Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • 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 +++ Epidemiology + • 5-8% of cardiac anomalies: PS with intact ventricular septum and normal aortic root• 50% of deaths from PS occur in infants +++ Symptoms and Signs + • Poor feeding, hypoxic spells• Occasionally sudden death• Older children may be asymptomatic• Fatigue, dyspnea on exertion, dizziness, angina• Murmur easily detected + • Evaluate for other cardiac or extracardiac anomalies + • Physical exam• Echocardiography + • Neonates: PGE1 (alprostadil) maintain patent ductus arteriosus until pulmonary valve obstruction relieved +++ Surgery + • Isolated pulmonary stenosis: Catheter balloon dilation > 90% successful• Surgical valvotomy (may need transannular patch, or systemic to pulmonary shunt) if catheter intervention unsuccessful +++ Indications + • Pulmonary valve gradient > 50 mm Hg• Severe RV failure• Cyanotic spells• Systemic RV pressure +++ Prognosis + • > 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 +++ 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] Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Download the Access App: iOS | Android Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.