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Pulmonic stenosis (PS) in neonates, infants, and children

Authors
Lynn F Peng, MD
Stanton Perry, MD
Section Editor
David R Fulton, MD
Deputy Editor
Carrie Armsby, MD, MPH

INTRODUCTION

Pulmonic stenosis (PS) is a common congenital heart defect, characterized by obstruction to flow from the right ventricle to the pulmonary arteries. PS can occur in isolation or be associated with other types of cardiac defects.

The pathophysiology, clinical features, diagnosis, and management of PS in children will be presented here. The clinical manifestations, evaluation, and management of PS in adults are discussed separately. (See "Clinical manifestations and diagnosis of pulmonic stenosis in adults" and "Natural history and treatment of pulmonic stenosis in adults" and "Echocardiographic evaluation of the pulmonic valve and pulmonary artery".)

ANATOMY AND EMBRYOLOGY

Anatomy — PS is defined as obstruction to right ventricle (RV) outflow at the level of the pulmonary valve. The obstruction can occur at several different locations, as follows:

Valvar stenosis is the most common type of PS, and is typically characterized by fused or absent commissures with thickened leaflets of the pulmonary valve. In most patients, the valve is a dome-shaped structure with a small orifice (movie 1) [1].

Severe PS is associated with RV hypertrophy and infundibular muscle hypertrophy, which can cause further dynamic obstruction below the pulmonary valve during RV contraction.

                                              

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Literature review current through: Nov 2016. | This topic last updated: Fri May 13 00:00:00 GMT 2016.
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References
Top
  1. Edwards JE. Congenital malformations of the heart and great vessels. In: Pathology of the Heart, Gould SE (Ed), Charles C. Thomas Publisher, Springfield, IL 1953.
  2. Beçu L, Somerville J, Gallo A. 'Isolated' pulmonary valve stenosis as part of more widespread cardiovascular disease. Br Heart J 1976; 38:472.
  3. Cabrera A, Martinez P, Rumoroso JR, et al. Double-chambered right ventricle. Eur Heart J 1995; 16:682.
  4. Noonan JA, Ehmke DA. Associated non-cardiac malformations in children with congenital heart disease. J Pediatr 1963; 63:468.
  5. Colquitt JL, Noonan JA. Cardiac findings in Noonan syndrome on long-term follow-up. Congenit Heart Dis 2014; 9:144.
  6. Collins RT 2nd, Kaplan P, Somes GW, Rome JJ. Long-term outcomes of patients with cardiovascular abnormalities and williams syndrome. Am J Cardiol 2010; 105:874.
  7. CAMPBELL M. Factors in the aetiology of pulmonary stenosis. Br Heart J 1962; 24:625.
  8. Driscoll DJ, Michels VV, Gersony WM, et al. Occurrence risk for congenital heart defects in relatives of patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect. Circulation 1993; 87:I114.
  9. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39:1890.
  10. Reller MD, Strickland MJ, Riehle-Colarusso T, et al. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr 2008; 153:807.
  11. Todros T, Paladini D, Chiappa E, et al. Pulmonary stenosis and atresia with intact ventricular septum during prenatal life. Ultrasound Obstet Gynecol 2003; 21:228.
  12. Freed MD, Rosenthal A, Bernhard WF, et al. Critical pulmonary stenosis with a diminutive right ventricle in neonates. Circulation 1973; 48:875.
  13. Vogelpoel L, Schrire V. Auscultatory and phonocardiographic assessment of pulmonary stenosis with intact ventricular septum. Circulation 1960; 22:55.
  14. Sholler GF, Colan SD, Sanders SP. Effect of isolated right ventricular outflow obstruction on left ventricular function in infants. Am J Cardiol 1988; 62:778.
  15. Danford DA, Salaymeh KJ, Martin AB, et al. Pulmonary stenosis: defect-specific diagnostic accuracy of heart murmurs in children. J Pediatr 1999; 134:76.
  16. Geva T, Greil GF, Marshall AC, et al. Gadolinium-enhanced 3-dimensional magnetic resonance angiography of pulmonary blood supply in patients with complex pulmonary stenosis or atresia: comparison with x-ray angiography. Circulation 2002; 106:473.
  17. Drossner DM, Mahle WT. A management strategy for mild valvar pulmonary stenosis. Pediatr Cardiol 2008; 29:649.
  18. Rowland DG, Hammill WW, Allen HD, Gutgesell HP. Natural course of isolated pulmonary valve stenosis in infants and children utilizing Doppler echocardiography. Am J Cardiol 1997; 79:344.
  19. Nugent EW, Freedom RM, Nora JJ, et al. Clinical course in pulmonary stenosis. Circulation 1977; 56:I38.
  20. Hayes CJ, Gersony WM, Driscoll DJ, et al. Second natural history study of congenital heart defects. Results of treatment of patients with pulmonary valvar stenosis. Circulation 1993; 87:I28.
  21. Levine OR, Blumenthal S. Pulmonic stenosis. Circulation 1965; 32:III33.
  22. Krabill KA, Wang Y, Einzig S, Moller JH. Rest and exercise hemodynamics in pulmonary stenosis: comparison of children and adults. Am J Cardiol 1985; 56:360.
  23. JOHNSON AM. Impaired exercise response and other residua of pulmonary stenosis after valvotomy. Br Heart J 1962; 24:375.
  24. Stone FM, Bessinger FB Jr, Lucas RV Jr, Moller JH. Pre- and postoperative rest and exercise hemodynamics in children with pulmonary stenosis. Circulation 1974; 49:1102.
  25. Nomura Y, Nakamura M, Kono Y, et al. Risk factors for persistence of pulmonary arterial branch stenosis in neonates and young infants. Pediatr Int 2001; 43:36.
  26. Rao PS. Percutaneous balloon pulmonary valvuloplasty: state of the art. Catheter Cardiovasc Interv 2007; 69:747.
  27. Sugiyama H, Veldtman GR, Norgard G, et al. Bladed balloon angioplasty for peripheral pulmonary artery stenosis. Catheter Cardiovasc Interv 2004; 62:71.
  28. Bergersen L, Jenkins KJ, Gauvreau K, Lock JE. Follow-up results of Cutting Balloon angioplasty used to relieve stenoses in small pulmonary arteries. Cardiol Young 2005; 15:605.
  29. Kannan BR, Qureshi SA. Catheterisation laboratory is the place for rehabilitating the pulmonary arteries. Ann Pediatr Cardiol 2008; 1:107.
  30. Kan JS, White RI Jr, Mitchell SE, Gardner TJ. Percutaneous balloon valvuloplasty: a new method for treating congenital pulmonary-valve stenosis. N Engl J Med 1982; 307:540.
  31. Radtke W, Keane JF, Fellows KE, et al. Percutaneous balloon valvotomy of congenital pulmonary stenosis using oversized balloons. J Am Coll Cardiol 1986; 8:909.
  32. Chen CR, Cheng TO, Huang T, et al. Percutaneous balloon valvuloplasty for pulmonic stenosis in adolescents and adults. N Engl J Med 1996; 335:21.
  33. Gournay V, Piéchaud JF, Delogu A, et al. Balloon valvotomy for critical stenosis or atresia of pulmonary valve in newborns. J Am Coll Cardiol 1995; 26:1725.
  34. Stanger P, Cassidy SC, Girod DA, et al. Balloon pulmonary valvuloplasty: results of the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Am J Cardiol 1990; 65:775.
  35. Garty Y, Veldtman G, Lee K, Benson L. Late outcomes after pulmonary valve balloon dilatation in neonates, infants and children. J Invasive Cardiol 2005; 17:318.
  36. Tabatabaei H, Boutin C, Nykanen DG, et al. Morphologic and hemodynamic consequences after percutaneous balloon valvotomy for neonatal pulmonary stenosis: medium-term follow-up. J Am Coll Cardiol 1996; 27:473.
  37. Rao PS, Galal O, Patnana M, et al. Results of three to 10 year follow up of balloon dilatation of the pulmonary valve. Heart 1998; 80:591.
  38. Poon LK, Menahem S. Pulmonary regurgitation after percutaneous balloon valvoplasty for isolated pulmonary valvar stenosis in childhood. Cardiol Young 2003; 13:444.
  39. Weber HS. Initial and late results after catheter intervention for neonatal critical pulmonary valve stenosis and atresia with intact ventricular septum: a technique in continual evolution. Catheter Cardiovasc Interv 2002; 56:394.
  40. Gentles TL, Lock JE, Perry SB. High pressure balloon angioplasty for branch pulmonary artery stenosis: early experience. J Am Coll Cardiol 1993; 22:867.
  41. Fogelman R, Nykanen D, Smallhorn JF, et al. Endovascular stents in the pulmonary circulation. Clinical impact on management and medium-term follow-up. Circulation 1995; 92:881.
  42. Rosales AM, Lock JE, Perry SB, Geggel RL. Interventional catheterization management of perioperative peripheral pulmonary stenosis: balloon angioplasty or endovascular stenting. Catheter Cardiovasc Interv 2002; 56:272.
  43. Bergersen LJ, Perry SB, Lock JE. Effect of cutting balloon angioplasty on resistant pulmonary artery stenosis. Am J Cardiol 2003; 91:185.
  44. Cunningham JW, McElhinney DB, Gauvreau K, et al. Outcomes after primary transcatheter therapy in infants and young children with severe bilateral peripheral pulmonary artery stenosis. Circ Cardiovasc Interv 2013; 6:460.
  45. Berman W Jr, Fripp RR, Raisher BD, Yabek SM. Significant pulmonary valve incompetence following oversize balloon pulmonary valveplasty in small infants: A long-term follow-up study. Catheter Cardiovasc Interv 1999; 48:61.
  46. DiSessa TG, Alpert BS, Chase NA, et al. Balloon valvuloplasty in children with dysplastic pulmonary valves. Am J Cardiol 1987; 60:405.
  47. Monge MC, Mainwaring RD, Sheikh AY, et al. Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndromes. J Thorac Cardiovasc Surg 2013; 145:476.
  48. Roos-Hesselink JW, Meijboom FJ, Spitaels SE, et al. Long-term outcome after surgery for pulmonary stenosis (a longitudinal study of 22-33 years). Eur Heart J 2006; 27:482.
  49. Earing MG, Connolly HM, Dearani JA, et al. Long-term follow-up of patients after surgical treatment for isolated pulmonary valve stenosis. Mayo Clin Proc 2005; 80:871.
  50. Van Hare GF, Ackerman MJ, Evangelista JA, et al. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 4: Congenital Heart Disease: A Scientific Statement From the American Heart Association and American College of Cardiology. Circulation 2015; 132:e281.