Pulmonary Stenosis

Question 1

Pulmonary stenosis presentation

Answer 1

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

Question 2

Clinical signs Pulmonary stenosis

Answer 2

1. Raised JVP with giant a waves
2. Left parasternal heave
3. Thrill in the pulmonary area (Lt 2nd intercostal space)
4. Auscultation:
   - Ejection systolic murmur (ESM) heard loudest in the pulmonary area in inspiration.
   - Widely split S2, due to a delay in RV emptying.
   - Severe: inaudible P2, longer murmur duration obscuring A2.
5. Right ventricular failure: ascites and peripheral oedema
6. Tetralogy of Fallot: PS, VSD, overriding aorta and RVH (sternotomy scar)
7. Noonan’s syndrome: phenotypically like Turner’s syndrome but male sex
8. Other murmurs: functional TR and VSD

Question 3

Auscultation in Pulmonary Stenosis

Answer 3

1. Ejection systolic murmur (ESM) heard loudest in the pulmonary area in inspiration.
2. Widely split second heart sounds, due to a delay in RV emptying.
3. Severe: inaudible P2, longer murmur duration obscuring A2.

Question 4

Investigation of Pulmonary stenosis

Answer 4

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

Question 5

Management of Pulmonary stenosis

Answer 5

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

Question 6

Carcinoid syndrome

Answer 6

1. Gut primary with liver metastasis secreting 5‐HT (5-Hydroxytryptamine) into the blood stream
2. Toilet‐symptoms: diarrhoea, wheeze and flushing!
3. Secreted mediators cause right‐sided heart valve fibrosis resulting in TR and/or PS.
4. 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
5. Treatment: octreotide or surgical resection