eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Pulmonary Stenosis, Valvar: Differential Diagnoses & Workup

Author: P Syamasundar Rao, MD, Professor of Pediatrics and Medicine, University of Texas-Houston Medical School; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas
Coauthor(s): Kurt Pflieger, MD, FAAP, Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Contributor Information and Disclosures

Updated: Jul 6, 2009

Differential Diagnoses

Aortic Stenosis, Valvar
Tetralogy of Fallot With Pulmonary Atresia
Atrial Septal Defect, Ostium Secundum
Tetralogy of Fallot: Surgical Perspective
Bundle Branch Block, Right
Ventricular Septal Defect, Supracristal
Double Outlet Right Ventricle, Normally Related Great Arteries
Ventricular Septal Defect: Surgical Perspective
Holt-Oram Syndrome
Partial Anomalous Pulmonary Venous Connection
Pulmonary Stenosis, Infundibular

Other Problems to Be Considered

Complex congenital heart disease associated with findings of pulmonary stenosis
Infundibular and/or subpulmonary stenosis
Supravalvar pulmonary stenosis
Double-chambered right ventricle
Syndrome of absent pulmonary valves

Workup

Laboratory Studies

  • Laboratory evaluation is usually not helpful in pulmonary valve stenosis.
  • Hemoglobin and hematocrit measurements in patients with cyanosis may be helpful in that they are increased in patients with right-to-left shunt.
    • The degree of polycythemia is proportional to the degree and duration of right to left shunt.
    • Microcytosis and hypochromia suggest iron deficiency and warrant treatment with iron supplement.
  • Oximetry provides information of potential right-to-left shunting in borderline cyanotic lesions but does not help in identifying the cause of the shunt (pulmonary, interatrial, interventricular, great arterial).
  • Although ABG analysis is usually not needed, one notable exception is the hyperoxia test in the newborn with cyanosis of undetermined origin.28,29
  • The fraction of inspired oxygen (FIO2) of 1 (100% oxygen) generally does not increase the partial pressure of oxygen to more than 100 mm Hg in patients with a cyanotic congenital heart defect (right-to-left intracardiac shunt).

Imaging Studies

  • Chest radiography
    • Chest radiographs reveal a prominent main pulmonary artery segment, but the size of the heart is usually normal (see Media file 2).

      Posteroanterior chest roentgenogram in a patient ...

      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.

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      Posteroanterior chest roentgenogram in a patient ...

      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.

    • Pulmonary vascular markings are usually normal, but they may be decreased in severe pulmonary stenosis with associated right-to-left shunt.
    • Cardiomegaly with right ventricular and right atrial enlargement may be seen in severe valvar pulmonary stenosis, with or without tricuspid insufficiency.
  • Echocardiography
    • The sine qua non of diagnosis is 2-dimensional and Doppler echocardiography.
    • A thickened pulmonary valve with restricted systolic motion (doming) in the parasternal short axis and subcostal views is demonstrated.30
    • Multiple views are used to confirm the absence of coexistent congenital cardiac disease.
    • Dilatation of the main pulmonary artery distal to the stenotic orifice commonly occurs.
    • Markedly thickened, nodular, and immobile pulmonary valve leaflets may be recognized easily and suggest dysplastic pulmonary valves.
    • The pulmonary valve annulus can also be visualized and measured. Measurements can be compared with normal values to determine if the annulus is hypoplastic. Such measurements are also useful in selecting the diameter of the balloon to be used during balloon valvuloplasty.
    • Pulsed, continuous-wave (or high-frequency pulsed), and color Doppler evaluation, in conjunction with 2-dimensional echocardiography, is most useful in confirming the clinical diagnosis and in quantitating the degree of obstruction.31,32,33,34
    • Pulsed Doppler interrogation of the right ventricular outflow tract with sample volume moved across the pulmonary valve demonstrates an abrupt increase in peak Doppler flow velocity, which suggests pulmonary valve obstruction. In addition, the flow pattern in the main pulmonary artery is turbulent instead of laminar. Color Doppler imaging also shows smooth, laminar subpulmonary flow (blue) and some flow acceleration (red) immediately beneath the pulmonary valve, with turbulent (mosaic) flow beginning immediately distal to the pulmonary valve leaflets.
    • Doppler studies can be used to accurately determine the velocity of flow at single or multiple levels, which then can be converted to reproducible pressure gradients by applying the modified Bernoulli equation, as follows: pressure gradient (in millimeters of mercury) = 4 X (velocity in meters/second)2.
    • The use of several views and measurements increases the accuracy of the predicted gradient of peak systolic pressure.
    • Doppler study should be performed when the patient is quiet and in a resting state. Young children and patients who are extremely anxious may have to be mildly sedated.
    • Severe pulmonary stenosis with gradients of more than 50 mm Hg, as diagnosed using a continuous-wave Doppler recording through the pulmonary valve, should be treated with balloon valvuloplasty or surgery. However, Doppler measurements represent peak instantaneous gradients, whereas catheterization gradients are peak-to-peak gradients; recognition of this concept is more important with aortic-valve gradients than with pulmonary-valve gradients. The peak instantaneous gradient was initially though to reflect the peak-to-peak systolic gradient measured during cardiac catheterization. However, this peak instantaneous gradient overestimates the peak-to-peak gradient, presumably because of a pressure-recovery phenomenon.35 In the authors' experience, the catheter peak-to-peak gradient is somewhere in between the Doppler peak instantaneous and mean gradients.
    • Infundibular gradients secondary to severe right ventricular hypertrophy may be present, but these are not usually observed because severe obstruction of the distal pulmonary valve masks infundibular gradients.36,37 However, the infundibular gradients do appear after balloon pulmonary valvuloplasty. A triangular pattern of Doppler signal, similar to that described in subaortic obstruction, is characteristic of infundibular obstruction (see Media file 3).

      Doppler flow velocity recordings from the main pu...

      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.

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      Doppler flow velocity recordings from the main pu...

      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.

    • Pulmonary insufficiency is easily seen during pulsed, continuous-wave, or color Doppler imaging, but is unlikely to be present without previous surgical or balloon pulmonary valvuloplasty.
    • Color Doppler and pulsed Doppler interrogation of the atrial septum is useful and may reveal a left-to-right or right-to-left shunt. Because of high sensitivity of color Doppler imaging, contrast echocardiography is not routinely used to document right-to-left shunt.
    • Most children with pulmonary stenosis do not require evaluation beyond echocardiography.

Other Tests

  • ECG
    • ECG findings are usually normal in mild pulmonary stenosis.
    • Right-axis deviation and right ventricular hypertrophy occur in moderate and severe valvar pulmonary stenosis.
    • The degree of right ventricular hypertrophy is well correlated with the severity of pulmonary stenosis.
    • Right atrial hypertrophy and right ventricular hypertrophy with strain pattern are observed when pulmonary stenosis is severe.
    • A superior QRS axis (left-axis deviation) is seen with dysplastic pulmonary valves and Noonan syndrome.
  • Other noninvasive studies
    • CT and MRI may reveal pulmonary valve stenosis, but the state-of-the-art echocardiography and Doppler studies are more useful than CT or MRI in diagnosing and quantitating pulmonary valve obstruction.1,38
    • Myocardial energy demands and perfusion may be evaluated by performing magnetic resonance spectroscopy and positron emission tomography (PET), respectively. However, the clinical use of these techniques in the management of pulmonic stenosis has not been established.1,38

Procedures

  • Cardiac catheterization and selective cineangiography39
    • Catheterization is not indicated in mild pulmonary stenosis but is essential in severe stenosis and is an integral part of balloon pulmonary valvuloplasty.
    • This procedure is used to confirm the diagnosis; to discern the degree of obstruction; to assess the morphology of the right ventricle, pulmonary outflow tract, and pulmonary arteries; and to exclude other associated cardiac abnormalities.
    • Patients with echocardiographic evidence of clinically significant pulmonary stenosis (50-60 mm Hg) should undergo diagnostic and therapeutic cardiac catheterization with preparation for balloon dilatation of the pulmonary valve.40
  • Monitoring of oxygen saturation: Oxygen-saturation data usually do not show evidence of left-to-right shunts. A right-to-left shunt across the patent foramen ovale (or an atrial defect) may be present in moderate-to-severe obstruction of the pulmonary valve.
  • Monitoring of pressures
    • Right atrial pressure (the particularly a wave) may be increased.
    • Right-ventricular peak systolic pressure is increased; the magnitude of the pressure is proportional to the degree of obstruction.
    • The transpulmonary valve peak-to-peak gradient also indicate the severity of obstruction. A peak-to-peak gradient in excess of 50 mm Hg is usually considered an indication for therapeutic intervention.40
  • Angiography
    • Right ventricular angiography usually reveals thickened and domed leaflets of the pulmonary valve with a thin jet of contrast material across the pulmonary valve (see Media file 4).

      Right ventricular (RV) cineangiogram in lateral v...

      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.

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      Right ventricular (RV) cineangiogram in lateral v...

      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.

    • Enlargement and hypertrophy of the right ventricle and a dilated main pulmonary artery are also seen.
    • In patients with severe or long-standing pulmonary valve obstruction, infundibular constriction may be seen.
    • Additional cineangiograms at other locations are not necessary unless the echocardiographic and hemodynamic data suggest other abnormalities.

Staging

  • The severity of pulmonary valve obstruction may be categorized (staged) as follows, based on peak-to-peak catheter-measured pulmonary valvar gradient:41
    • Trivial – Gradient of less than 25 mm Hg
    • Mild – Gradient of 25-49 mm Hg
    • Moderate – Gradient of 50-79 mm Hg
    • Severe – Gradient of more than 80 mm Hg
  • This severity classification is useful in categorizing patients for natural history studies and in formulating treatment algorithms.

More on Pulmonary Stenosis, Valvar

Overview: Pulmonary Stenosis, Valvar
Differential Diagnoses & Workup: Pulmonary Stenosis, Valvar
Treatment & Medication: Pulmonary Stenosis, Valvar
Follow-up: Pulmonary Stenosis, Valvar
Multimedia: Pulmonary Stenosis, Valvar
References
Further Reading
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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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Keywords

valvar pulmonary stenosis, pulmonary artery, right ventricle, valvular pulmonary stenosis, valvate pulmonary stenosis, pulmonary stenosis with a normal aortic root, pulmonary stenosis with an intact ventricular septum, isolated pulmonary valve stenosis, ventricular septal defect, rheumatic fever, pulmonary valve dysplasia, atrial septal defect, Noonan syndrome, Turner syndrome, rubella syndrome, Williams syndrome, ventricular septal defect, VSD, tetralogy of Fallot, heart failure, patent foramen ovale, underdeveloped right ventricle, hypoplastic right ventricle, syncope, angina pectoris, tricuspid regurgitation, hepatosplenomegaly, treatment, diagnosis

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Contributor Information and Disclosures

Author

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

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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.

CME Editor

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.

 
 
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