Heart Flashcards

1
Q

What is the most common cause of congenital arrhythmia?

A

Maternal systemic lupus erythematosus (SLE).

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2
Q

What is the most common congenital cardiac malformation? Most common congenital cardiac anomaly?

A
  • “VSD is most common congenital cardiac malformation
  • “ASD is most common congenital cardiac anomaly, usually asymptomatic until adulthood”
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3
Q

What heart abnormality is seen in Turner’s?

A

Preductal coarctation of the aorta

They also get single umbilical artery

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4
Q

What are the 4 components of Tetralogy of Fallot?

A

Right to left shunt

– Overriding aorta

– Pulmonary valve stenosis

– Ventricular septal defect

– Right ventricular hypertrophy

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5
Q

What is the caveat when dating myocardial infarctions?

A

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

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6
Q

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

A

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!)

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7
Q

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

A

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

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10
Q

What disease is this? (instant recognition!)

A

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)

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11
Q

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

A

GET EM!

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

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12
Q

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

A

Arrhythmogenic RV Dysplasia

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13
Q

What cardiac tumor is being shown?

A

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”

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14
Q

What is a McCallum’s patch?

A

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

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15
Q

What is Libman Sacks endocarditis?

A

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

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16
Q

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

A
Defect in ß myosin heavy chain on
chromosome 14 (50%)
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19
Q

Where do cardiac myxomas occur?

A

75% in the left atrium

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23
Q

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

A

Cardiac hypertrophy

24
Q

BQ SLIDE!

What is this lesion in the heart?

A

Rheumatic Heart Disease

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

25
Q

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
A

Papillary Elastofibroma

26
Q

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

A

HS will have eosinophils (shown here)

27
Q

What is the most common congenital heart malformation?

A

VSD

28
Q

What is tetralogy of Fallot?

A

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

29
Q

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

A

Tricuspid valve insufficiency

30
Q

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?

A

Naxos syndrome

31
Q

What are the large, medium and small vessel vasculitides?

A

Large: Giant Cell Arteritis and Takayasu’s

Medium: polyarteritis nodosa and Kawasaki’s

Small: Wegener’s, Churg Strauss and microscopic polyangiitis

32
Q

What cardiac tumor is positive for calretinin?

A

Cardiac myxoma

Other calretinin + tumors:

Adenomatoid tumor

Sex-cord stromal tumors

Adrenocortical tumors

Mesothelioma

33
Q

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

A

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

37
Q

What is the number one cause of bacterial myocarditis worldwide?

A

Corynebacterium diphtheriae

39
Q

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
A

Cardiac allograft vasculopathy

41
Q

What are the lesions that recur after cardiac transplant?

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