Station 3.6: Pulmonary stenosis

Question 1

clinical signs

What are the clinical signs of Pulmonary stenosis?

Pulmonary stenosis

Examine this patient’s cardiovascular system. He has had swollen ankles.

Answer 1

• Raised JVP with giant a waves
• Left parasternal heave
• Thrill in the pulmonary area
• Auscultation
  ⚬ Ejection systolic murmur (ESM) heard loudest in the pulmonary area in inspiration.
  ⚬ Widely split second heart sounds, due to a delay in RV emptying.
  ⚬ Severe: inaudible P2, longer murmur duration obscuring A2.
• Right ventricular failure: ascites and peripheral oedema
• Tetralogy of Fallot: PS, VSD, overriding aorta and RVH (sternotomy scar)
• Noonan’s syndrome: phenotypically like Turner’s syndrome but male sex
• Other murmurs: functional TR and VSD

Question 2

Discussion - investigation

What investigations do you recommend for Pulmonary stenosis?

Pulmonary stenosis

Examine this patient’s cardiovascular system. He has had swollen ankles.

Answer 2

• ECG: p‐pulmonale, RVH and RBBB
• CXR: oligaemic lung fields and large right atrium
• TTE: severity (pressure gradient), RV function and associated cardiac lesions

Question 3

Discussion - management

What mangement do you recommend for Pulmonary stenosis?

Pulmonary stenosis

Examine this patient’s cardiovascular system. He has had swollen ankles.

Answer 3

• Pulmonary valvotomy – if gradient >70mm Hg or there is RV failure
• Percutaneous pulmonary valve implantation (PPVI)
• Surgical repair/replacement

Question 4

Discussion - management

Explain Carcinoid syndrome?

Pulmonary stenosis

Examine this patient’s cardiovascular system. He has had swollen ankles.

Answer 4

• Gut primary with liver metastasis secreting 5‐HT into the blood stream
• Toilet‐symptoms: diarrhoea, wheeze and flushing!
• Secreted mediators cause right‐sided heart valve fibrosis resulting in tricuspid regurgitation and/or pulmonary stenosis
• Rarely a bronchogenic primary tumour or a right‐to‐left shunt can release 5‐HT into the systemic circulation and cause left‐sided valve scarring
• Treatment: octreotide or surgical resection