MHD: Cardiomyopathy Flashcards

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1
Q

What is a cardiomyopathy?

A

A primary abnormality of the myocardium not attributable to pressure or volume overload. It involves a progressive impairment of the structure and function of the muscular walls of the heart chambers.

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2
Q

What are the 3 main types of cardiomyopathy?

A

Dilated
Hypertrophic
Restrictive

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3
Q

Which type of cardiomyopathy is most common?

A

Dilated cardiomyopathy

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4
Q

Describe the morphological changes seen in dilated cardiomyopathy

A

Biventricular dilatation causes contractile dysfunction

The myocardium compensates for the dilation with hypertrophy. Interstitial fibrosis can also develop.

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5
Q

Proteins of the ________ are involved with genetic forms of dilated cardiomyopathy

A

Proteins of the CYTOSKELETON are involved with genetic forms of dilated cardiomyopathy

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6
Q

What are the non-genetic causes of dilated cardiomyopathy?

A

Myocarditis
Peripartum (due to elevated PRL)
Toxic
Idiopathic

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7
Q

What are the clinical consequences of cardiomyopathy?

A

Heart failure
Sudden death
Atrial fibrilation
Stroke

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8
Q

What is hypertrophic cardiomyopathy?

A

Marked LV hypertrophy (septum>free wall)

AKA: IHSS, hypertrophic obstructive cardiomyopathy

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9
Q

How are diastole and systole affected by dilated cardiomyopathy compared to hypertrophic cardiomyopathy?

A

Systole is affected by dilated cardiomyopathy

Diastole is affected by hypertrophic cardiomyopathy

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10
Q

What is the classical shape of the ventricle in hypertrophic cardiomyopathy?

A

Banana shaped due to an enlarged intraventricular septum

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11
Q

Describe the histology of hypertrophic cardiomyopathy

A

Myocytes are hypertrophied and appear haphazardly organized. Interstitial fibrosis can also be seen.

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12
Q

What is the major cause of hypertrophic cardiomyopathy?

A

Most cases are familial

Autosomal dominant mutation in gene encoding sarcomeric proteins

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13
Q

Mechanically, dilated cardiomyopathy is a defect in ________ whereas hypertrophic cardiomyopathy is a defect in _________

A

Mechanically, dilated cardiomyopathy is a defect in FORCE GENERATION whereas hypertrophic cardiomyopathy is a defect in ENERGY TRANSFER

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14
Q

Clinical outcome of hypertrophic cardiomyopathy

A
Diastolic heart failure
Exertional dyspnea
Harsh systolic ejection murmur
Anginal pain
Intractable heart failure
Arrhythmias
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15
Q

What is the most common cause of sudden death in young athletes?

A

Hypertrophic cardiomyopathy

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16
Q

What is the treatment for hypertrophic cardiomyopathy?

A

Medications to enhance ventricular contraction (beta blockers, calcium channel blockers)
Surgical excision of muscle

17
Q

What is restrictive cardiomyopathy?

A

A primary decrease in ventricular compliance prevents ventricular filling (expansion) during diastole (systolic function is preserved)

18
Q

Describe the morphological changes seen in restrictive cardiomyopathy

A

Enlarged left atrium with are normal LV cavity size, slightly thickened LV wall

19
Q

What are the causes of restrictive cardiomyopathy?

A
Radiation fibrosis
Amyloidosis
Sarcoidosis
Inborn errors of Metabolism
Endocardial fibroelastosis
Loeffler endomyocarditis
20
Q

What is an amyloid?

A

A misfolded protein that desposits in the extracellular space causing tissue damage

21
Q

What are the common features of amyloid deposits?

A

Beta pleated sheet configuration

Stain congo red in tissue that appears apple-green under polarized light

22
Q

What is myocarditis?

A

Inflammation of the myocardium that causes myocardial injury

23
Q

What are the causes of myocarditis?

A

Viral (Coxsakie A and B, cytomegalovirus, HIV)
Bacterial (Diptheria, Lyme disease)
Parasitic (Chaga’s disease, trichinosis, toxoplasmosis)
Noninfectious (Immune hypersensitivity, rheumatic fever, giant cell myocarditis, sarcoidosis)

24
Q

What is the clinical manifestation of the myocarditis?

A

Wide spectrum

Can cause acute congestive heart failure, arrhythmias and can progress to dilated cardiomyopathy

25
Q

What liquids can be involved with pericardial effusion?

A

Serous fluid (clear or yellow)
Blood
Pus

26
Q

How does the timing of pericardial effusion affect the clinical outcome?

A

Slowly developing may be clinically silent
Rapid or large effusions can compress the atria and vena cava (or ventricles in severe cases), leading to decreased cardiac filling

27
Q

What is pericarditis?

A

Inflammation of the pericardium usually secondary to cardiac, thoracic or systemic process

28
Q

What are the causes of pericarditis?

A

Infections (viruses, bacteria, TB, fungi, parasites)
Immune-mediated (rheumatic fever, SLE, post-MI)
Uremia
Neoplasia
Trauma
Radiation

29
Q

What is fibrinous pericarditis?

A

“Bread and butter” pericarditis
The pericardial surface appears shaggy due to fibrinous exudate
Exam finding: pericardial friction rub

30
Q

What is the cause of suppurative pericarditis?

A

Acute bacterial infection can lead to purulent surface of the percardium

31
Q

What can cause hemorrhagic pericarditis?

A

Tuberculosis

Malignancy

32
Q

What can cause caseous pericarditis?

A

Tuberculosis

33
Q

Describe the presentation of pericarditis

A

Can be silent, or cause chest pain, systemic complaints
Friction rub is often found on physical exam
EKG changes: diffuse ST elevation

34
Q

Describe the healing process of pericarditis

A

Focal plaque like thickenings
Mild adhesions
Constrictive pericarditis can cause the heart to be surrounded by a dense scar, which prevents expansion

35
Q

What is the treatment of constrictive pericarditis?

A

Surgical removal of the scarred, constrictive pericardium

36
Q

What are the complications of cardiac transplantation?

A

Acute or chronic rejection
Infections
Post tranplant lymphoma
Late progressive diffuse stenosing of coronary arteries

37
Q

What is the success rate of cardiac transplantation?

A

70-80% 1 year survival

>60% 5 year survival

38
Q

How are heart biopsies obtained?

A

A bioptome (biopsy tool) is inserted transvenously into the right side of the heart and the biopsy is taken from the septum