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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
What is the morphology of Arrhythmogenic Right Ventricular Cardiomyopathy
right ventricular wall is severely thinned because of loss of myocytes, with extensive fatty infiltration and fibrosis
26
• Clinical– Occurs in young adults– Arrhythmias (ventricular tachycardia) – Sudden death – Right ventricular failureWhat is this?
Arrhythmogenic right ventricular cardiomyopathy/dysplasia
27
What is the essential feature of Arrhythmogenic right ventricularcardiomyopathy histology?
fatty infiltration
28
What is Hypertrophic cardiomyopathy (HCM)?
myocardial hypertrophy, poorly compliant left ventricular myocardium leads to abnormal diastolic filling
29
What happens to the heart wall in HCM?
Heart is thick-walled, heavy, and hypercontracting (contrast to flabby heart of DCM)
30
Is diastolic or systolic function affected by HCM?
Primarily diastolic dysfunction; systolic function is usually preserved intermittent left ventricular outflow obstruction
31
Describe ventricular hypertrophy.
– Disproportionate thickening of the septum – subaortic region (“Banana”shape) – No ventricular dilatation, ventricle compressed
32
What happens to the atrium in HCM?
Atrium enlarged due to decreased ventricular compliance
33
Describe the histology of HCM.
Marked hypertrophy (>40 µm, normal 15 µm) Interstitial fibrosis Myofiber disarray
34
What kinds of genetic components can HCM have?
Missense mutations in genes encoding sarcomeric proteins with Familial, Autosomal dominant, Variable expression
35
Which sarcomeric proteins are usually deffective?
myosin heavy chain β-MHC myosin binding protein MYBP-C cardiac TnT
36
How do defects in sarcomeric proteins affect the heart?
Defects in direct sarcomere function and transfer of energy to sarcomere
37
Half of HCM pt have left ventricular outflow tract obstruction what is it?
Caused in two ways: narrowed left ventricular outflow tract due to hypertrophied interventricular septum or anterior displacement of mitral valve leaflets during systole
38
Mitral valve regurgitation of anterior leaflets & chordae sucked into the outflow tract during mid-late systole causing obstruction secondary to HCM is called what?
Venturi effect
39
Diastolic dysfunction can occur with HCM, how?
the myocardium is stiff, non-compliant - the left ventricular diastolic pressure is elevated - the filling of the ventricle in diastole is impaired
40
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?
Myocardial ischemia in the absence significant coronary arteries athersclerosis
41
What arrhythmias can occur as a result of HCM?
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
Over the course of a lifetime, when does HCM usually present?
Presentation after puberty
43
Signs and symptoms of HCM?
▫ 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
Treatment for HCM?
surgical excision & ventricular relaxing drugs
45
Risk factors for cardiac death with HCM:
* 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
Pharm treatments of HCM include:
Beta-blockers (first line) Calcium-channel blockers (in combo or pt who cannot tolerate beta) Disopyramide
47
Non-pharm therapy includes:
Surgical septal myectomy Alcohol induced septal ablation (infarction of injected area) Dual-chamber pacemaker (at RV apex) Heart transplant
48
Implantable cardioverter-defibrillators can help prevent this serious complication?
Sudden cardiac death
49
What is restrictive cardiomyopathy?
Primary decrease in ventricular compliance, resulting in impaired ventricular filling during diastole
50
What are the causes of restrictive cardiomyopathy?
``` Idiopathic or Secondary to: – Post-radiation fibrosis – Amyloidosis – Sarcoidosis – Metastases – Inborn errors of metabolism (Fabry’s disease, Pompe’ s disease) ```
51
Gross anatomy description is of what disease? – Normal ventricles (size, wall thickness) – bi-atrial dilation – firm myocardium – interstitial fibrosis
Restrictive cardiomyopathy
52
What is the histology of Restrictive cardiomyopathy?
Patchy or diffuse interstitial fibrosis and disease specific changes
53
Clinical features of restrictive cardiomyopathy?
congestive heart failure with severe pulmonary and hepatic congestion
54
What test would you use to diagnose restrictive cardiomyopathy?
endomyocardial biopsy
55
Types of amyloidosis of the heart?
Senile cardiac amyloidosis: most common | Systemic AA or AL amyloidosis can also involve the heart
56
Causes of senile cardic amyloidosis?
– 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
What does amyloidosis of the heart do to the hemodynamics?
It varies: – Asymptomatic – Pressure atrophy of fibers – Deposition in regions of conduction system leads to arrhythmias
58
Describe the morphology of amyloidosis of the heart.
Heart normal or firm and rubbery with Eosinophilic deposits of amyloid
59
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?
Loeffler endomyocarditis
60
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?
Endocardial fibroelastosis
61
What are the cardiac effects of iron overload?
Heart is usually dilated, rust-brown colored, hemosiderin accumulation, fibrosis may be present
62
Cardiac effects of hyperthyroidism?
tachycardia, palpitations, and cardiomegaly
63
Cardiac effects of hypothyroidism?
CO decreased, fluid accumulation in pericardial sac, flabby enlarged dilated heart
64
Cardiac histological features of hypothyroidism?
myofiber swelling with loss of striations and basophilic degeneration, interstitial mucopolysaccharide-rich edema fluid
65
What is sarcoidosis?
Restriction Conduction System Disease Ventricular Arrhythmias (Sudden Cardiac Death)
66
What are a few cardiotoxic drugs?
Lithium, phenothiazines, chloroquine and cocaine
67
What is Takotsubo cardiomyopathy and what causes it?
Sudden, intense emotional or physical stress leading to acute left ventricular dysfunction which on catecholamines

CV Quiz 2 (16 decks)

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