Pulmonary Stenosis

Question 1

What may cause pulmonary stenosis

Answer 1

congenital rubella
ToF (subvalvular obstruction)
Noonans Syndrome
Turns syn
Downs syn
congenital pul stenosis
IE
Rheumatic fever
carcinoid syndrome
Williams syndrome

Question 2

Features of Noonan’s syndrome

Answer 2

cubital valgus
webbed neck
widely spaced nipples
short stature
ptosis
proptosis
strabismus
mild intellectual disability
Triangular face
low set ears
pectus deformity
easy bruising (coagulation defects)
Delayed puberty

Question 3

Symptoms of pulmonary stenosis

Answer 3

SOB
ETT reduced
cyanosis if ASD/VAD
Signs of right sided HF
Syncope

Question 4

Clinical findings of pulmonary stenosis

Answer 4

Large A waves
RV heave
functional TR
widely split S2

Question 5

Cardiac manifestations of Noonans

Answer 5

FCM
septal lesions
Pulmonaary stenosis

Question 6

What is the genetics behind Noonans

Answer 6

Autosomal dominant
Mutation in PTPN11 gene chromosome 12/ SOS1 gene chromosome 2

Question 7

Auscultation of pulmonary stenosis

Answer 7

ESM heard loudest in pulmonary area in inspiration
May radiate to left infraclavicular area/ centre of back
widely split P2 - why? delay in RV emptying

Question 8

Investigation of pulmonary stenosis

Answer 8

ECG: PBBB + P-pulmonale
XR Chest
TTE
Cardiac MRI - other structure abnormalities
Cardiac catheterisation

Question 9

Management of pulmonary stenosis

Answer 9

Pulmonary valvotomy
Percutanous valve replacement
balloon valvuloplasty of the RVOT (palliative)
Surgical replacement