Cardiac Pathology

Question 1

What is the caveat when dating myocardial infarctions?

Answer 1

If the patient has had ANY type of reperfusion therapy, you can no longer accurately date the infarct

Question 2

BQ! If you see a heart slide showing giant cells, lymphocytes and eosinophils, what should be in your differential OTHER than sarcoidosis?

Answer 2

Giant cell myocarditis aka Fieldler’s myocarditis

Rapidly progressing disease of young adults, immediate need for transplant

Other differential is hypersensitivity myocarditis secondary to methyldopa (high yield!)

Question 3

What type of myocarditis is being shown here and how can you tell?

Answer 3

Rheumatic myocarditis which is a pancarditis

The picture is showing Anitschkow cell from an Aschoff nodule—

fibrinoid necrosis of heart muscle
– Anitschkow cell (histiocytes with condensed chromatin give caterpillar like appearance)
– Aschoff cells (MN giant cells)
– Collagenolysis

Question 4

What uncommon disorder is characterized by markedly thin RV replaced by fat?

Answer 4

Arrhythmogenic RV Dysplasia

Question 5

What is molecular event seen in 50% of hypertrophic cardiomyopathy?

Answer 5

Defect in ß myosin heavy chain on
chromosome 14 (50%)

Question 6

What disease is this? (instant recognition!)

Answer 6

Fabry’s disease

X-linked recessive form of sphingolipidosis due to
deficiency of alphagalactosidase A
Angiokeratomas, renal insufficiency & cardiac failure
Histology: vacuolated myocytes

EM shows intralysosomal dense lamallae with concentric
or packed arrangement (Zebra bodies)

Question 7

If stem gives a person who is taking Adriamycin, what is the next step in management?

Answer 7

GET EM!

Grading scheme based on sarcotubular dilatation
(vacuolization) and myofibrillar loss

Question 8

Where do cardiac myxomas occur?

Answer 8

75% in the left atrium

Question 9

What cardiac tumor is being shown?

Answer 9

Rhabdomyoma

MOST COMMONLY IN LEFT VENTRICLE

Most common primary cardiac tumor of
infancy and childhood

May be sporadic, but >50% associated with TS
Eosinophilic, polygonal cells with large glycogen-rich cytoplasmic vacuoles with stranding “spider cells”

Question 10

What is a McCallum’s patch?

Answer 10

Endocardial inflammation in acute rheumatic heart disease
Endocardium becomes white (scar)

Question 11

What is Libman Sacks endocarditis?

Answer 11

seen in lupus erythematosus
Vegetations covering both sides of valve surfaces
Fibrinoid necrosis & inflammation

Question 12

Alterations in the expression of miRNAs (micro RNAs) is associated with which pathologic process in the heart?

Answer 12

Cardiac hypertrophy

Question 13

What is the most common congenital heart malformation?

Answer 13

VSD

Question 14

What is tetralogy of Fallot?

Answer 14

VSD with an obstruction of the right ventricular outflow track known as subpulmonary stenosis, an overriding aorta, and right ventricular hypertophy

Question 15

What cardiac manifestation is most likely to develop secondary to carcinoid?

Answer 15

Tricuspid valve insufficiency

Question 16

What syndrome has arrhythmogenic right ventricle cardiomyopathy and hyperkeratosis of the palmer and plantar skin surfaces and is from a mutation in the plakoglobin gene?

Answer 16

Naxos syndrome

Question 17

What are the large, medium and small vessel vasculitides?

Answer 17

Large: Giant Cell Arteritis and Takayasu’s

Medium: polyarteritis nodosa and Kawasaki’s

Small: Wegener’s, Churg Strauss and microscopic polyangiitis

Question 18

What cardiac tumor is positive for calretinin?

Answer 18

Cardiac myxoma

Other calretinin + tumors:

Adenomatoid tumor

Sex-cord stromal tumors

Adrenocortical tumors

Mesothelioma

Question 19

What is the mutation in Carney syndrome (cardiac myxomas, endocrine issues, etc)?

Answer 19

Autosomal dominant mutation of PRKAR1A (protein kinase A)

Myxoid lesions: cardiac myxoma, skin angiomyxoma, myxoid fibroadenoma of the breast

Pigmented and calcifying lesions: spotty skin pigmentation, epithelioid blue nevus, pigmented nodular adrenocortical hyperplasia, psammomatous melanotic schwannoma, large cell calcifying sertoli cell tumor

Endocrine hyperactivity: pituitary adenoma, chondroid hamartoma

Question 20

BQ SLIDE!

What is this lesion in the heart?

Answer 20

Rheumatic Heart Disease

Picture is showing the characteristic Aschoff nodule composed of Anitschkow cells (seen here)

Question 21

This tumor shown:

• Second most common primary heart tumor, ¾ of all valve tumors
• Aortic valve
• Grossly resembles lambl excrescences (LE) but is on surface of valve and LE are on valve closure
• Histo – avascular papillae lined by endothelium

Answer 21

Papillary Elastofibroma

Question 22

What is the main difference between giant cell myocarditis (shown here) and hypersensitivity myocarditis?

Answer 22

HS will have eosinophils (shown here)

Question 23

What is the number one cause of bacterial myocarditis worldwide?

Answer 23

Corynebacterium diphtheriae

Question 24

This lesion:

• Related to coronary artery dz but occurs only in transplant pts
• Histo – concentric intimal thickening of intramyocardial vessels
• Can see complete obstruction of vessel lumen and ischemic damage
• Limiting factor for long term success of heart transplant and main cause of death in long term survivors

Answer 24

Cardiac allograft vasculopathy

Question 25

What are the lesions that recur after cardiac transplant?

Answer 25

• Sarcoid
• Giant cell myocarditis
• Amyloid
• Chagas’ dz
• Fabry dz