Path-myocarditis, pericarditis, vasculitis

Question 1

In myocarditis, inflammation is the (cause/response) of the injury

Answer 1

Cause

Question 2

Myocarditis

Active phase

Answer 2

Interstitial inflammatory infiltrate
Focal myocyte necrosis
+/- myocyte hypertrophy
Diffuse mononuclear infiltrate is most common (mostly lymphocytes)

Question 3

In acute myocarditis, numerous ____ are associated with mononuclear cell infiltrates, including ___ whereas in chronic myocarditis, inflammatory cells such as ___ are mainly present in areas with ___.

Answer 3

1. Necrotic myocytes
2. CD3+ T cells
3. CD68+ macrophages
4. Fibrosis

Question 4

In the chronic phase of myocarditis, ___ resolves and one of these develops:

Answer 4

Inflammation

1. Resolution of lesions
2. Progressive fibrosis

Question 5

10 Most common viral causes of myocarditis

Answer 5

1. Parvovirus B19
2. HHV6
3. Coxsackie A and B
4. Other enteroviruses
5. Hep C
6. Adenovirus
7. Echovirus
8. CMV
9. HIV
10. Influenza A and B

Question 6

Most common bacterial causes of myocarditis

Answer 6

C. diphtheria
N. meningitidis
Borrelia burgdorferi (Lyme disease)
Ehrlichia chaffeensis

Question 7

The leading cause of dilated cardiomyopathy in Central and South America is ___. Myocarditis occurs in the majority of these infected patients. What is pathognomonic for this disease?

Answer 7

T. cruzi (Chagas disease)

Left ventricular apical aneurysms

Question 8

The most common helminth to cause myocarditis is ___

Answer 8

Trichinella spiralis (undercooked or raw pork, or wild game)

Question 9

poststreptococcal myocarditis (rheumatic fever) results from immune responses to ___ strep. Antibodies directed against ___ proteins of strep cross-react with self-antigens in the heart. ___ cells specific for strep peptides react with self-antigens and produce ____. RF typicallay arises ____ after strep pharyngitis infections.

Answer 9

1. Group A
2. M
3. CD4+ T
4. Cytokines that activate macrophages
5. 10 days-6 weeks

Question 10

____ are composed of lymphocytes, few plasma cells and activated macrophages (called ___) which are pathognomonic for RF and have round-oval nuclei with chromatin dispersed around a central wavy ribbon (hence the nickname “___”)

Answer 10

1. Aschoff bodies
2. Anitschkow cells
3. Caterpillar cells

Question 11

Diffuse inflammation and Aschoff bodies may be found in the ____ during acute RF. This is called ___

Answer 11

1. Endo-, myo-, or pericardium

2. Pancarditis

Question 12

Myocarditis with many non-necrotizing epithelioid granulomas

Negative for organisms

Answer 12

Sarcoidosis

Question 13

Rapid onset of chest pain, fever; and hemodynamic compromise
Often with V tach or AV block
Unknown cause
Poor prognosis
Often fatal without transplant
Widespread inflammatory infiltrate with lymphocytes, plasma cells, macrophages, eosinophils, and multinucleated giant cells

Answer 13

Giant cell myocarditis

Question 14

Sudden intense emotional or physical stress due to production of catecholamines, which cause myocardial stunning

Answer 14

Pheochromocytoma

Question 15

Diagnosis of myocarditis includes:

Answer 15

ECG
Cardiac biomarkers
CXR
Labs often normal
Recommend testing BNP or NT-proBNP if HF is suspected

Question 16

Differential dx for myocarditis

Answer 16

Ischemic or valvular heart disease
Congenital heart disease
Pulmonary disease

*echo is helpful in distinguishing

Question 17

True or false:
Cardiomyopathies are major structural abnormalities that are limited to the myocardium and are secondary to ischemia, valvular disease, or hypertensive heart disease.

Answer 17

False. First half is true. But they are NOT secondary to any of those

Question 18

3 types of cardiomyopathies

Answer 18

1. Dilated (DCM)
2. Hypertrophic (HCM)
3. Restrictive (RCM)

Question 19

Definition of DCM

Answer 19

Dilation and impaired contraction of one or both ventricles, with systolic dysfunction.

Question 20

Most patients with DCM present between the ages of ___. Symptoms vary

Answer 20

20-60

Question 21

Causes of DCM

Answer 21

1. Idiopathic (~50%)
2. Infectious myocarditis
3. Infiltrative disease (sarcoidosis, carcinoma)
4. Periosteum state
5. Toxins (ethanol, cocaine, cobalt, anthracyclines)
6. CT disease
7. Metabolic disorders
8. End-stage renal disease
9. Genetic abnormalities

Question 22

Gross morphology of DCM

Answer 22

Heart is large and flabby
Poor contractile function
Valves and coronary arteries are usually normal

Question 23

Prognosis of DCM

Answer 23

Indolent and progressive CHF

Fewer than 50% survive more than 5 yrs after onset of symptoms unless they receive a transplant

Question 24

HCM is characterized by __ and is not caused by ___

Answer 24

Unexplained septal or LV hypertrophy that is NOT caused by chronic pressure overload (not caused by systemic HTN, congenital heart defects, or aortic stenosis)

Question 25

Gross morphology of the heart in HCM

Answer 25

``` Heavy Muscular Hypercontractile Stiff Poor diastolic relaxation Ventricular outflow obstruction is seen in 1/3 of cases Asymmetric thickening of IV septum Banana like shape of LV cavity ```

Question 26

Microscopic morphology of HCM

Answer 26

1. Cardiac myocyte hypertrophy or disarray 2. Enlarged hyperchromatic nuclei 3. Interstitial replacement fibrosis of myocardium

Question 27

A majority of HCM cases involve a mutation of the ___ gene, typically with ___ inheritance. Other genes involved encode ___ and ___

Answer 27

1. Myosin heavy chain 2. Autosomal dominant 3. Troponin T and myosin binding protein C

Question 28

Symptoms of HCM

Answer 28

- most patients are asymptomatic - sometimes v fib and sudden cardiac death - symptomatic progression is usually slow - symptomatic disease usually presents in young adults with dyspnea, angina, and/or syncope

Question 29

Complications of HCM

Answer 29

1. A fib 2. Mural thrombus 3. Embolization 4. Congestive heart failure 5. Infectious endocarditis 6. Sudden cardiac death

Question 30

___ is the most common cause of sudden cardiac death in young athletes

Answer 30

HCM

Question 31

RCM is characterized by __

Answer 31

Abnormally rigid ventricles that are usually of normal thickness and are not dilated. Impaired diastolic filling Systolic function is usually normal Non-specific interstitial myocardial fibrosis

Question 32

RCM usually results from ___

Answer 32

Either fibrosis of the endomyocardium or infiltration of the myocardium by an abnormal substance

Question 33

___ is the most common cause of RCM in non tropical countries

Answer 33

Amyloidosis

Question 34

Mainly a disease of children and young adults in tropical regions. Fibrosis of ventricular endocardium and subendocardium

Answer 34

Endomyocardial fibrosis

Question 35

Associated with peripheral blood eosinophilia and eosinophilic infiltration of organs. Endomyocardial fibrosis, typically with large mural thrombus. Can be rapidly fatal

Answer 35

Loeffler endocarditis

Question 36

Endocardial fibroelastosis is most common in ___

Answer 36

Children under 2

Question 37

Other cardiomyopathies

Answer 37

1. Arrhythmogenic right ventricular CM 2. Stress-induced CM - transient left ventricular apical ballooning, takotsubo CM, and broken heart syndrome CM

Question 38

True or false: | Arrhythmogenic right ventricular CM is autosomal recessive

Answer 38

False. Autosomal dominant

Question 39

Gross morphology of Arrhythmogenic right ventricular CM

Answer 39

1. RV wall is thinned due to loss of myocytes 2. Extensive fatty infiltration 3. Fibrosis

Question 40

Acute coronary syndrome, usually with ST elevation, with focal systolic dysfunction of the LV, occurring in the absence of critical CAD

Answer 40

Stress-induced CM

Question 41

Stress-induced CM occurs mostly in ___

Answer 41

Older women

Question 42

Symptoms of Stress-induced CM

Answer 42

``` Acute substernal chest pain (most common) Dyspnea Syncope HF Arrhythmias Cardiogenic shock ```

Question 43

Lab findings for Stress-induced CM

Answer 43

Elevated troponin Normal or slightly elevated CK BNP or NT-proBNP usually elevated

Question 44

Prognosis of Stress-induced CM

Answer 44

Even in severe cases, almost all patients recover within 1-4 weeks

Question 45

___ and ___ are the most common pericarditis

Answer 45

Fibrinois and serofibrinous

Question 46

Caseous pericarditis is ___ until proven otherwise

Answer 46

Tuberculosis

Question 47

___ obliterates the pericardial sac and leaves the parietal layer tethered to mediastinal tissue. ___ is marked by thick, dense, fibrous obliteration of the pericardial sac, often with calcification. This limits diastolic expansion and restricts CO

Answer 47

Adhesive mediastinopericarditis | Constrictive pericarditis

Question 48

In pericardial effusions, inflammation typically results in ___ while non-inflammatory conditions typically result in ___

Answer 48

Exudates | Transudates

Question 49

Chylous effusions occur in response to ___

Answer 49

Lymphatic obstruction of pericardial drainage by tuberculosis or a tumor

Question 50

Transudate vs. exudate

Answer 50

``` Transudates: clear low cell counts specific gravity <1.015 protein <3 g/dL Fluid:serum protein ratio <0.5 Fluid:serum LDH ratio <0.6 Fluid:serum glucose ratio >1 ``` *Exudates are all opposite

Question 51

The most common cause of pericardial effusion is ___

Answer 51

Congestive heart failure

Question 52

Too much fluid causes restriction of heart motion, leading to ___, where pericardial fluid accumulates under high pressure, compresses the cardiac chambers, and severely limits filling. This can lead to hypotensive shock and death

Answer 52

Cardiac tamponade

Question 53

Takayasu arteritis is vasculitis of ___ vessels while Kawasaki disease is vasculitis of ___ vessels. What is Wegener's disease?

Answer 53

Large Medium Granulomatis is with polyangiitis (small vessels)

Question 54

The two most common pathogenic mechanisms of vasculitis are ___ and ___

Answer 54

Immune-mediated inflammation and direct invasion of vascular walls by pathogenic organisms

Question 55

Non-infectious vasculitis is mainly mediated by ___

Answer 55

Immune complex

Question 56

What is the most common form of vasculitis in the elderly? | Which is most commonly seen in young Asian or African females?

Answer 56

Temporal (Giant Cell) Arteritis | Takayasu Arteritis

Question 57

Chronic vasculitis of medium to large sized arteries. It is a systemic disease that mainly involves the cranial vessels, but can involve the aortic arch and its branches

Answer 57

Temporal (Giant Cell) Arteritis | Giant Cell aortitis

Question 58

Etiology for Temporal Arteritis

Answer 58

Immune response against vascular wall antigens

Question 59

Morphology of Temporal Arteritis

Answer 59

Patchy, medial granulomatous inflammation centered on the internal elastic lamina of medium-small arteries

Question 60

Chronic granulomatous vasculitis of unknown etiology that involves the aorta and its major branches

Answer 60

Takayasu Arteritis

Question 61

Progression of Takayasu Arteritis is characterized by ___

Answer 61

Ocular disturbances, neurological symptoms, and upper extremity pulselessness (pulseless disease)

Question 62

How can you distinguish between Takayasu Arteritis and Giant Cell Arteritis histologically?

Answer 62

You can't

Question 63

Systemic disease characterized by necrotizing vasculitis of small-medium sized arteries of kidney, heart, liver, and GI tract. Not pulmonary circulation

Answer 63

PAN | Polyarteritis Nodosa

Question 64

PAN usually affects ____. It is fatal if untreated but with treatment 90% may remit or be cured

Answer 64

Middle aged and older adults, but can affect any age

Question 65

1/3 of PAN cases are associated with ___ and ____, the rest are ___. There is no association with ____.

Answer 65

1. Chronic hep B 2. immune complex deposition 3. Idiopathic 4. ANCA

Question 66

Acute, febrile, usually self-limited illness of infancy and childhood. Results from arteritis affects my large to medium sized or even small vessels. May follow a viral illness

Answer 66

Kawasaki disease aka | Mucocutaneous lymph node syndrome

Question 67

Kawasaki disease primarily affects ___

Answer 67

Children under 4 (80%)

Question 68

Kawasaki disease is the most common cause of ___

Answer 68

Acquired heart disease in children in the U.S.

Question 69

Treatment for Kawasaki disease

Answer 69

Aspiring | IV gamma globulin

Question 70

Necrotizing vasculitis of Arteritis les, capillaries, and venules; unlike PAN, all lesions are at the same stage and are distributed more widely

Answer 70

Microscopic polyangitis

Question 71

Necrotizing granulomas of upper resp tract, lungs, or both. Of medium or small vessels, often crescentic, glomerulonephritis. Clinically resembles PAN

Answer 71

Granulomatosis with Polyangitis aka | Wegeners Granulomatosis

Question 72

Granulomatosis with Polyangitis aka | Wegeners Granulomatosis commonly affects ___

Answer 72

Males over 40

Question 73

If untreated, ___% of people with Granulomatosis with Polyangitis die within 1 year.

Answer 73

80%

Question 74

90% of cases of active generalized Granulomatosis with Polyangitis have ___ and 60% of limited disease have ____. ____ is usually negative, unless there is ____

Answer 74

c-ANCA (PR3-ANCA) c-ANCA P-ANCA Crescentic glomerulonephritis

Question 75

Acute and chronic segmental thrombosing inflammation of medium m and small vessels (mainly tibial and radial) with occasional extension into veins and nerves of extremities

Answer 75

Thromboangiitis obliterans aka | Buergers disease

Question 76

Thromboangiitis obliterans is most common in ___

Answer 76

Far and Middle East, strong association with cigarettes, almost exclusively in young adults (usually under 35)

Question 77

Paroxysmal cyanosis of the extremities, particularly the fingers and toes; also tip of nose, earlobes, or lips. Exaggerated vasoconstriction of arteries or arterioles.

Answer 77

Raynaud phenomenon

Question 78

Which disease is described as patriotic? Why?

Answer 78

Raynaud phenomenon red, white, and blue color change from most proximal to distant, correlating with proximal vasodilation, central vasoconstriction, and distal cyanosis

Question 79

Primary Raynaud Phenomenon

Answer 79

Young women Symmetrically affects extremities Severity is static over time Usually benign Chronicity can lead to atrophy of skin, subcutaneous tissues, and muscle Ulceration and ischemic gangrene are rare

Question 80

Secondary Raynaud Phenomenon

Answer 80

Asymmetric involvement of extremities Progressively worsens Ulceration and ischemic gangrene are more common