Pulmonic Stenosis

Question 1

Noonan Syndrome

Answer 1

• Associated with Pulmonic Stenosis

- classified as a cardiofacial syndrome with PS, HCM and ASD (30%)

Question 2

Pulmonic Stenosis Etiology

Answer 2

1. Congenital (most common)
2. Rheumatic (rare)
3. Carcinoid
4. Peripheral (PPS- junction of the R and L PAs)
5. Infundibular (subvalvlar)
6. Prosthetic valve dysfunction

Question 3

Pulmonic Stenosis most common etiology?

Answer 3

Congenital

Question 4

Pulmonic Stenosis most rare etiology?

Answer 4

Rheumatic

Question 5

Pulmonic Stenosis Pathophysiology

Answer 5

1. Systolic pressure overload
2. Regainal Hypertrophy
3. Commonly associated with other congenital malformations
4. RV chamber size usually normal, RA will enlarge
5. Increased risk for endocarditis.

Question 6

Pulmonic Stenosis Pathophysiology

Systolic pressure overload leads to?

Answer 6

Right ventricular hypertrophy (RVH)

Question 7

Pulmonic Stenosis Pathophysiology

Regainal Hypertrophy may lead to?

Answer 7

Infundibular stenosis

Question 8

Pulmonic Stenosis Pathophysiology

Commonly associated with which congenital malformations?

Answer 8

1. VSDs
2. ASDs
3. Tetralogy of Fallot

Question 9

Pulmonic Stenosis murmur

Answer 9

Systolic ejection murmur left upper sternal border (LUSB)

Question 10

Pulmonic Stenosis

Physical Signs

Answer 10

1. Dyspnea on exertion
2. Pulmonic ejection sound, decreased /delayed P2
3. Increased A wave or jugular venous pulsation (JVP)
4. Sustained RV impulse at mid-Lower left sternal border (LSB).

Question 11

Pulmonic Stenosis

M-mode may show?

Answer 11

An increase in the pulmonic “a” dip of more than 7mm (useful for severe PS only.)

Question 12

Pulmonic Stenosis

Echo

Answer 12

1. Valvular thickeneing and systolic doming (2D)
2. RVH
3. Post-stenotic dilation of the pulmonary artery (PA)
4. Narrowing of RVOT in infundibular PS (subvalvular in RVOT)

Question 13

Know that PS does not cause?

Answer 13

Pulmonary hypertension (it increases RVH which prevents it from developing)

Question 14

What do you see on M-mode in pulmonic stenosis vs. PHTN

Answer 14

Pulmonic stenosis= exaggerated “a” dip

PHTN= the “a” dip disappears

Question 15

Pulmonic Stenosis

Doppler

Answer 15

1. increased velocities and turbulence at level of obstruction (valvular, or suprevalvular)
2. Use pulsed/color Doppler to locate level of obstruction
3. check for coexisting pulmonic regurg
4. Measure peak and mean gradients (parasternal short axis AoV level and RVOT long axis are best)

Question 16

Nomral pulmonary velocity is about/

Answer 16

1m/sec

Question 17

If unable to obtain PS gradient from the parasternal window where else can you go?

Answer 17

subcostal short axis

Question 18

AHA/ACC guidelines for Pulmonic Stenosis severity:
Peak velocity (m/s)
mild-
mod-
severe-

Answer 18

mild- <3.0
mod= 3.0-4.0
severe= >4.0

Question 19

AHA/ACC guidelines for Pulmonic Stenosis severity:
Peak gradient (mmHg)
mild=
mod-
severe-

Answer 19

mild- <36
mod= 36-64
severe= >64

Question 20

Pulmonic Stenosis gradients vary with?

Answer 20

respiration