Cardiovascular Pathology 5

Question 1

in fetal circulation, the _____ resistance is high leading the shunt to go ___ → ____

Answer 1

pulmonary; (lungs have not expanded yet but after birth, the pulmonary resistance will ↓)

R→ L bypassing the lungs

Question 2

what are the two physiologic R → L shunts in the fetus?

Answer 2

• foramen ovale

- ductus arteriosus

Question 3

the patency of the ductus arteriosus can be maintained by giving _____

Answer 3

prostaglandins

Question 4

what leads to the closure of the ductus arteriosus after birth?

Answer 4

prostaglandins help keep it open. they are produced by the placenta and metabolized by the lungs.
following birth there is ↓ prostaglandins due to: ↓ production and ↑ metabolism

Question 5

What are some complications of a L → R shunt?

Answer 5

• right heart failure because it is not used to the large volume of blood.
• pulmonary circulation congestion (plethoric lung fields) → recurrent episodes of infections

Question 6

with congenital malformations can cause a R→ L shunt?

Answer 6

• Tetralogy of Fallot
• transposition of the great vessels
• truncus arteriosus
• tricuspid atresia
• total anomalous pulmonary venous connection

Question 7

what are the four malformations seen in tetralogy of fallot?

Answer 7

• Pulmonary stenosis: (degree determines prognosis)
• Right ventricular hypertrophy
• overriding aorta
• VSD

PROV

Question 8

what determines the degree of prognosis in a patient with tetralogy of fallot?

Answer 8

the degree of pulmonary stenosis

Question 9

why do patients with tetralogy of fallot squat a lot?

Answer 9

squatting ↑ the systemic resistance and thus helps alleviate the R → L shunt by forcing more of the blood the go through the pulmonary vessels from the right ventricle

Question 10

squatting ↑/ ↓ ________ resistance

Answer 10

↑ systemic (aortic) resistance

Question 11

tetralogy of fallot is associated with ________ syndrome

Answer 11

down syndrome

Question 12

the VSD in tetralogy of Fallot puts these patients at a higher risk for developing_____

Answer 12

infective endocarditis

Question 13

_____ and _____ help with survival in a patient with transposition of the great vessel

Answer 13

PDA and VSD because they help mix the blood

Question 14

what is truncus arteriosus?

Answer 14

failure of partitioning of the embryologic truncus into aorta and pulmonary artery;

single great artery gets blood from BOTH ventricles
underlying VSD

Question 15

tricuspid atresia is usually associated with _____

Answer 15

ASD to bypass obstruction

Question 16

what is the developmental abnormality in total anomalous pulmonary venous connection (TAPVC)

Answer 16

the pulmonary veins drain into the left inominate vein or coronary sinus or right atrium instead of the left atrium like normal

Question 17

TAPVC is technically a L→ R shunt because: _______ but it is associated with a _____ in order to be compatible with life

Answer 17

blood flow that should be going the left atrium is instead going to the right atrium;

ASD → right to left shunt due to the lower left atrial pressure → cyanosis

Question 18

list of left to right shunts:

Answer 18

D’s:

• ASD
• VSD
• PDA

Question 19

clinical features of VSD:

Answer 19

• pulmonary HTN
• CHF
• pan systolic murmor

Question 20

_____ murmur can be heard with VSD

Answer 20

pan systolic

Question 21

what is eisenmenger complex?

Answer 21

shunt reversal seen with VSD due to the ↑ pulmonary HTN → ↑ pressure in the right atrium → reversal of shunt from L→ R to R→ L because pressure on the R > L now
eisemneger complex leads to cyanosis

Question 22

____ is the most common congenital cardiac malformation diagnosed in adults

Answer 22

ASD

Question 23

what are the three types of ASD and which is the most common?

Answer 23

• ostium primum
• ostium secundum (MOST COMMON)
• sinus venosus

Question 24

continuous “machine like” murmur is seen with ______

Answer 24

PDA

Question 25

_______ can be given to close the PDA

Answer 25

indomethacin (prostaglandin synthesis inhibitor)

Question 26

maternal ______ infection is associated with PDA

Answer 26

rubella

Question 27

PDA connects and causes blood to flow from _____ to ____ and usually closes due to _____

Answer 27

aortic arch to the left pulmonary artery closes due to high oxygen tension

Question 28

what fetal condition is maternal rubella infection associated with?

Answer 28

PDA

Question 29

what are 3 cardiac malformations that can cause obstruction?

Answer 29

- coarctation of aorta - pulmonary stenosis and atresia - aortic stenosis and atresia (hypo plastic left heart syndrome)

Question 30

coarctation of the aorta is associated with ______ syndrome

Answer 30

Turner's (XO)

Question 31

what are the two types of coarctation of the aorta and how are they different?

Answer 31

- preductal (Infantile coarctation): has PDA and the coarctation is just before the PDA - post ductal (adult) coarctation: ductus arteriosus is closed

Question 32

what is differential cyanosis and what defect is it associated with?

Answer 32

cyanosis only in the lower limbs of the body; associated with preductal coarctation of the aorta the PDA will pass deoxygenated blood into the aorta that goes down to the lower limbs

Question 33

marked blood pressure difference between the upper and lower limb is seen in ______

Answer 33

post ductal coarctation of the aorta

Question 34

what are some clinical features in post ductal coarctation of the aorta?

Answer 34

- difference in blood pressures in upper and lower limb - intermittent claudication - notching of ribs due to collaterals - no selective cyanosis - HTN in upper extremities

Question 35

notching of the ribs is seen in _____

Answer 35

post ductal coarctation of the aorta due to the collaterals trying to supply blood to the lower extremities

Question 36

_______ is only seen in preductal coarctation of the aorta but NOT in the post ductal

Answer 36

cyanosis

Question 37

aortic stenosis is classified into what three types based on location?

Answer 37

- valvular: affects valve cusps - subvalvular: ring of fibrous tissue between valve cusps resulting in LVH - supravalvular: elastin defect involving the ascending aorta and with a thickening of the wall leading to luminal obstruction

Question 38

complete obstruction of the aortic outflow leads to ____ of the left ventricle and aorta and must have _____ for survival

Answer 38

hypoplasia; PDA

Question 39

what is a gross feature of the underdeveloped left ventricle in hypo plastic left heart syndrome

Answer 39

- dense endocardial fibroelastosis

Question 40

cardiac myxoma is a ____ tumor seen in the ______

Answer 40

benign; left atrium

Question 41

how can you try to diagnose a cardiac myxoma?

Answer 41

do an X Ray because they often calcify and these can be seen on x ray

Question 42

patient has a lobulated pedunculated mass in her left atrium. what would you expect to see on histology slide?

Answer 42

cardiac myxoma; multinucleated stellate cells suspended in an edematous mucopolysaccharide rich stroma

Question 43

stellate cells in stroma is seen in the histology of what disease?

Answer 43

cardiac myxoma

Question 44

what are four common primary sites of tumors that can lead to secondary tumors in the heart?

Answer 44

- lung cancer - breast - lymphoma - malignant melanoma