Valvar Pulmonary Stenosis 

  • Author: P Syamasundar Rao; Chief Editor: Stuart Berger, MD   more...
 
Updated: Mar 9, 2010
 

Background

Diseases of the pulmonary valve are most often congenital, and only rarely do acquired disorders such as carcinoid and rheumatic fever affect the pulmonary valve.[1] The pulmonary valve may be stenotic or atretic, or the leaflets of the valve may be absent. Pulmonary stenosis may be valvar, supravalvar, or subvalvar (ie, double-chambered right ventricle); it may also be in the branch pulmonary arteries. These lesions are collectively associated with obstruction of the right ventricular outflow tract. In this article, only valvar pulmonary stenosis is reviewed.

A stenotic pulmonary valve may occur without associated congenital abnormalities, although it is most often associated with other structural abnormalities of the heart. To distinguish the former from the latter, terms such as pulmonary stenosis with a normal aortic root or pulmonary stenosis with an intact ventricular septum have been used. However, the term isolated pulmonary valve stenosis is preferred.[1] The term isolated may be used even when a patent foramen ovale or a small atrial septal defect is present. The term congenital need not be used because most are congenital.

Pathologic Anatomy

Pathologic features of the stenotic pulmonary valve vary.[2] The most common pathology is a dome-shaped pulmonary valve. The fused leaflets of the pulmonary valve protrude from their attachment into the pulmonary artery as a conical, windsock-like structure. The size of the pulmonary valve orifice varies from a pinhole to several millimeters. The orifice is most usually central but can be eccentric. Raphae, presumably fused commissures of the valve, extend from the stenotic orifice to a variable distance down into the base of the dome-shaped valve. The number of the raphe may vary from 0-7. Relatively uncommon variants are unicommissural, bicuspid, and tricuspid valves. The valve annulus is abnormal in most cases, and the fibrous back bone is partially or completely lacking; therefore, a true annulus may not be present.[2]

Hypoplasia of the pulmonary valve ring and dysplastic pulmonary valves may be present in a few of patients. Pulmonary valve dysplasia is characterized by thickened, nodular, and redundant valvular leaflets with minimal or no commissural fusion; hypoplasia of the valve ring; and lack of poststenotic dilatation of the pulmonary artery.[3, 4] The obstruction is mainly related to thickened, myxomatous, immobile pulmonary valve cusps and hypoplasia of the valve ring.

Changes secondary to pulmonary valve obstruction occur in the right ventricle and pulmonary artery.[1, 2] Hypertrophy of the right ventricular muscle is proportional to the degree (and perhaps the duration) of obstruction. The muscle hypertrophy is particularly prominent in the infundibular region and may become physiologically important; this appears to be related to the degree and duration of obstruction.[5] Mild dilatation of the right ventricular cavity is present. In extremely severe or critical obstruction, the right ventricular cavity may be markedly dilated. In rare cases, the right ventricle may be hypoplastic.

The main pulmonary artery is dilated in almost all cases. This dilatation is independent of the severity of the pulmonary valve obstruction and presumably related to a high-velocity jet across the stenotic valve.[6, 7] As noted above, such poststenotic dilatation is remarkably absent in patients with dysplastic pulmonary valves.

An interatrial communication, a patent foramen ovale or an atrial septal defect may be present and may be the seat for right-to-left shunt in patients with severe or long-standing pulmonary stenosis.

Pulmonary valve stenosis is the most common cardiac lesion in Noonan syndrome (phenotypically Turner syndrome and genotypically normal [XX or XY]).[8, 9]

Supravalvar pulmonary stenosis is often associated with rubella syndrome and Williams syndrome (ie, elfin facies, supravalvar aortic stenosis, and hypercalcemia with or without mental retardation).

Isolated infundibular or subvalvar pulmonary stenosis is uncommon and usually associated with a ventricular septal defect (VSD), such as in tetralogy of Fallot.

Peripheral pulmonary stenosis is frequently observed in newborns. It is related to relative narrowing of the branch pulmonary arteries and the acute angle of the origin of the branch pulmonary arteries at this age. This represents fetal pattern and, in most cases, resolves over time.

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Pathophysiology

Clinically significant narrowing of a valve or a blood vessel increases pressure proximal to the obstruction. This pressure gradient is necessary to maintain flow across the stenotic site. In pulmonic stenosis, hypertrophy of the right ventricle ensues and maintains this forward flow. The magnitude of right ventricular pressure and the pressure gradient across the pulmonary valve are generally proportional to the degree of obstruction. Under usual circumstances, proportional right ventricular hypertrophy maintains normal pulmonary blood flow. If the normal output is not maintained, right-sided heart failure ensues. This occurs in neonates with critical pulmonary stenosis and in patients with severe obstruction that occurs in childhood or adulthood.

Changes in the geometry of the left ventricle and decreased left ventricular function can also occur.[10, 11] The changes are proportional to the degree of right ventricular hypertrophy; however, they revert to normal after obstruction of the right ventricular outflow tract is relieved.

With increasing right ventricular hypertrophy, right ventricular compliance decreases with a resultant increase in end-diastolic pressure and with prominent a waves in the right atrium. As right atrial pressure rises, a right-to-left shunt may occur if the foramen ovale is patent or if an atrial septal defect is present; this change results in systemic arterial desaturation and clinically discernible cyanosis. This shunting may occur even without measurable elevation of right atrial pressure and is attributable to decreased right ventricular compliance.[12] Such a right-to-left shunt can also occur in patients with an underdeveloped (hypoplastic) right ventricle.[13]

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Epidemiology

Frequency

United States

Pulmonary stenosis represents 8-12% of all congenital heart defects in children.[14, 15] In adults, pulmonary stenosis represents approximately 15% of all congenital heart defects.[16, 17, 18] Isolated valvar pulmonary stenosis with an intact ventricular septum is the second most common congenital cardiac defect in children. It may occur in as many as 50% of all patients with congenital heart disease associated with other congenital cardiac lesions.

Mortality/Morbidity

The severity of the valvar dysplasia determines its related morbidity and mortality. Mild-to-moderate valvar pulmonary stenosis is extremely well tolerated. Severe pulmonary stenosis can be associated with decreased pulmonary flow, right ventricular hypertrophy, early congestive heart failure (CHF) with critical obstructions, and cyanosis. The morbidity formerly associated with surgery is lessened with balloon pulmonary valvuloplasty.

Race

Racial difference in the prevalence of pulmonary stenosis is unlikely.[19]

Sex

The male-to-female ratio is 1:1.[16]

Age

The patient's age at presentation is related to the severity of the obstruction. If the stenosis is severe, patients may present in the neonatal period or in infancy. Patients with mild obstruction may present in childhood with asymptomatic murmurs.

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Contributor Information and Disclosures
Author

P Syamasundar Rao  MD, Professor of Pediatrics, Division of Cardiology, University of Texas Medical School at Houston; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas

P Syamasundar Rao is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, American Pediatric Society, Medical Association of Georgia, Society for Cardiac Angiography and Interventions, Society for Pediatric Research, Southern Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Kurt Pflieger, MD, FAAP  Active Staff, Department of Pediatrics, Lake Pointe Medical Center

Kurt Pflieger, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, American Heart Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA  Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital

Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, Cardiac Electrophysiology Society, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, and Texas Pediatric Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John W Moore, MD, MPH  Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD  Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

References

  1. Rao PS. Pulmonary Valve Disease. In: Alpert JS, Dalen JE, Rahimtoola S, eds. Valvular Heart Disease. 3rd ed. Philadelphia, PA: Lippencott Raven; 2000:339-76.

  2. Gikonyo BM, Lucas RV, Edwards JE. Anatomic features of congenital pulmonary valvar stenosis. Pediatr Cardiol. 1987;8(2):109-16. [Medline].

  3. Koretzky ED, Moller JH, Korns ME, et al. Congenital pulmonary stenosis resulting from dysplasia of valve. Circulation. Jul 1969;40(1):43-53. [Medline].

  4. Jeffery RF, Moller JH, Amplatz K. The dysplastic pulmonary valve: a new roentgenographic entity; with a discussion of the anatomy and radiology of other types of valvular pulmonary stenosis. Am J Roentgenol Radium Ther Nucl Med. Feb 1972;114(2):322-39. [Medline].

  5. Thapar MK, Rao PS. Significance of infundibular obstruction following balloon valvuloplasty for valvar pulmonic stenosis. Am Heart J. Jul 1989;118(1):99-103. [Medline].

  6. Holman E. On circumscribed dilation of an artery immediately distal to a partially occluding band: poststenotic dilatation. Surgery. Jul 1954;36(1):3-24. [Medline].

  7. Rodbard S, Ikeda K, Montes M. An analysis of mechanisms of post stenotic dilatation. Angiology. Jun 1967;18(6):349-67. [Medline].

  8. Abadir S, Edouard T, Julia S. Severe aortic valvar stenosis in familial Noonan syndrome with mutation of the PTPN11 gene. Cardiol Young. Feb 2007;17(1):95-7. [Medline].

  9. Noonan JA. Hypertelorism with Turner phenotype. A new syndrome with associated congenital heart disease. Am J Dis Child. Oct 1968;116(4):373-80. [Medline].

  10. Harinck E, Becker AE, Groot AC, Oppenheimer-Dekker A, Versprille A. The left ventricle in congenital isolated pulmonary valve stenosis. A morphological study. Br Heart J. Apr 1977;39(4):429-35. [Medline].

  11. Sholler GF, Colan SD, Sanders SP. Effect of isolated right ventricular outflow obstruction on left ventricular function in infants. Am J Cardiol. Oct 1 1988;62(10 Pt 1):778-84. [Medline].

  12. Rao PS. Right ventricular filling following balloon pulmonary valvuloplasty. Am Heart J. Apr 1992;123(4 Pt 1):1084-6. [Medline].

  13. Williams JC, Barratt-Boyes BG, Lowe JB. Underdeveloped right ventricle and pulmonary stenosis. Am J Cardiol. Apr 1963;11:458-68. [Medline].

  14. Nadas A. Pulmonary stenosis. In: Fyler DC, ed. Nadas' Pediatric Cardiology. Hanley & Belfus;1992:459-470.

  15. Keith JD, Rowe RD, Vlad P. Heart Disease in Infancy and Childhood. 3rd ed. New York, NY: Macmillan Co; 1978:4-6, 761-88.

  16. Johnson LW, Grossman W, Dalen JE, Dexter L. Pulmonic stenosis in the adult. Long-term follow-up results. N Engl J Med. Dec 7 1972;287(23):1159-63. [Medline].

  17. Abrahams DG, Wood P. Pulmonary stenosis with normal aortic root. Br Heart J. Oct 1951;13(4):519-48. [Medline].

  18. Campbell M. Simple pulmonary stenosis; pulmonary valvular stenosis with a closed ventricular septum. Br Heart J. Jul 1954;16(3):273-300. [Medline].

  19. Rao PS. Demographic features of tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mt. Kisco, NY: Futura; 1992:23-37.

  20. Blount SG Jr, Komesu S, McCord MC. Asymptomatic isolated valvular pulmonary stenosis; diagnosis by clinical methods. N Engl J Med. Jan 1 1953;248(1):5-11. [Medline].

  21. Ainsworth H, Hunt J, Joseph M. Numerical evaluation of facial pattern in children with isolated pulmonary stenosis. Arch Dis Child. Sep 1979;54(9):662-9. [Medline].

  22. Vogelpoel L, Schrire V. Ausculatory and phonocardiographic assessment of pulmonary stenosis with intact ventricular septum. Circulation. 1960;22:55.

  23. Rao PS. Evaluation of cardiac murmurs in children. Indian J Pediatr. Jul-Aug 1991;58(4):471-91. [Medline].

  24. Nora JJ. Multifactorial inheritance hypothesis for etiology of congenital heart disease. Circulation. 1968;38:604-17.

  25. Campbell M. Natural history of cyanotic malformations and comparison of all common cardiac malformations. Br Heart J. Jan 1972;34(1):3-8. [Medline].

  26. Nora JJ, Torres FG, Sinha AK, McNamara DG. Characteristic cardiovascular anomalies of XO Turner syndrome, XX and XY phenotype and XO-XX Turner mosaic. Am J Cardiol. Jun 1970;25(6):639-41. [Medline].

  27. Nora JJ, Nora AH. Recurrence risks in children having one parent with a congenital heart disease. Circulation. Apr 1976;53(4):701-2. [Medline].

  28. Shannon DC, Lusser M, Goldblatt A, Bunnell JB. The cyanotic infant--heart disease or lung disease. N Engl J Med. Nov 9 1972;287(19):951-3. [Medline].

  29. Rao PS, Marino BL, Robertson AF 3rd. Usefulness of continuous positive airway pressure in differential diagnosis of cardiac from pulmonary cyanosis in newborn infants. Arch Dis Child. Jun 1978;53(6):456-60. [Medline].

  30. Weyman AE, Hurwitz RA, Girod DA, Dillon JC, Feigenbaum H, Green D. Cross-sectional echocardiographic visualization of the stenotic pulmonary valve. Circulation. Nov 1977;56(5):769-74. [Medline].

  31. Lima CO, Sahn DJ, Valdes-Cruz LM, et al. Noninvasive prediction of transvalvular pressure gradient in patients with pulmonary stenosis by quantitative two-dimensional echocardiographic Doppler studies. Circulation. Apr 1983;67(4):866-71. [Medline].

  32. Johnson GL, Kwan OL, Handshoe S, Noonan JA, DeMaria AN. Accuracy of combined two-dimensional echocardiography and continuous wave Doppler recordings in the estimation of pressure gradient in right ventricular outlet obstruction. J Am Coll Cardiol. Apr 1984;3(4):1013-8. [Medline].

  33. Currie PJ, Seward JB, Chan KL, et al. Continuous wave Doppler determination of right ventricular pressure: a simultaneous Doppler-catheterization study in 127 patients. J Am Coll Cardiol. Oct 1985;6(4):750-6. [Medline].

  34. Rao PS. Doppler ultrasound in the prediction of transvalvar pressure gradients in patients with valvar pulmonary stenosis. Int J Cardiol. May 1987;15(2):195-203. [Medline].

  35. Singh GK, Singh GK, Balfour IC, et al. Lesion Specific Pressure Recovery Phenomenon in Pediatric Patients: A Simultaneous Doppler and Catheter Correlative Study. Poster presentation at the 52nd Annual Scientific Session of the American College of Cardiology, Chicago, IL, March 30 - April 2, 2003,. J Am Coll Cardiol. 2003;41:493A.

  36. Silove ED, Vogel JH, Grover RF. The pressure gradient in ventricular outflow obstruction: influence of peripheral resistance. Cardiovasc Res. Jul 1968;2(3):234-42. [Medline].

  37. Rao PS, Linde LM. Pressure and energy in cardiovascular chambers. Chest. 1974;66:176-8.

  38. Balfour IC, Rao PS. Pulmonary Stenosis. Curr Treat Options Cardiovasc Med. Dec 2000;2(6):489-498. [Medline].

  39. Krasemann T. [Catheter interventions for congenital heart disease]. Herz. Dec 2008;33(8):592-600. [Medline].

  40. Rao PS. Indications for balloon pulmonary valvuloplasty. Am Heart J. Dec 1988;116(6 Pt 1):1661-2. [Medline].

  41. Nugent EW, Freedom RM, Nora JJ, et al. Clinical course in pulmonary stenosis. Circulation. Aug 1977;56(1 Suppl):I38-47. [Medline].

  42. Drossner DM, Mahle WT. A management strategy for mild valvar pulmonary stenosis. Pediatr Cardiol. May 2008;29(3):649-52. [Medline].

  43. Herberg U, Goltz D, Weiss H, Gembruch U, Breuer J. Combined Pulmonary and Aortic Valve Stenosis - Prenatal Diagnosis and Postnatal Interventional Therapy. Neonatology. May 12 2009;96(4):244-247. [Medline].

  44. Galindo A, Gutierrez-Larraya F, Velasco JM, de la Fuente P. Pulmonary balloon valvuloplasty in a fetus with critical pulmonary stenosis/atresia with intact ventricular septum and heart failure. Fetal Diagn Ther. 2006;21(1):100-4. [Medline].

  45. Lange PE, Onnasch DGW, Heintzen PH. Valvular pulmonary stenosis: natural history and right ventricular function. In: Doyle EF, et al, eds. Pediatric Cardiology. New York, NY: Springer-Verlag; 1986:395-8.

  46. Krabill KA, Wang Y, Einzig S, Moller JH. Rest and exercise hemodynamics in pulmonary stenosis: comparison of children and adults. Am J Cardiol. Aug 1 1985;56(4):360-5. [Medline].

  47. Rubio-Alvarez V, Limon-Lason R, Soni J. Valvulotomias intracardiacas por medio de un cateter. Arch Inst Cordiol Mexico. 1952;23:183-92.

  48. Semb BK, Tjonneland S, Stake G, Aabyholm G. "Balloon valvulotomy" of congenital pulmonary valve stenosis with tricuspid valve insufficiency. Cardiovasc Radiol. Nov 1979;2(4):239-41. [Medline].

  49. Kan JS, White RI, Mitchell SE, Gardner TJ. Percutaneous balloon valvuloplasty: a new method for treating congenital pulmonary-valve stenosis. N Engl J Med. Aug 26 1982;307(9):540-2. [Medline].

  50. Dotter CT, Judkins MP. Transluminal treatment of arteriosclerotic obstruction: description of a new technique and a preliminary report of its application. Circulation. 1967;30:654.

  51. Rao PS. Role of Interventional Cardiology In Neonates: Part I. Non-Surgical Atrial Septostomy. Congenital Cardiol Today. 2007;5(12):1-12.

  52. Rao PS. Balloon pulmonary valvuloplasty for isolated pulmonic stenosis. In: Transcatheter Therapy in Pediatric Cardiology. Wiley-Liss; 1993:59-104.

  53. Rao PS. Transcatheter treatment of pulmonary outflow tract obstruction: a review. Prog Cardiovasc Dis. Sep-Oct 1992;35(2):119-58. [Medline].

  54. Rao PS. Influence of balloon size on short-term and long-term results of balloon pulmonary valvuloplasty. Tex Heart Inst J. Mar 1987;14(1):57-61. [Medline].

  55. Rao PS. Percutaneous balloon pulmonary valvuloplasty: state of the art. Catheter Cardiovasc Interv. Apr 1 2007;69(5):747-63. [Medline].

  56. Rao PS. Pulmonary valve stenosis. In: Sievert H, Qureshi SA, Wilson N, Hijazi Z, eds. Percutaneous Interventions in Congenital Heart Disease,. Oxford, UK: Informa Health Care; 2007:185-95.

  57. Radtke W, Keane JF, Fellows KE, et al. Percutaneous balloon valvotomy of congenital pulmonary stenosis using oversized balloons. J Am Coll Cardiol. Oct 1986;8(4):909-15. [Medline].

  58. Rao PS. Further observations on the effect of balloon size on the short term and intermediate term results of balloon dilatation of the pulmonary valve. Br Heart J. Dec 1988;60(6):507-11. [Medline].

  59. Berman W, 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. Sep 1999;48(1):61-5; discussion 66. [Medline].

  60. Rao PS. Late pulmonary insufficiency after balloon dilatation of the pulmonary valve [letter]. Cathet Cardiovasc Intervent. 2000;49:118-9.

  61. Rao PS. How big a balloon and how many balloons for pulmonary valvuloplasty?. Am Heart J. Aug 1988;116(2 Pt 1):577-80. [Medline].

  62. Rao PS, Fawzy ME. Double balloon technique for percutaneous balloon pulmonary valvuloplasty: comparison with single balloon technique. Intervent Cardiol. 1988;1:257.

  63. Bahl VK, Chandra S, Goel A, et al. Versatility of Inoue balloon catheter. Int J Cardiol. Mar 1997;59(1):75-83. [Medline].

  64. Abels JE. Balloon catheters and transluminal dilatation: technical considerations. Am J Roentgenol. 1980;135:901.

  65. Rao PS. Balloon angioplasty and valvuloplasty in infants, children, and adolescents. Curr Probl Cardiol. Aug 1989;14(8):417-97. [Medline].

  66. Walls JT, Lababidi Z, Curtis JJ, Silver D. Assessment of percutaneous balloon pulmonary and aortic valvuloplasty. J Thorac Cardiovasc Surg. Sep 1984;88(3):352-6. [Medline].

  67. Ettedgui JA, Ho SY, Tynan M, Jones OD, Martin RP, Baker EJ. The pathology of balloon pulmonary valvoplasty. Int J Cardiol. Sep 1987;16(3):285-93. [Medline].

  68. Burrows PE, Benson LN, Smallhorn JS, Moes CA, Freedom RM, Burrows FA. Angiographic features associated with percutaneous balloon valvotomy for pulmonary valve stenosis. Cardiovasc Intervent Radiol. Apr 1988;11(2):111-6. [Medline].

  69. Benson LN, Smallhorn JS, Freedom RM, Trusler GA, Rowe RD. Pulmonary valve morphology after balloon dilatation of pulmonary valve stenosis. Cathet Cardiovasc Diagn. 1985;11(2):161-6. [Medline].

  70. Rao PS. Balloon dilatation in infants and children with dysplastic pulmonary valves: short-term and intermediate-term results. Am Heart J. Nov 1988;116(5 Pt 1):1168-73. [Medline].

  71. Marantz PM, Huhta JC, Mullins CE, Murphy DJ Jr, Nihill MR, Ludomirsky A. Results of balloon valvuloplasty in typical and dysplastic pulmonary valve stenosis: Doppler echocardiographic follow-up. J Am Coll Cardiol. Aug 1988;12(2):476-9. [Medline].

  72. Rao PS. Balloon pulmonary valvuloplasty: a review. Clin Cardiol. Feb 1989;12(2):55-74. [Medline].

  73. Rao PS. Pulmonary valve in children. In: Sigwart U, Bertrand M, Serruys PW, eds. Handbook of Cardiovascular Interventions. New York, NY: Churchill Livingstone; 1996:273-310.

  74. Rao PS, Galal O, Patnana M. Results of three to 10 year follow up of balloon dilatation of the pulmonary valve. Heart. Dec 1998;80(6):591-5. [Medline].

  75. Fontes VF, Esteves CA, Sousa JE, et al. Regression of infundibular hypertrophy after pulmonary valvuloplasty for pulmonic stenosis. Am J Cardiol. Nov 1 1988;62(13):977-9. [Medline].

  76. Engle ME, Holswade GR, Goldberg HP, Lukas DS, Glenn F. Regression after open valvotomy of infundibular stenosis accompanying severe valvar pulmonary stenosis. Circulation. 1958;17:862.

  77. Johnson AM. Hypertonic infundibular stenosis complicating simple pulmonary valve stenosis. Br Heart J. 1959;21:429.

  78. Gilbert JW, Morrow AG, Talbert JL. The surgical significance of hypertophic infundibular obstruction accompanying valvular pulmonic stenosis. J Thorac Cardiovasc Surg. Oct 1963;46:457-67. [Medline].

  79. Rao PS. Long-term follow-up results after balloon dilatation of pulmonic stenosis, aortic stenosis, and coarctation of the aorta: a review. Prog Cardiovasc Dis. Jul-Aug 1999;42(1):59-74. [Medline].

  80. Rao PS, Solymar L. Electrocardiographic changes following balloon dilatation of valvar pulmonic stenosis. J Intervent Cardiol. 1988;1:189.

  81. Rao PS. Value of echo-Doppler studies in the evaluation of the results of balloon pulmonary valvuloplasty. J Cardiovasc Ultrasonography. 1986;309.

  82. Rao PS, Thapar MK, Kutayli F, Carey P. Causes of restenosis after balloon valvuloplasty for valvular pulmonary stenosis. Am J Cardiol. Nov 1 1988;62(13):979-82. [Medline].

  83. McCrindle BW. Independent predictors of long-term results after balloon pulmonary valvuloplasty. Valvuloplasty and Angioplasty of Congenital Anomalies (VACA) Registry Investigators. Circulation. Apr 1994;89(4):1751-9. [Medline].

  84. Rao PS, Galal O, Wilson AD. Feasibility and effectiveness of repeated balloon dilatation of restenosed congenital obstructions after previous balloon valvuloplasty/angioplasty. Am Heart J. Aug 1996;132(2 Pt 1):403-7. [Medline].

  85. O'Connor BK, Beekman RH, Lindauer A, Rocchini A. Intermediate-term outcome after pulmonary balloon valvuloplasty: comparison with a matched surgical control group. J Am Coll Cardiol. Jul 1992;20(1):169-73. [Medline].

  86. Rao PS. Balloon valvuloplasty in the neonate with critical pulmonary stenosis. J Am Coll Cardiol. Feb 1996;27(2):479-80. [Medline].

  87. Jureidini SB, Rao PS. Critical Pulmonary Stenosis in the Neonate: Role of Transcatheter Management. J Invasive Cardiol. Sep 1996;8(7):326-331. [Medline].

  88. 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. Feb 1996;27(2):473-8. [Medline].

  89. Sellors TH. Surgery of pulmonary stenosis; a case in which the pulmonary valve was successfully divided. Lancet. Jun 26 1948;1(6513):988. [Medline].

  90. Brock RC. Pulmonary valvotomy for relief of congenital stenosis: report of 3 cases. Br Med J. 1948;1:1121.

  91. Castenada AR, Jonas RA, Meyer JE. Surgery for infants with congenital heart defects In: Cardiac Surgery of the Neonate and Infant. 1st ed. 1993:1013-1035.

  92. McNamara DG, Latson LA. Long-term follow-up of patients with malformations for which definitive surgical repair has been available for 25 years or more. Am J Cardiol. Sep 1982;50(3):560-8. [Medline].

  93. Rao PS, Liebman J, Borkat G. Right ventricular growth in a case of pulmonic stenosis with intact ventricular septum and hypoplastic right ventricle. Circulation. Feb 1976;53(2):389-94. [Medline].

  94. Freedom RM. Pulmonary Atresia with Intact Ventricular Septum. Mount Kisco, NY: Futura Publishing, Co; 1989:1-257.

  95. Rao PS. Comprehensive management of pulmonary atresia with intact ventricular septum. Ann Thorac Surg. Oct 1985;40(4):409-13. [Medline].

  96. [Guideline] Ho VB, Yucel EK, Khan A, et al. Suspected congenital heart disease in the adult. American College of Radiology. 2007;[Full Text].

  97. [Guideline] Bonow RO, Carabello BA, Chatterjee K, et al. ACC/AHA 2006 guideline for the management of patients with valvular heart disease. J Am Coll Cardiol. Aug 2006;48(3):e1-148. [Full Text].

  98. [Guideline] Vahanian A, Baumgartner H, Bax J, Butchart E, et al. Guidelines on the management of valvular heart disease: The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology. Eur Heart J. Jan 2007;28(2):230-68. [Medline].

 
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In valvar pulmonic stenosis, the severity of obstruction may be judged by auscultatory findings. In mild stenosis, the ejection click (EC) is clearly separated from the first heart sound (S1). The murmur starts with the click, peaks in early systole, and ends way before the aortic component of the second heart sound (A2) The pulmonary component of the second heart sound (P2) is normal to increased in intensity. In moderate pulmonic stenosis, the click is closer to the first heart sound, the ejection murmur peaks later in the systole and the murmur reaches the A2, and the second heart sound is widely split with soft pulmonary component. In severe valvar obstruction, the click is either absent or occurs so close to S1 that it cannot be heard separately, and the murmur peaks late in systole and extends beyond the A2. The second heart sound is widely split with an extremely soft or inaudible P2. Reproduced from Rao PS: Evaluation of cardiac murmur in children. Indian J Pediatr 1991 Jul-Aug; 58(4): 471-91.
Posteroanterior chest roentgenogram in a patient with valvar pulmonic stenosis showing normal-sized heart with normal pulmonary vascular markings. Note prominent main pulmonary artery (arrow). Reproduced with permission from Rao PS: Diagnosis and management of acyanotic heart disease: Part I – Obstructive lesions. Indian J Pediatr 2005; 72: 495-502.
Doppler flow velocity recordings from the main pulmonary artery prior to (left) and 1 day (center) and 10 months (right) after successful balloon pulmonary valvuloplasty. Note that no significant fall in the peak flow velocity is present on the day after balloon procedure, but a characteristic triangular pattern is present, indicative of infundibular obstruction. At 10-month follow-up, the flow velocity decreased, suggesting resolution of infundibular obstruction. Reproduced with permission from Thapar MK: Significance of infundibular obstruction following balloon valvuloplasty for valvar pulmonic stenosis. Am Heart J 1989; Jul; 118(1): 99-103.
Right ventricular (RV) cineangiogram in lateral view in a child with valvar pulmonary stenosis demonstrating thickened and domed pulmonary valve leaflets and poststenotic dilatation of the pulmonary artery (PA). Reproduced with permission from Rao PS: Diagnosis and management of acyanotic heart disease: Part I – Obstructive lesions. Indian J Pediatr 2005; 72: 495-502.
Selected cineradiographic frames of a balloon dilatation catheter placed across a stenotic pulmonary valve. Note "waisting" of the balloon during the initial phases of the balloon inflation (A), which was almost completely abolished during the later phases of balloon inflation (B). Reproduced from Rao PS: Balloon pulmonary valvuloplasty for isolated pulmonic stenosis. In: Rao PS, ed: Transcatheter Therapy in Pediatric Cardiology New York, NY: Wiley-Liss; 1993: 59-104.
Selected frames from lateral view of the right ventricular (RV) cineangiogram showing severe infundibular stenosis (A) immediately following balloon valvuloplasty (corresponding Media file 3, center). At 10 months after balloon valvuloplasty, the right ventricular outflow tract (B) is wide open and corresponds to Media file 3, right. Peak-to-peak pulmonary valve gradient was 20 mm Hg and no infundibular gradient was present. PA = Pulmonary artery. Reproduced with permission from Thapar MK: Significance of infundibular obstruction following balloon valvuloplasty for valvar pulmonic stenosis. Am Heart J 1989; Jul; 118(1): 99-103.
 
 
 
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