CHD Causing Stenosis/Regurgitation

Question 1

Which valvular stenosis is most commonly congenital (other 3 valves most commonly acquired)

Answer 1

PV stenosis

Question 2

What is a bicuspid AV?

Answer 2

Typically fusion of RCC and LCC, with a resultant “2” cusps

Question 3

What is the most common CHD found in adulthood?

Answer 3

Bicuspid AV?

Question 4

Describe the murmur associated with a bicuspid AV

Answer 4

systolic ejection “click” right after S1

• if there is associated AS, we would also hear systolic crescendo decrescendo murmur

Question 5

Describe the motion of the 2 cusps of a bicuspid AV during the cardiac cycle

Answer 5

“doming” concave during systole

“hammock” convex during diastole

Question 6

What would we see on M-Mode with a bicuspid AV?

Answer 6

Eccentric closure

Question 7

Is AI or AS more commonly associated with a bicuspid AV?

Answer 7

AI is more common, but AS can occur

Question 8

What is associated with bicuspid AV?

Answer 8

CoAo
Dilated Ao
Marfan’s syndrome (connective tissue disorder)

Question 9

What is membranous subvalvular AS?

Answer 9

Membrane/ridge of tissue in the LVOT, near the AV causing LVOT obstruction

Question 10

What might we see with subvalvular AS? (think LV and AV)

Answer 10

LVH (increased pressure)

Early systolic closure of AV (m-mode) from pressure changes

Question 11

Is the AV normal or abnormal with isolated subvalvular AS?

Answer 11

Normal valve opening

Question 12

What is supravalvular AS?

Answer 12

Narrowed AoR causing stenosis

Question 13

Is supravalvular AS common?

Answer 13

No

Question 14

What would a congenital cause of supravalvular AS be? What about acquired?

Answer 14

Congenital - William’s syndrome

Acquired - accumulated fibrous tissue/inflammation (ie. from takayasu arteritis)

Question 15

Is the AV normal or abnormal with supravalvular AS?

Answer 15

Normal valve opening

Question 16

Will the continuity equation be accurate when supravalvular or subvalvular AS is present? Why?

Answer 16

No because the AV is normal. The values would be a false representation of the stenosis because the stenosis is NOT caused by the AV itself

Question 17

What is AV prolapse? (AVP)

Answer 17

Downward displacement of the AV cusps below the AV/sinotubular junction

Question 18

What is AVP most commonly seen with?

Answer 18

Bicuspid AV

Question 19

What is associated with AVP?

Answer 19

AoR dilation, MVP, severe MR, perimembranous VSD

Question 20

What will 100% be seen with AVP?

Answer 20

AI

Question 21

What is a Cleft MV?

Answer 21

A division in the AMVL

Question 22

What is most commonly seen with a cleft MV?

Answer 22

Partial AVCD

*also seen with primum ASD

Question 23

Describe the motion of the AMVL of a cleft MV during diastole

Answer 23

“Doming” during diastole with no stenosis

Question 24

What results from a Cleft MV?

Answer 24

MR

Question 25

What is a Parachute MV?

Answer 25

One pap (usually only posteromedial) attaches to both sets of chordae

Question 26

What does Parachute MV present similar to?

Answer 26

MS

Question 27

What is a double orifice (outlet) MV

Answer 27

Accessory orifice - like 2 MV's

Question 28

What does a double outlet MV cause?

Answer 28

Can be normal or result in MS or MR

Question 29

What is Cor Triatriatum?

Answer 29

Membrane across the mid LA that disrupts flow Looks like a 3rd atrium

Question 30

What kind of MS can occur from Cor Triatriatum?

Answer 30

Supravalvular MS

Question 31

What does the presentation of Aor Triatriatum mimic?

Answer 31

It mimics MS with turbulence, increased PSV and PPV

Question 32

80% of those with Cor Triatriatum also have what?

Answer 32

ASD

Question 33

Is Cor Triatriatum more common in the LA or RA? What is it called when seen in the RA?

Answer 33

LA more common Cor Triatriatum dexter if in RA

Question 34

What is Supravalvular MS (Ring)

Answer 34

A membrane or tissue thickening, at the level of the MV annuli causing an LVIT obstruction

Question 35

What is associated with Supravalvular (Ring) MS?

Answer 35

ASD VSD CoAo Persistent LSVC Shone Complex

Question 36

What is MV Prolapse? (MVP)

Answer 36

A prolapsing leaflet (doesn't coapt properly) resulting in MR **Usually congenital but doesn't present until later in life

Question 37

What is Pulmonary Vein Stenosis? (PVS)

Answer 37

Narrowing of 1 or more PV's at the entrance to the LA

Question 38

What does PVS cause?

Answer 38

PHTN (increased pulmonary pressures), resulting in RVH, RV + RA dilation

Question 39

What would we see on PW with PVS?

Answer 39

Increased S + D waves with flow reversal in between the systolic and diastolic flow