Term
| What symptoms and signs occur in PS? |
|
Definition
Dyspnea
Fatigue
Syncope
Hypoxemia and cyanosis |
|
|
Term
True or False
The murmur of PS is louder over the left posterior lung field than the right |
|
Definition
True
The LPA courses directly posterior whereas the RPA takes an abrupt turn |
|
|
Term
| How does the ejection click in valvar PS vary with respiration? |
|
Definition
| It gets louder with expiration. In inspiration, the higher RVEDP from increased venous return results in pre-ejection movement of the cusps. |
|
|
Term
| What valvar morphologic abnormalities are present in PS? |
|
Definition
Acommisural
Unicommsiural
bicuspid
tricuspid with fused commissures resulting in doming
dysplastic with thick, rigid leaflets |
|
|
Term
| Which echo windows can be used to image the pulmonic valve |
|
Definition
PSAX
PLAX-RV outflow view
Apical 4 chamber with anterior and leftward angulation
Subcostal 4 chamber view with anterior angulation
Subcostal SAX |
|
|
Term
|
Definition
|
|
Term
True or False
In valvar PS, hypertrophy of muscles bundles in the infundibulum often causes subvalvar obstruction. |
|
Definition
False
The infundibulum is usually wide open and significant dynamic obstruction is rare. However, after valvuloplasty decreases RV afterload, hypertrophied infundibular muscle may contract enough to cause a high gradient that can lead to shock. That is known as the "suicide ventricle". |
|
|
Term
True or False
Unlike in AS, the extent of post stenotic dilatation in valvar PS does correlate with the severity of stenosis.
|
|
Definition
|
|
Term
| What are the causes of TR in patients with PS? |
|
Definition
RV dilatation due to high RVSP
RV dysfunction due to endocardial fibrosis
Structural abnormalities of the tricuspid valve |
|
|
Term
| A patient has a PDA and PS. The TR jet is 4.5 m/s and the PV jet is 2.5 m/s. Which jet is the better measure of the severity of the PS? |
|
Definition
The TR jet.
The PDA raises the pressure in the PA therefore lowering the gradient between the RV and PA. Of course, a small PDA would have no or little effect on the gradient. |
|
|
Term
| PV dysplasia is associated with which syndrome? |
|
Definition
Noonan's syndrome
about 1 in 4 pts with with Noonan's syndrome has PS. Of those with PS, about 1 in 4 have dysplastic valves. Annular hypoplasia is often present. |
|
|
Term
| What conditions must be met before patients with PS can be treated with balloon valvuloplasty? |
|
Definition
Normal or minimally decreased annulus
absence of RV or TV hypoplasia
|
|
|
Term
| After valvuloplasty, what should the echocardiographer look for? |
|
Definition
Residual PS
Residual PI
Dynamic obstruction |
|
|
Term
| Are RV and TV hypoplasia seen in mild or severe PS? |
|
Definition
|
|
Term
| What PV gradients partition mild, moderate and severe PS |
|
Definition
By catheterization, peak gradients of 30 and 50 mmHg are the partition values
By echo, peak gradients of 40 and 60mmHg are used. |
|
|
Term
| What is the valve area of the PV? |
|
Definition
|
|
Term
| When is mechanical (balloon valvotomy or surgery)indicated in asymptomatic patients? |
|
Definition
| When the peak Doppler gradient is >60 or the mean is >40 mmHg |
|
|
Term
| How often should an asymptomatic patient with mild PS be followed according to current ACC/AHA guidelines? |
|
Definition
|
|
Term
| How often should an asymptomatic patient with moderate PS be followed according to current ACC/AHA guidelines? |
|
Definition
|
|
Term
| When is balloon valvuloplasty or surgery indicated in symptomatic patients? |
|
Definition
| When the peak Doppler gradient is >50 mmHg and the mean >30 mmHg |
|
|
Term
| When cardiac catheterization derived gradients are used, what are the thresholds for pulmonary valvuloplasty or surgery? |
|
Definition
Asymptomatic patients 40 mmHg
Symptomatic patients 30 mmHg |
|
|
Term
| How does Doppler agree with catheterization in terms of PV gradients? |
|
Definition
Doppler peak gradients are up to 20 mmHg higher than cath gradients
Agreement between mean gradients is much better and one can be substituted for the other |
|
|
Term
| What congenital syndromes can PS be found in? |
|
Definition
|
|
Term
| What is the RV systolic pressure using Doppler in mild, moderate, and severe PS? |
|
Definition
Mild-25-49mmHg
Moderate-50-79 mmHg
Severe->80 mmHg |
|
|
Term
| Are women with moderate PS at high risk during pregnancy? |
|
Definition
No. Only women with severe PS (even if asymptomatic)are at high risk for complications.
When pregnancy is planned, balloon valvuloplasty should precede conception. |
|
|
Term
True or False
Mild PS remains stable over time |
|
Definition
False
PS tends to worsen over time, but the rate of worsening may be very slow |
|
|
Term
| What is the prognosis for untreated patients with moderate or severe stenosis? |
|
Definition
| Most develop symptoms due to RV failure in their 40s. The risk of death at that point is high. |
|
|
Term
| Is the dilated pulmonary artery in pts with PS prone to dissection or rupture? |
|
Definition
| While there are case reports of rupture and dissection, these catastrophes are rare. Possibly that reflects low PA pressure. |
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