pulmonic valvular stenosis

Etiology: - may occur as an isolated lesion - may occur in conjunction with ventricular septal defect - Noonan's syndrome - Williams-Beuren syndrome Pathology: 1) valve is frequently pliable 2) valve may be bicuspid 3) thickened dysplastic valve occurs in Noonan's syndrome 4) right ventricular hypertrophy may occur Clinical manifestations: 1) often asymptomatic 2) prominent a-wave in jugular venous pulse 3) right ventricular heave 4) pulmonic ejection click immediately after S1 a) diminishes with inspiration b) earlier the click, the more severe the stenosis c) click indicates valve is pliable & noncalcified d) results from rapid opening of the pulmonary vavle leaflets 5) systolic ejection murmur a) crescendo-decrescendo systolic murmur heard best at the upper left sternal border (2nd left intercostal space) [2] b) radiation to the left clavicle c) the longer the murmur & later peaking, the more severe the stenosis 6) soft & late P2 - with more severe stenosis, P2 becomes inaudible - S2 may become fixed with severe stenosis & inaudible P2 [2] 7) systolic thrill due to high flow velocity across the pulmonary valve [2] Special laboratory: - electrocardiogram - if right ventricular systolic pressure is < 60 mm Hg, electrocardiogram is normal - otherwise - right axis deviation - right ventricular hypertrophy - right atrial enlargement - echocardiogram a) right ventricular hypertrophy b) increased right ventricular pressure c) pressure gradient across pulmonic valve Radiology: - chest X-ray a) post-stenotic pulmonary dilatation b) pulmonary oligemia only with severe stenosis c) calcification of pulmonary valve (rare) d) right atrial enlargement [2] Complications: - severe pulmonary regurgitation after pulmonary valvuloplasty [2] Management: 1) no treatment (surgery) unless a) symptomatic b) right ventricular pressure approaches 2/3 systemic pressure c) small pulmonary valve annulus d) pulmonary regurgitation > moderate e) subvalvular or supravalvular pulmonary stenosis f) cardiac surgery for another reason [2] 2) repair of severe pulmonary valvular stenosis regardless of symptoms in a patient with Noonan's syndrome [2] - patients with dysplastic valve (Noonan's syndrome) should undergo pulmonary valve replacement - participation in sports not recommended in patients with severe pulmonary valvular stenosis - pulmonary valve replacement recommended [2] 3) percutaneous balloon valvuloplasty for pliable valve - not clear that this would correct pulmonary regurgitation 4) surgical replacement for caclified valve 5) lifelong cardiac survelliance if: a) balloon valvuloplasty b) pulmonary valve replacement 6) prophylaxis for bacterial endocarditis not indicated

General

pulmonic valve disease

References

Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 47
Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18, 19 American College of Physicians, Philadelphia 2009, 2012, 2015, 2018, 2022.

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