25 Pulmonary stenosis
Salient features
History
• Patients may be asymptomatic.
• Ask if there is a history of maternal rubella.
• Dyspnoea on exertion or fatigability may occur (when the stenosis is severe), less often, patients may have retrosternal chest pain or syncope with exertion. Eventually, right ventricular failure may develop, with resultant peripheral oedema and abdominal swelling.
• Cyanosis and clubbing (if the foramen ovale is patent, shunting of blood from the right to the left atrium may occur).
Examination
• Prominent ‘a’ wave in the JVP
• Ejection click, which decreases on inspiration (this is the only right sided sound that decreases with inspiration) (valvular stenosis) (Br Heart J 1951;13:519)
• Soft P2, with a wide split second sound
• Ejection systolic murmur in the left upper sternal border, best heard on inspiration. The murmur radiates to the left shoulder and left lung posteriorly. The more severe the stenosis, the longer is the murmur, obscuring the second aortic sound A2
• Then proceed by looking for central cyanosis and clubbing (Fallot’s tetralogy).
Advanced-level questions
How is the severity of pulmonary valve stenosis determined?
Mild: valve area larger than 1.0 cm2/m2, transvalvular gradient <50 mmHg or peak right ventricular systolic pressure <75 mmHg.
Moderate: valve area 0.5–1.0 cm2/m2, transvalvular gradient 50–80 mmHg or right ventricular systolic pressure 75–100 mmHg.
Severe: valve area <0.5 cm2/m2, transvalvular gradient >80 mmHg or right ventricular systolic pressure >100 mmHg.
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