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  • • An obstructive congenital heart lesion

    • Pulmonary stenosis (PS) impedes forward blood flow and increases ventricular afterload

    • Without ventricular septal defect (VSD), obstructive lesion causes hypertrophy of corresponding ventricle

    • Ventricular hypertrophy can cause dysrhythmias, ischemia, and fibrosis

    • In most, commissures are fused causing a dome like structure

    • Most patients have patent foramen ovale

    • Few patients have atrial septal defect (ASD)

    • May be associated with poorly developed right ventricle, may require urgent intervention

    • Isolated infundibular stenosis (below pulmonary valve) is extremely rare and often less severe

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Epidemiology

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  • • 5-8% of cardiac anomalies: PS with intact ventricular septum and normal aortic root

    • 50% of deaths from PS occur in infants

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Symptoms and Signs

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  • • Poor feeding, hypoxic spells

    • Occasionally sudden death

    • Older children may be asymptomatic

    • Fatigue, dyspnea on exertion, dizziness, angina

    • Murmur easily detected

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  • • Evaluate for other cardiac or extracardiac anomalies

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  • • Physical exam

    • Echocardiography

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  • • Neonates: PGE1 (alprostadil) maintain patent ductus arteriosus until pulmonary valve obstruction relieved

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Surgery

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  • • Isolated pulmonary stenosis: Catheter balloon dilation > 90% successful

    • Surgical valvotomy (may need transannular patch, or systemic to pulmonary shunt) if catheter intervention unsuccessful

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Indications

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  • • Pulmonary valve gradient > 50 mm Hg

    • Severe RV failure

    • Cyanotic spells

    • Systemic RV pressure

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Prognosis

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  • • > 90% respond well to catheter or surgical therapy

    Critical PS: Mortality is 3-10% after treatment

    • Restenosis 10-25%

    • RV hypertrophy: Regresses after stenosis relieved

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References

Earing MG et al: Long-term follow-up of patients after surgical treatment for isolated pulmonary stenosis. Mayo Clin Proc 2005;80:871.  [PubMed: 16007892]
Peterson C et al: Comparative long-term results of surgery versus balloon valvuloplasty for pulmonary valve stenosis in infants and children. Ann Thorac Surg 2003;76:1078.  [PubMed: 14529989]
Poon LK, Menahem S: Pulmonary regurgitation after percutaneous balloon valvoplasty for isolated pulmonary valvar stenosis in childhood. Cardiol Young 2003;13:444.  [PubMed: 14694939]

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