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IM Cardiology > 287 Cardiomyopathy and Myocarditis > Flashcards

287 Cardiomyopathy and Myocarditis Flashcards

1
Q

It is a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic.

A

Cardiomyopathy

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2
Q

This type of cardiomyopathy presents as:
EF: Usually <30% when symptoms severe

Left ventricular wall thickness: Normal or decreased

Atrial size: Increased

Valvular regurgitation: Related to annular dilation; mitral appears earlier during decompensation; tricuspid regurgitation with right ventricular dysfunction

Common first symptoms: Exertional intolerance

Congestive symptoms: Left before right, except right prominent in young adults

Arrhythmias: Ventricular tachyarrhythmia; conduction block in Chagas’ disease, and some families. Atrial fibrillation.

A

Dilated Cardiomyopathy

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3
Q

This type of cardiomyopathy presents as:
EF: >60%

Left ventricular wall thickness: Markedly increased

Atrial size: Increased; related to elevated filling pressures

Valvular regurgitation: Related to valve-septum interaction; mitral regurgitation

Common first symptoms: Exertional intolerance; may have chest pain

Congestive symptoms: Left-sided congestion at rest may develop late

Arrhythmias: Ventricular tachyarrhythmias; atrial fibrillation

A

Hypertrophic Cardiomyopathy

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4
Q

This type of cardiomyopathy presents as:
EF: 25-50%

Left ventricular wall thickness: Normal or increased

Atrial size: Increased; may be massive

Valvular regurgitation: Related to endocardial involvement; frequent mitral and tricuspid regurgitation, rarely severe

Common first symptoms: Exertional intolerance, fluid retention early, may have dominant right-sided symptoms

Congestive symptoms: Right often dominates

Arrhythmias Ventricular uncommon except in sarcoidosis, conduction block in sarcoidosis and amyloidosis. Atrial fibrillation.

A

Restrictive Cardiomyopathy

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5
Q

Most common protozoan associated with infectious myocarditis?

A

Trypanosoma cruzi

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6
Q

Viruses that can cause infectious myocarditis

A

Coxsackie, Adenovirus, HIV, Hepatitis C

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7
Q

True or False

Systolic failure is more marked than diastolic dysfunction in dilated cardiomyopathy

A

True

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8
Q

It can result from multiple causes but is most commonly attributed to infective agents that can injure the myocardium through direct invasion, production of cardiotoxic substances, or chronic inflammation with or without persistent infection

A

Myocarditis

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9
Q

It facilitates viral replication and infection through degradation of the myocyte protein dystrophin, which is crucial for myocyte stability

A

Enteroviral protease 2A

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10
Q

The initial evaluation for sus- pected myocarditis includes ______

A

ECG, echocardiogram, serum troponin, creatine phosphokinase

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11
Q

True or False

Endomyocardial biopsy is often indicated for the initial evaluation of suspected viral myocarditis

A

False

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12
Q

It is diagnosed when a patient has a typical viral syndrome but no cardiac symptoms, with one or more of the following:
• Elevated biomarkers of cardiac injury (troponin or CK-MB)
• ECG findings suggestive of acute injury
• Reduced left ventricular ejection fraction or regional wall motion • Abnormality on cardiac imaging, usually echocardiography

A

Possible subclinical acute myocarditis

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13
Q

It is diagnosed when the above criteria are met and accompanied also by cardiac symptoms, such as shortness of breath or chest pain, which can result from pericarditis or myocarditis or when clinical findings of pericarditis (pleuritic chest pain, ECG abnormalities, pericardial rub or effusion) are accompanied by elevated troponin or CK-MB or abnormal cardiac wall motion

A

Probable acute myocarditis

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14
Q

It is diagnosed when there is histologic or immunohistologic evidence of inflammation on endomyocardial biopsy (see below) and does not require any other laboratory or clinical criteria

A

Definite myocarditis

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15
Q

True or False

There is currently no specific therapy recommended during any stage of viral myocarditis

A

True

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16
Q

True or False

During acute infection, therapy with anti- inflammatory or immunosuppressive medications is avoided

A

True

  • their use has been shown to increase viral replication and myocardial injury in animal models
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17
Q

It is the third most common parasitic infection in the world and the most common infective cause of cardiomyopathy

A

Chaga’s disease

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18
Q

It is the vector of T. cruzi

A

Reduviid bug

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19
Q

What are the other modes of transmission of T. cruzi?

A

blood transfusion, organ donation, from mother to fetus, and occasionally orally

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20
Q

Typical features of Chaga’s disease?

A

conduction system abnormalities (sinus node and atrioventricular (AV) node dysfunction and right bundle branch block), atrial fibrillation, ventricular tachyarrhythmias, small ventricular aneurysms

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21
Q

Antiparasitic therapy for Chaga’s disease?

A

benznidazole and nifurtimox

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22
Q

West African trypanosomiasis is caused by?

A

Trypanosoma brucei gambiense

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23
Q

East African trypanosomiasis is caused by?

A

T. brucei rhodesiense

24
Q

It is the vector of African trypanosomiasis?

A

tsetse fly

25
Q

This bacillus releases a toxin that impairs protein synthesis and may particularly affect the conduction system.

Almost 1/2 of cases with this infection affects the heart.

Cardiac involvement is the most common cause of death in patients with this infection

A

Diphtheria

26
Q

It is most commonly associated with acute rheumatic fever and is characterized by inflammation and fibrosis of cardiac valves and systemic connective tissue, but it can also lead to a myocarditis with focal or diffuse infiltrates of mononuclear cells

A

Streptococcal infection with beta haemolytic streptococci

27
Q

It can occur due to hematogenous or direct spread of infection from other sites, as has been described for aspergil- losis, actinomycosis, blastomycosis, candidiasis, coccidioidomycosis, cryptococcosis, histoplasmosis, and mucormycosis

A

Fungal myocarditis

28
Q

It is the most commonly diagnosed noninfective inflammatory myocarditis which includes sarcoidosis and giant cell myocarditis

A

Granulomatous Myocarditis

29
Q

It accounts for 10–20% of biopsy-positive cases of myocarditis and typically presents with rapidly progressive heart failure and tachyarrhythmias. Diffuse granulomatous lesions are surrounded by extensive inflammatory infiltrate unlikely to be missed on endo- myocardial biopsy, often with extensive eosinophilic infiltration.

A

Giant cell myocarditis

30
Q

It is often an unexpected diagnosis, made when the biopsy reveals infiltration with lymphocytes and mononuclear cells with a high proportion of eosinophils. Most commonly, the reaction is attributed to antibiotics, particularly those taken chronically, but thiazides, anticonvulsants, indomethacin, and methyldopa have also been implicated.

A

Hypersensitivity myocarditis

31
Q

Treatment for hypersensitivity myocarditis?

A

High-dose glucocorticoids and discontinuation of the trigger agent

32
Q

It develops during the last tri- mester or within the first 6 months after pregnancy, with a frequency between 1:2000 and 1:15,000 deliveries

A

Peripartum cardiomyopathy

33
Q

Risk factors for permpartum cardiomyopathy?

A

Increased maternal age, increased parity, twin pregnancy, malnutrition, use of tocolytic therapy for premature labor, and preeclampsia or toxemia of pregnancy

34
Q

It is the most common toxin implicated in chronic dilated cardiomyopathy

A

Alcohol

35
Q

The alcohol consumption necessary to produce cardiomyopathy in a normal heart_______

A

Five to six drinks (about 4 ounces of pure ethanol) daily for 5–10 years, but frequent binge drink- ing may also be sufficient

36
Q

These are the most common drugs implicated in toxic cardiomyopathy

A

Chemotherapy agents

37
Q

This disease is initially a vasodilated state with very high output heart failure that can later progress to a low output state.

It is due to thiamine deficiency can result from poor nutrition in undernourished populations and in patients deriving most of their calories from alcohol, and has been reported in teenagers subsisting only on highly processed foods.

A

Beri-beri heart disease

38
Q

Deficiency of this element causes Keshan’s disease

A

Selenium

39
Q

It is essential for excitation-contraction coupling.

Chronic deficiency of this element can occur with hypoparathyroidism (particularly postsurgical) or intestinal dysfunction (from diarrheal syndromes and following extensive resection), can cause severe chronic heart failure that responds over days or weeks to vigorous repletion.

A

Calcium

40
Q

It is variably classified as a metabolic or storage disease.

It is included among the causes of restrictive cardiomyopathy, but the clinical presentation is often that of a dilated cardiomyopathy.

A

Hemochromatosis

41
Q

This can also be acquired from iron overload due to hemolytic anemia and transfusions. Excess iron is deposited in the perinuclear compartment of cardiomyocytes, with resulting disruption of intracellular architecture and mitochondrial function.

A

Hemochromatosis

42
Q

Treatment for Hemochromatosis

A

Phlebotomy

Iron chelation therapy with desferrioxamine (deferoxamine) or deferasirox

43
Q

Mutations in this gene, encoding the giant sarcomeric protein titin, are the most common cause of dilated cardiomyopathy, accounting for up to 25% of familial disease.

A

TTN mutations

44
Q

The most recognisable familial cardiomyopathy syndromes with extra cardiac manifestations

A

Muscular dystrophies

Duchenne’s and Becker’s dystrophy

45
Q

Genetic defects in this proteins disrupt myocyte junctions and adhesions, leading to replacement of myocardium by deposits of fat.

It is also associated with striking “woolly hair” and thickened skin on the palms and soles.

A prominent family history of sudden death or ventricular tachycardia before clinical cardiomyopathy suggests genetic defects in this protein.

It is riginally described as affecting the right ventricle (arrhythmogenic right ventricular dysplasia [ARVD])

A

Genetic defects in desmosomal proteins

46
Q

The apical ballooning syndrome, or stress-induced cardiomyopathy, occurs typically in older women after sudden intense emotional or physical stress. The ventricle shows global ventricular dilation with basal contraction, forming the shape of the narrow-necked jar.

Presentations include pulmonary edema, hypotension, and chest pain with ECG changes mimicking an acute infarction.

A

Takotsubo Cardiomyopathy

47
Q

It results from a deficiency of the lysosomal enzyme alpha-galactosidase A caused by one of more than 160 mutations.

Electron microscopy of endomyocardial biopsy tissue shows diagnostic vesicles containing concentric lamellar figures.

A

Fabry’s Disease

48
Q

In this disease, cerebroside-rich cells accumulate in multiple organs due to a deficiency of beta-glucosidase. Cerebroside rich cells infiltrate the heart, which can also lead to a hemorrhagic pericardial effusion and valvular disease.

A

Gaucher’s disease

49
Q

Causes of Restrictive Cardiomyopathy:

Infiltrative Cardiomyopathy

A 
Amyloidosis
Primary (light chain amyloid)
Familial (abnormal transthyretin)a
Senile (normal transthyretin or atrial peptides)
Inherited metabolic defects
50
Q

Causes of Restrictive Cardiomyopathy:

Storage

A

Hemochromatosis (iron)
Inherited metabolic defects
Fabry’s disease
Glycogen storage disease (II, III)

51
Q

Causes of Restrictive Cardiomyopathy:

Fibrotic Cardiomyopathy

A

Radiation

Scleroderma

52
Q

Causes of Restrictive Cardiomyopathy:

Endomyocardial Disease

A 
Possibly related fibrotic diseases
Tropical endomyocardial fibrosis
Hypereosinophilic syndrome (Löffler’s endocarditis)
Carcinoid syndrome
Radiation
Drugs: e.g., serotonin, ergotamine
53
Q

It is defined as left ventricular hypertrophy that develops in the absence of causative hemodynamic factors, such as hypertension, aortic valve disease, or systemic infiltrative or storage diseases.

It has previously been termed hypertrophic obstructive cardiomyopathy (HOCM), asymmetric septal hypertrophy (ASH), and idiopathic hypertrophic subaortic stenosis (IHSS).

A

Hypertrophic cardiomyopathy

54
Q

First line agents that reduce the severity of obstruction by slowing heart rate, enhancing diastolic filling, and decreasing contractility

A

β-Adrenergic blocking agents and L-type calcium channel block- ers (e.g., verapamil)

55
Q

Major Risk Factor Sudden Death in Hypertrophic Cardiomyopathy

A

History of cardiac arrest or spontaneous sustained ventricular tachycardia

Syncope

Family history of sudden cardiac death

Spontaneous nonsus- tained ventricular tachycardia

LV thickness >30 mm

Abnormal blood pressure response to exercise

IM Cardiology (9 decks)

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