Cardiomyopathy, Myocarditis, Pericardial Disease Flashcards by Tom O

What is cardiomyopathy?

Primary abnormality of the myocardium not attributable to pressure or volume overload

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What is the most common cardiomyopathy? Least common?

Most common: Dilated

Least common: Restrictive

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Dilated Cardiomyopathy

What is the dysfunction?

Contractile (SYSTOLIC) dysfunction.

Causes 4 chamber dilatation of the heart and biventricular failure

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What are some causes of Dilated Cardiomyopathy?

Myocarditis
Peripartum Cardiomyopathy
Toxic (alcohol, doxorubicin)
Idiopathic
Genetic

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Genetic disorders affecting what cellular component may cause dilated cardiomyopathy?

Cytoskeleton of the myocytes

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What are long term complications of dilated cardiomyopathy?

Progressive systolic CHF
Arrhythmias
Mural thrombi with emboli

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Hypertrophic Cardiomyopathy is AKA…

IHSS = idiopathic hypertrophic subaortic atenosis
HOCM = Hypertrophic obstructive cardiomyopathy

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Hypertrophic Cardiomyopathy

What is the dysfunction?

Marked LV myocardial hypertrophy (particularly in the septum), leading to abnormal DIASTOLIC filling and diastolic HF

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Which part of the ventricular wall is typically hypertrophied in hypertrophic cardiomyopathy?

Septum

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Which cardiomyopathy leaves a “banana shaped LV cavity?”

Hypertrophic Cardiomyopathy

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Hypertrophic Cardiomyopathy

What do the myocytes look like histologically?

Myocytes are hypertrophied and haphazard.

Commonly see interstitial fibrosis

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What is the etiology of Hypertrophic Cardiomyopathy?

Most cases are genetic (familial)

Autosomal dominant

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Where do most genetic mutations causing Hypertrophic Cardiomyopathy effect the cell?

Most mutations are in genes encoding proteins in the sarcomere

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Long Term Complications and Risks of Hypertrophic Cardiomyopathy

Diastolic HF
High risk for ventricular and atrial arrhythmias (most common cause of sudden death in young athletes)
Exertional dyspnea
Anginal pain

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Hypertrophic Cardiomyopathy

Treatment

Medical therapy (beta blockers) to enhance relaxation of ventricle

Surgical excision of muscle (septum)

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Restrictive Cardiomyopathy

What is the primary dysfunction?

Decrease in ventricular compliance, impeding LV filling during diastole

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Causes of Restrictive Cardiomyopathy

Idiopathic
Radiation Therapy
Sarcoidosis (non caseating granulomas developing in the heart)
Amyloidosis

What is an amyloid?

Abnormally folded protein that begins to deposit into extracellular spaces, causing tissue damage

In what secondary protein structure are amyloids arranged?

Beta pleated sheets

What stain can be used to identify amyloids? How would amyloid appear under normal light with this stain? Under polarized light?

Congo Red Stain
Appears salmon pink under normal light.
Appears apple-green under polarized light

What is myocarditis?

Inflammatory process resulting in myocardial injury

What is the most common cause of myocarditis?

Viral infection, particularly Coxsackie A and B
Cytomegalovirus and HIV also can be causes

Can also be cause by bacteria (diptheria, Lyme) or parasites (Chagas)

What are some noninfectious causes of myocarditis?

Immune mediated (hypersensitivity, rheumatic fever)
Giant cell myocarditis
Sarcoidosis

What are the clinical manifestations of myocarditis?

Wide spectrum, could be asymptomatic or nonspecific (fever, fatigue).
Acute congestive heart failure
Arrhythmias
Progression to dilated cardiomyopathy

Pericardial Effusion | What might you see in a pericardial effusion in the case of trauma?

Bloody pericardial effusion

Pericardial Effusion | What might you see in a pericardial effusion in the case of an infection?

Pus (dead PMNs)

Pericardial Effusion | What might you see in a pericardial effusion in the case of serious heart failure?

Yellow, clear serous fluid

Above what volume will a pericardial effusion become clinically significant?

500 mL Note: a rapid increase in fluid, even below 500mL, may still cause hemodynamic instability

As a pericardial effusion grows, what happens?

First, you get compression of the atria and the vena cavae. Soon, you gte compression fo the ventricles, leading to less filling of the ventricles during diastole and decreased outflow

What is pericarditis?

Inflammation of the pericardium, usually secondary to another problem

What is fibrinous pericarditis? What might you hear on auscultation? How will the pericardial surface appear?

Lots of fibrin deposited in the pericardium Hear a pericardial friction rub The surface will appear irregular and shaggy

What might cause hemorrhagic pericarditis?

Tuberculosis | Malignancy

What might cause caseous pericarditis?

Tuberculosis

What might cause suppurative pericarditis?

Bacterial infection

What are the symptoms of pericarditis?

Could be silent Chest Pain Systemic complaints Friction rub heard on auscultation EKG Changes -- diffuse ST segment elevation

Healing of the pericardium in pericarditis can lead to what?

CONSTRICTIVE pericarditis | Heart surrounded by a dense scar, limiting diastolic expansion of the heart

How can one treat constrictive pericarditis?

Pericardiectomy

Which cardiomyopathies may be treated by heart transplant?

Dilated cardiomyopathy | Ischemic cardiomyopathy

What are some complications of heart transplant?

Need chronic immunosuppressive therapy Higher risk of infection Acute and chronic rejection EBV infection could lead to lymphoma or malignancy Aggressive intimal thickening of the coronary arteries

How are heart biopsies obtained?

Enter through a large vein, travel through the SVC, go into the RA, them RV, biopsy the SEPTUM. Do not biopsy the RV free wall because that is thinner and you may puncture it