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CV Quiz 2 > Cardiomyopathies > Flashcards

Cardiomyopathies Flashcards

1
Q

What is cardiomyopathy?

A

heart disease resulting from an abnormality in myocardium

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2
Q

What kind of abnormalities occur in cardiomyopathy?

A

abnormalities in cardiac wall thickness, chamber size, mechanical and/or electrical dysfunction

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3
Q

What is primary cardiomyopathy?

A

confined to heart muscle

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4
Q

What is secondary cardiomyopathy?

A

myocardial involvement as a component of systemic or multiorgan disorder

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5
Q

What is dilated cardiomyopathy and what happens?

A

progressive cardiac dilation: systolic dysfunction, hypertrophy, Enlarged, heavy, flabby, dilated heart, HEART FAILURE, Thrombi may form, Regurgitation- may be due to ventricular dilation (functional regurgitation)

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6
Q

x-linked dilated cardiomyopathy is usually caused by mutation in what?

A

dystrophin gene

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7
Q

In 20-50% of dilated cardiomyopathy genetically causes cases what is the mutation?

A

Predominantly autosomal dominant mutations encoding cytoskeletal proteins like α-cardiac actin, desmin, & nuclear lamins A/C

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8
Q

What mutations in the mitochondrial genome cause dilated cardiomyopathy?

A

Mutations in genes involved in oxidative phosphorylation & fatty acid ß-oxidation

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9
Q

What virus usually cause acute viral myocarditis?

A

Coxasackie B or echovirus usually Self-limited infection in young people

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10
Q

How does acute viral myocarditis lead to dilated cardiomyopathy?

A

Mechanism: unclear– Myocyte cell death and fibrosis– Immune mediated injury– No change with immunosuppressive drugs

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11
Q

Two toxins that can cause dilated cardiomyopathy?

A

Alcohol and doxorubicin

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12
Q

What does alcohol do to cause dilated cardiomyopathy?

A

direct toxic effect myocardium &indirect toxic effect associated with thiamine deficiency lends to beriberi heart disease

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13
Q

What does doxorubicin (adriamycin) cause?

A

Dilated cardiomyopathy and heart failure• Anthracycline toxicity is dose-dependent

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14
Q

When does peripartum cardiomyopathy occur?

A

Late in pregnancy or several weeks to months postpartum

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15
Q

Possible causes of peripartum cardiomyopathy?

A

– Pregnancy-associated hypertension– volume overload– nutritional deficiency– metabolic derangements– immunological reaction– prolactin

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16
Q

Dilated cardiomyopathy causes cardiomegaly, what’s that?

A

heart is 2-3 times normal weight & globular appearance

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17
Q

Are the valves normal in cardiomyopathy?

A

Yes, however Mitral or tricuspid regurgitation may be present due to dilation of chamber

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18
Q

What are a few other gross morphologies of cardiomyopathy?

A

• Four chamber dilatation• Variable wall thickness( normal, hypertrophy, flabby)• Mural thrombi

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19
Q

What does dilated cardiomyopathy look like on histology?

A

Histologic changes are not specific for DCM (except for iron overload –stainable) Some fibers may appear stretched or irregular; no necrosisHypertrophy and fibrosis are usual

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20
Q

Can iron overload cause dilated cardiomyopathy?

A

Yes, via Hemochromatosis & Multiple transfusions

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21
Q

Increased risk of ldiopathic dilated cardiomyopathy associated with what?

A

– MALE GENDER– BLACK RACE– HYPERTENSION– CHRONIC BETA-AGONIST USE

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22
Q

Clinical presentation of idiopathic dilated cardiomyopathy?

A

• Heart failure symptoms 75%-85%• Anginal chest pain 8%-20%• Emboli (systemic or pulmonary) 1%-4%• Syncope <1%

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23
Q

Prognosis of idiopathic dilated cardiomyopathy?

A

50% of patients die within 2 years, 25% survive longer than 5 yearsDeath due to cardiac failure, arrhythmia or thromboembolic complications

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24
Q

What is Arrhythmogenic Right Ventricular Cardiomyopathy?

A

Inherited autosomal dominant disease of cardiac muscleright ventricular failure and various rhythem disturbances ventricular tachycardia or fibrillationcan lead to sudden death

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25
Q

What is the morphology of Arrhythmogenic Right Ventricular Cardiomyopathy

A

right ventricular wall is severely thinned because of loss of myocytes, with extensive fatty infiltration and fibrosis

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26
Q

• Clinical– Occurs in young adults– Arrhythmias (ventricular tachycardia) – Sudden death – Right ventricular failureWhat is this?

A

Arrhythmogenic right ventricular cardiomyopathy/dysplasia

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27
Q

What is the essential feature of Arrhythmogenic right ventricularcardiomyopathy histology?

A

fatty infiltration

28
Q

What is Hypertrophic cardiomyopathy (HCM)?

A

myocardial hypertrophy, poorly compliant left ventricular myocardium leads to abnormal diastolic filling

29
Q

What happens to the heart wall in HCM?

A

Heart is thick-walled, heavy, and hypercontracting (contrast to flabby heart of DCM)

30
Q

Is diastolic or systolic function affected by HCM?

A

Primarily diastolic dysfunction; systolic function is usually preserved intermittent left ventricular outflow obstruction

31
Q

Describe ventricular hypertrophy.

A

– Disproportionate thickening of the septum
– subaortic region (“Banana”shape)
– No ventricular dilatation, ventricle compressed

32
Q

What happens to the atrium in HCM?

A

Atrium enlarged due to decreased ventricular compliance

33
Q

Describe the histology of HCM.

A

Marked hypertrophy (>40 µm, normal 15 µm)
Interstitial fibrosis
Myofiber disarray

34
Q

What kinds of genetic components can HCM have?

A

Missense mutations in genes encoding sarcomeric proteins with Familial, Autosomal dominant, Variable expression

35
Q

Which sarcomeric proteins are usually deffective?

A

myosin heavy chain β-MHC
myosin binding protein MYBP-C
cardiac TnT

36
Q

How do defects in sarcomeric proteins affect the heart?

A

Defects in direct sarcomere function and transfer of energy to sarcomere

37
Q

Half of HCM pt have left ventricular outflow tract obstruction what is it?

A

Caused in two ways:
narrowed left ventricular outflow tract due to hypertrophied interventricular septum or anterior displacement of mitral valve leaflets during systole

38
Q

Mitral valve regurgitation of anterior leaflets & chordae sucked into the outflow tract during mid-late systole causing obstruction secondary to HCM is called what?

A

Venturi effect

39
Q

Diastolic dysfunction can occur with HCM, how?

A

the myocardium is stiff, non-compliant

  • the left ventricular diastolic pressure is elevated
  • the filling of the ventricle in diastole is impaired
40
Q

Due to the hypertrophy of HCM there can be supply/demand mismatch, impaired relaxation during diastole and abnormal intramyocardial arteries which can lead to what?

A

Myocardial ischemia in the absence significant coronary arteries athersclerosis

41
Q

What arrhythmias can occur as a result of HCM?

A

1 Paroxysmal supraventricular arrhythmias (Occur in 30-50%)
2 Atrial fibrillation
3 Non-sustained ventricular tachycardia
4 Sustained ventricular tachycardia/ventricular fibrillation (lethal in many pt)

42
Q

Over the course of a lifetime, when does HCM usually present?

A

Presentation after puberty

43
Q

Signs and symptoms of HCM?

A

▫ Asymptomatic
▫ Exertional dyspnea due to limitation of cardiac output
▫ Syncope due to left ventricular outflow obstruction
▫ Sudden death in young athletes
▫ Atrial fibrillation with mural thrombus formation

44
Q

Treatment for HCM?

A

surgical excision & ventricular relaxing drugs

45
Q

Risk factors for cardiac death with HCM:

A
  • Marked ventricular wall hypertrophy (>30mm)
  • Young age at presentation (< 40 y)
  • History of syncope
  • History of aborted cardiac arrest
  • Family history of sudden cardiac death
  • Certain genetic mutations
46
Q

Pharm treatments of HCM include:

A

Beta-blockers (first line)
Calcium-channel blockers (in combo or pt who cannot tolerate beta)
Disopyramide

47
Q

Non-pharm therapy includes:

A

Surgical septal myectomy
Alcohol induced septal ablation (infarction of injected area)
Dual-chamber pacemaker (at RV apex)
Heart transplant

48
Q

Implantable cardioverter-defibrillators can help prevent this serious complication?

A

Sudden cardiac death

49
Q

What is restrictive cardiomyopathy?

A

Primary decrease in ventricular compliance,
resulting in impaired ventricular filling during
diastole

50
Q

What are the causes of restrictive cardiomyopathy?

A 
Idiopathic or Secondary to:
– Post-radiation fibrosis
– Amyloidosis 
– Sarcoidosis
– Metastases 
– Inborn errors of metabolism (Fabry’s disease, Pompe’
s disease)
51
Q

Gross anatomy description is of what disease?
– Normal ventricles (size, wall thickness)
– bi-atrial dilation
– firm myocardium
– interstitial fibrosis

A

Restrictive cardiomyopathy

52
Q

What is the histology of Restrictive cardiomyopathy?

A

Patchy or diffuse interstitial fibrosis and disease specific changes

53
Q

Clinical features of restrictive cardiomyopathy?

A

congestive heart failure with severe pulmonary and hepatic congestion

54
Q

What test would you use to diagnose restrictive cardiomyopathy?

A

endomyocardial biopsy

55
Q

Types of amyloidosis of the heart?

A

Senile cardiac amyloidosis: most common

Systemic AA or AL amyloidosis can also involve the heart

56
Q

Causes of senile cardic amyloidosis?

A

– Transthyretin (prealbumin)– deposits in ventricles and atria
– > 60 yrs age; African-Amer vs. Caucasians = 4:1
– Gene mutation in 4% (isoleucine is substituted for valine), autosomal dominant familial form

57
Q

What does amyloidosis of the heart do to the hemodynamics?

A

It varies:
– Asymptomatic
– Pressure atrophy of fibers
– Deposition in regions of conduction system leads to arrhythmias

58
Q

Describe the morphology of amyloidosis of the heart.

A

Heart normal or
firm and rubbery with Eosinophilic
deposits of amyloid

59
Q

Endomyocardial fibrosis, large mural thrombi, peripheral
eosinophilia, eosinophilic infiltrates in organs
Eosinophils release major basic protein which may
cause endomyocardial necrosis and scarring

What cardiomyopathy is this?

A

Loeffler endomyocarditis

60
Q

Most common in first 2 years of life
Disease of children and young adults in Africafocal or diffuse fibroelastic thickening usually involving mural left ventricular endocardium
What cardiomyopathy is this?

A

Endocardial fibroelastosis

61
Q

What are the cardiac effects of iron overload?

A

Heart is usually dilated, rust-brown colored, hemosiderin accumulation, fibrosis may be present

62
Q

Cardiac effects of hyperthyroidism?

A

tachycardia, palpitations, and cardiomegaly

63
Q

Cardiac effects of hypothyroidism?

A

CO decreased, fluid accumulation in pericardial sac, flabby enlarged dilated heart

64
Q

Cardiac histological features of hypothyroidism?

A

myofiber swelling with loss of striations and basophilic degeneration, interstitial mucopolysaccharide-rich edema fluid

65
Q

What is sarcoidosis?

A

Restriction
Conduction System Disease
Ventricular Arrhythmias
(Sudden Cardiac Death)

66
Q

What are a few cardiotoxic drugs?

A

Lithium, phenothiazines, chloroquine and cocaine

67
Q

What is Takotsubo cardiomyopathy and what causes it?

A

Sudden, intense emotional or physical stress leading to acute left ventricular dysfunction which on catecholamines

CV Quiz 2 (16 decks)

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