CHD of the Right Heart

Question 1

What is pulmonary stenosis? (PS)

Answer 1

Narrowing of the OV causing obstruction to RVOT flow

Question 2

Is congenital or acquired PS more common?

Answer 2

Congenital, most often in pediatric pt’s and rarely seen in adults

If acquired, typically from carcinoid syndrome

Question 3

What does PS do to the RV and RA?

Answer 3

RVH (increased pressures)

RA dilation

Question 4

What is most commonly associated with PS?

Answer 4

Maternal rubella
Clubbed fingers
Turner’s
Noonan’s
Polycythemia Vera

*Peripheral PAS associated w/ William’s
*Infundibular PAS associated w/TOF

Question 5

What are S+S of PS?

Answer 5

SOB
SOBOE
Chest pain
Syncope
Fatigue

Question 6

What US finding is most commonly seen with PS? (Hint: septal defect)

Answer 6

VSD

Question 7

What happens to the PV leaflets with PS?

Answer 7

Thickened
Doming in systole

Question 8

If the PS jet is eccentric, what can happen to the MPA?

Answer 8

Dilation

Question 9

What is an M-Mode finding with PS?

Answer 9

Increased A - wave

Question 10

What is Pulmonary Atresia?

Answer 10

Absence of PV opening

Question 11

With Pulmonary Atresia, how does the PA get blood?

Answer 11

Ductus arteriosus

*Must remain open after birth! Or have associated defects

Question 12

What is associated with Pulmonary Atresia?

Answer 12

VSD with overriding Ao

Question 13

What is Tricuspid Atresia?

Answer 13

Absence of TV opening

Question 14

What associated defects must be present for survival with Tricuspid Atresia?

Answer 14

VSD AND PFO

• once PDA closes, no deoxygenated blood can reach the PA without a VSD and PFO

Question 15

What happens to the heart chambers with Tricuspid Atresia?

Answer 15

Hypoplastic RV
Biatrial enlargement with PFO (R to L or bi-directional)

Question 16

What is Ebstein Anomaly?

Answer 16

When the TV originates very inferior, toward the apex

Question 17

Are the TV annuli inferior or in their normal position with Ebstein’s?

Answer 17

Normal

Question 18

What is the normal measurement (in mm and mm/m^2) of distance between the TV and MV

Answer 18

20 mm
8 mm/m^2

Question 19

What happens to the heart chambers with Ebstein’s?

Answer 19

Very large RA ++++
Small RV
Small, ‘D’ shaped LV

Question 20

What does Ebstein’s cause?

Answer 20

Severe TR

Question 21

What is associated with Ebstein’s?

Answer 21

Secundum ASD (most common), R to L shunt

30% have WPW

Question 22

What is Eisenmenger’s Syndrome

Answer 22

Occurs when there is a shunt between the L and R side. Normal shunt flow is from R to L, but if the right pressures exceed the L, the shunt direction reverses

Question 23

What does Eisenmenger’s cause?

Answer 23

Cyanosis and right heart failure

Question 24

What defect does Eisenmenger’s usually occur with?

Answer 24

VSD (most common)
ASD, PDA, AVCD

Question 25

What is a Persistent Left SVC (PLSVC)

Answer 25

Blood from left UE drains directly into the coronary sinus through the brachiocephalic vein

Question 26

What would we most commonly see on US with PLSVC? What if we used contrast?

Answer 26

Dilated coronary sinus

If saline contrast is injected into the left arm, and the coronary sinus fills BEFORE the RA, PLSVC is present

Question 27

What is an Aortopulmonary Window? (AoPW)

Answer 27

A direct connection between the AscAo and MPA (L to R shunt, unless Eisenmenger’s is present)

Question 28

What does an Aortopulmonary Window do to the descending aorta?

Answer 28

Diastolic flow reversal

Question 29

What murmur is heard with diastolic flow reversal in the Desc Ao from an AoPW?

Answer 29

Continuous murmur

Question 30

What are S+S of AoPW?

Answer 30

Respiratory infection
Tachypnea
Tachycardia

Question 31

What can occur as a result of AoPW?

Answer 31

CHF
PHTN

Question 32

What is a Patent Ductus Arteriosus? (PDA)

Answer 32

When DA doesnt close after birth and blood continues to shunt from the descending Ao to the PA

Question 33

What does a PDA cause?

Answer 33

Cyanosis, especially in the lower extremities

LVVO, dilated LV and LA

May see diastolic flow reversal in desc Ao