Pulmonary Stenosis

• What is pulmonary stenosis?


  Narrowing of pulmonary valve (mostly congenital) → restricts blood flow from RV into pulmonary arteries → increase RV pressure → R heart strain

  Mostly congenital, associated with…

  • Noonan syndrome
  • Tetralogy of Fallot
• What are the signs & symptoms of PS?

  Chest pain

  Syncope

  SOB

  Fatigue

  Failure to thrive (faltering growth)

  Cyanosis

  Signs of RHF

  Ejection systolic murmur (with or without systolic click) → loudest in pulmonary area with deep inspiration

  Widely split S2

  Thrill in pulmonary area

  Raised JVP with giant A waves

  Peripheral oedema

  Ascites
• What are the RFs of PS?

  Noonan syndrome

  Alagille syndrome

  Williams syndrome

  Congenital rubella syndrome

  IE
• What are the Inx of PS?

  Obs

  Full Hx & examination

  ECG

  Bloods (FBC, U&Es, LFTs, CRP, ABG (if cyanotic))

  CXR

  Echo (w/Doppler) -> confirms diagnosis
• What does an ECG show in PS?


  Mild PS → typically normal or mild right axis deviation

  Moderate PS → right axis deviation (abnormal for age) and RV conduction delay (abnormal for age)

  Severe PS → extreme right axis deviation, R wave large for age, right atrial enlargement with tall and peaked P wave in lead II and in precordial leads V1 to V3
• What does a CXR show in PS?

  Usually normal

  May show prominent main pulmonary artery shadow; marked cardiomegaly, right atrial and ventricular enlargement

  Decreased pulmonary vascularity (severe disease)
• What does an Echo show in PS?


  Confirms diagnosis

  Abnormal morphology of valve

  Increased transvalvular gradient across pulmonary valve during systole (>10 mmHg)
• What are the DDx of PS?


  Innocent murmur

  Straight back syndrome

  Idiopathic dilation of pulmonary artery

  Pulmonary artery stenosis

  Aortic valve stenosis

  Atrial septal defect (ASD)

  Ventricular septal defect

  Ebstein's anomaly

  Tetralogy of Fallot

  Pulmonary valve atresia with intact ventricular septum
• What is the treatment of mild PS?


  Observation

  Asymptomatic & rarely progressive
• What is the treatment of moderate PS?


  Percutaneous balloon pulmonary valvuloplasty (1st line)

  Surgical valvuloplasty (2nd line)
• What is the treatment of severe to critical PS?


  • W/o resp distress or cyanosis
  • Percutaneous balloon pulmonary valvuloplasty (1st line)
  • Surgical valvuloplasty (2nd line)
  • W/ resp distress or cyanosis
  • Supplemental O2 ± alprostadil and percutaneous balloon pulmonary valvuloplasty (1st line)
  • Surgical valvuloplasty (2nd line)