICD
59
Q
  • Rare congenital cardiomyopathy; 0.05% of echoes
  • Altered myocardial wall due to intrauterine
    arrest of compaction of the loose interwoven meshwork

what type of cardiomyopathy?

A

Left Ventricular Noncompaction

60
Q

presentation and tx for Left Ventricular Noncompaction

A
  1. Presentation: CHF, thromboembolism, and ventricular arrhythmias occur
  2. dx: Cardiac MRI
  3. tx: cardiac transplant
61
Q
  • 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

what type of cardiomyopathy

A

Stress-Induced Cardiomyopathy
AKA broken heart syndrome and Takotsubo cardiomyopathy

62
Q

dx and tx Stress-Induced Cardiomyopathy

A
  • dx: LV apical ballooning on echo or LV angiography (systolic dysfunction of the apex and/or mid segments)
  • tx: Almost all recover in a few wks
    — BB at least 1 yr

Cardiovascular (24 decks)

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