Guidelines Summary

2018 American Heart Association/American College of Cardiology (AHA/ACC) guidelines

Class I and IIa recommendations for valvar pulmonary stenosis^[47]

Balloon valvuloplasty is recommended in adults with moderate or severe valvar pulmonary stenosis and otherwise unexplained symptoms of heart failure, cyanosis from interatrial right-to-left communication, and/or exercise intolerance (class I, level of evidence [LOE]: B).

Surgical repair is recommended in adults with moderate or severe valvular pulmonary stenosis and otherwise unexplained symptoms of heart failure, cyanosis, and/or exercise intolerance who are not candidates for balloon valvuloplasty or for whom balloon valvuloplasty was unsuccessful (class I, LOE: B).

In asymptomatic adults with severe valvar pulmonary stenosis, intervention is reasonable (class IIa, LOE: C).

Class I and IIb recommendations for isolated pulmonary regurgitation (PR) after repair of pulmonary stenosis^[47]

Pulmonary valve replacement is recommended in symptomatic patients with moderate or greater PR resulting from treated isolated pulmonary stenosis, with right ventricular (RV) dilation or RV dysfunction (class I, LOE: C).

For asymptomatic patients with residual PR as a result of treatment of isolated pulmonary stenosis with a dilated RV, serial follow-up is recommended (class I, LOE: C).

In asymptomatic patients with moderate or greater PR from treatment of isolated pulmonary stenosis with progressive RV dilation and/or RV dysfunction, pulmonary valve replacement may be reasonable (class IIb, LOE: C).

2020 ESC guidelines

The European Society of Cardiology (ESC) updated their 2010 guidelines on the management of adult congenital heart disease (ACHD) in 2020.^{[48, 49]} Their class I and III recommendations for RV outflow tract obstruction (OTO) are outlined below.

In valvular pulmonary stenosis, balloon valvuloplasty is the intervention of choice, if anatomically suitable.

As long as no valve replacement is required, RVOTO intervention at any level is recommended regardless of symptoms when the stenosis is severe (Doppler peak gradient >64 mmHg).

If surgical valve replacement is the only option, it is indicated in (1) symptomatic patients with severe stenosis; or (2) asymptomatic patients with severe stenosis in the presence of ≥1 of the following:

Objective decrease in exercise capacity
Falling RV function and/or progression of tricuspid regurgitation (TR) to at least moderate
RV systolic pressure (SP) >80 mmHg
Right-to-left (RL) shunting via an atrial septal defect (ASD) or ventricular septal defect (VSD)

Medication

Rao PS. Pulmonary Valve Disease. Alpert JS, Dalen JE, Rahimtoola S, eds. Valvular Heart Disease. 3rd ed. Philadelphia, PA: Lippencott Raven; 2000. 339-76.
Gikonyo BM, Lucas RV, Edwards JE. Anatomic features of congenital pulmonary valvar stenosis. Pediatr Cardiol. 1987. 8(2):109-16. [Medline].
Koretzky ED, Moller JH, Korns ME, et al. Congenital pulmonary stenosis resulting from dysplasia of valve. Circulation. 1969 Jul. 40(1):43-53. [Medline].
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. 1972 Feb. 114(2):322-39. [Medline].
Thapar MK, Rao PS. Significance of infundibular obstruction following balloon valvuloplasty for valvar pulmonic stenosis. Am Heart J. 1989 Jul. 118(1):99-103. [Medline].
Holman E. On circumscribed dilation of an artery immediately distal to a partially occluding band: poststenotic dilatation. Surgery. 1954 Jul. 36(1):3-24. [Medline].
Rodbard S, Ikeda K, Montes M. An analysis of mechanisms of post stenotic dilatation. Angiology. 1967 Jun. 18(6):349-67. [Medline].
Abadir S, Edouard T, Julia S. Severe aortic valvar stenosis in familial Noonan syndrome with mutation of the PTPN11 gene. Cardiol Young. 2007 Feb. 17(1):95-7. [Medline].
Noonan JA. Hypertelorism with Turner phenotype. A new syndrome with associated congenital heart disease. Am J Dis Child. 1968 Oct. 116(4):373-80. [Medline].
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. 1977 Apr. 39(4):429-35. [Medline]. [Full Text].
Sholler GF, Colan SD, Sanders SP. Effect of isolated right ventricular outflow obstruction on left ventricular function in infants. Am J Cardiol. 1988 Oct 1. 62(10 Pt 1):778-84. [Medline].
Rao PS. Right ventricular filling following balloon pulmonary valvuloplasty. Am Heart J. 1992 Apr. 123(4 Pt 1):1084-6. [Medline].
Williams JC, Barratt-Boyes BG, Lowe JB. Underdeveloped right ventricle and pulmonary stenosis. Am J Cardiol. 1963 Apr. 11:458-68. [Medline].
Nora JJ. Multifactorial inheritance hypothesis for etiology of congenital heart disease. Circulation. 1968. 38:604-17.
Campbell M. Natural history of cyanotic malformations and comparison of all common cardiac malformations. Br Heart J. 1972 Jan. 34(1):3-8. [Medline]. [Full Text].
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. 1970 Jun. 25(6):639-41. [Medline].
Nora JJ, Nora AH. Recurrence risks in children having one parent with a congenital heart disease. Circulation. 1976 Apr. 53(4):701-2. [Medline].
Nadas A. Pulmonary stenosis. In: Fyler DC, ed. Nadas' Pediatric Cardiology. Hanley & Belfus. 1992:459-470.
Keith JD, Rowe RD, Vlad P. Heart Disease in Infancy and Childhood. 3rd ed. New York, NY: Macmillan Co; 1978. 4-6, 761-88.
Johnson LW, Grossman W, Dalen JE, Dexter L. Pulmonic stenosis in the adult. Long-term follow-up results. N Engl J Med. 1972 Dec 7. 287(23):1159-63. [Medline].
Abrahams DG, Wood P. Pulmonary stenosis with normal aortic root. Br Heart J. 1951 Oct. 13(4):519-48. [Medline]. [Full Text].
Campbell M. Simple pulmonary stenosis; pulmonary valvular stenosis with a closed ventricular septum. Br Heart J. 1954 Jul. 16(3):273-300. [Medline]. [Full Text].
Rao PS. Demographic features of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mt. Kisco, NY: Futura; 1992. 23-37.
Blount SG Jr, Komesu S, McCord MC. Asymptomatic isolated valvular pulmonary stenosis; diagnosis by clinical methods. N Engl J Med. 1953 Jan 1. 248(1):5-11. [Medline].
Ainsworth H, Hunt J, Joseph M. Numerical evaluation of facial pattern in children with isolated pulmonary stenosis. Arch Dis Child. 1979 Sep. 54(9):662-9. [Medline].
Vogelpoel L, Schrire V. Ausculatory and phonocardiographic assessment of pulmonary stenosis with intact ventricular septum. Circulation. 1960. 22:55.
Rao PS. Evaluation of cardiac murmurs in children. Indian J Pediatr. 1991 Jul-Aug. 58(4):471-91. [Medline].
Shannon DC, Lusser M, Goldblatt A, Bunnell JB. The cyanotic infant--heart disease or lung disease. N Engl J Med. 1972 Nov 9. 287(19):951-3. [Medline].
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. 1978 Jun. 53(6):456-60. [Medline].
Nugent EW, Freedom RM, Nora JJ, et al. Clinical course in pulmonary stenosis. Circulation. 1977 Aug. 56(1 Suppl):I38-47. [Medline].
Weyman AE, Hurwitz RA, Girod DA, Dillon JC, Feigenbaum H, Green D. Cross-sectional echocardiographic visualization of the stenotic pulmonary valve. Circulation. 1977 Nov. 56(5):769-74. [Medline].
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. 1983 Apr. 67(4):866-71. [Medline].
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. 1984 Apr. 3(4):1013-8. [Medline].
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. 1985 Oct. 6(4):750-6. [Medline].
Rao PS. Doppler ultrasound in the prediction of transvalvar pressure gradients in patients with valvar pulmonary stenosis. Int J Cardiol. 1987 May. 15(2):195-203. [Medline].
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.
Silove ED, Vogel JH, Grover RF. The pressure gradient in ventricular outflow obstruction: influence of peripheral resistance. Cardiovasc Res. 1968 Jul. 2(3):234-42. [Medline].
Rao PS, Linde LM. Pressure and energy in cardiovascular chambers. Chest. 1974. 66:176-8.
Balfour IC, Rao PS. Pulmonary Stenosis. Curr Treat Options Cardiovasc Med. 2000 Dec. 2(6):489-498. [Medline].
Krasemann T. [Catheter interventions for congenital heart disease]. Herz. 2008 Dec. 33(8):592-600. [Medline].
Rao PS. Indications for balloon pulmonary valvuloplasty. Am Heart J. 1988 Dec. 116(6 Pt 1):1661-2. [Medline].
Drossner DM, Mahle WT. A management strategy for mild valvar pulmonary stenosis. Pediatr Cardiol. 2008 May. 29(3):649-52. [Medline].
Herberg U, Goltz D, Weiss H, Gembruch U, Breuer J. Combined Pulmonary and Aortic Valve Stenosis - Prenatal Diagnosis and Postnatal Interventional Therapy. Neonatology. 2009 May 12. 96(4):244-247. [Medline].
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].
Lange PE, Onnasch DGW, Heintzen PH. Valvular pulmonary stenosis: natural history and right ventricular function. Doyle EF, et al, eds. Pediatric Cardiology. New York, NY: Springer-Verlag; 1986. 395-8.
Krabill KA, Wang Y, Einzig S, Moller JH. Rest and exercise hemodynamics in pulmonary stenosis: comparison of children and adults. Am J Cardiol. 1985 Aug 1. 56(4):360-5. [Medline].
[Guideline] Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019 Apr 2. 139 (14):e698-e800. [Medline]. [Full Text].
[Guideline] Baumgartner H, De Backer J, Babu-Narayan SV, et al, for the ESC Scientific Document Group . 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2020 Aug 29. ehaa554. [Medline]. [Full Text].
Baumgartner H, De Backer J. The ESC clinical practice guidelines for the management of adult congenital heart disease 20. Eur Heart J. 2020 Nov 14. 41 (43):4153-4. [Medline]. [Full Text].
Rubio-Alvarez V, Limon-Lason R, Soni J. Valvulotomias intracardiacas por medio de un cateter. Arch Inst Cordiol Mexico. 1952. 23:183-92.
Semb BK, Tjonneland S, Stake G, Aabyholm G. "Balloon valvulotomy" of congenital pulmonary valve stenosis with tricuspid valve insufficiency. Cardiovasc Radiol. 1979 Nov. 2(4):239-41. [Medline].
Kan JS, White RI, Mitchell SE, Gardner TJ. Percutaneous balloon valvuloplasty: a new method for treating congenital pulmonary-valve stenosis. N Engl J Med. 1982 Aug 26. 307(9):540-2. [Medline].
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.
Rao PS. Role of Interventional Cardiology In Neonates: Part I. Non-Surgical Atrial Septostomy. Congenital Cardiol Today. 2007. 5(12):1-12.
Kilic T, Sahin T, Karauzum K, Ural E, Pedersen WR. New pulmonary valvuloplasty technique by use of an hourglass-shaped balloon in 3 adults with severe pulmonary valve stenosis. Tex Heart Inst J. 2018 Apr. 45 (2):76-83. [Medline]. [Full Text].
Rao PS. Balloon pulmonary valvuloplasty for isolated pulmonic stenosis. Transcatheter Therapy in Pediatric Cardiology. Wiley-Liss; 1993. 59-104.
Rao PS. Transcatheter treatment of pulmonary outflow tract obstruction: a review. Prog Cardiovasc Dis. 1992 Sep-Oct. 35(2):119-58. [Medline].
Rao PS. Influence of balloon size on short-term and long-term results of balloon pulmonary valvuloplasty. Tex Heart Inst J. 1987 Mar. 14(1):57-61. [Medline].
Rao PS. Percutaneous balloon pulmonary valvuloplasty: state of the art. Catheter Cardiovasc Interv. 2007 Apr 1. 69(5):747-63. [Medline].
Rao PS. Pulmonary valve stenosis. Sievert H, Qureshi SA, Wilson N, Hijazi Z, eds. Percutaneous Interventions in Congenital Heart Disease,. Oxford, UK: Informa Health Care; 2007. 185-95.
Radtke W, Keane JF, Fellows KE, et al. Percutaneous balloon valvotomy of congenital pulmonary stenosis using oversized balloons. J Am Coll Cardiol. 1986 Oct. 8(4):909-15. [Medline].
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. 1988 Dec. 60(6):507-11. [Medline].
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. 1999 Sep. 48(1):61-5; discussion 66. [Medline].
Rao PS. Late pulmonary insufficiency after balloon dilatation of the pulmonary valve [letter]. Cathet Cardiovasc Intervent. 2000. 49:118-9.
Rao PS. How big a balloon and how many balloons for pulmonary valvuloplasty?. Am Heart J. 1988 Aug. 116(2 Pt 1):577-80. [Medline].
Rao PS, Fawzy ME. Double balloon technique for percutaneous balloon pulmonary valvuloplasty: comparison with single balloon technique. Intervent Cardiol. 1988. 1:257.
Bahl VK, Chandra S, Goel A, et al. Versatility of Inoue balloon catheter. Int J Cardiol. 1997 Mar. 59(1):75-83. [Medline].
Rao PS. Balloon dilatation in infants and children with dysplastic pulmonary valves: short-term and intermediate-term results. Am Heart J. 1988 Nov. 116(5 Pt 1):1168-73. [Medline].
Moguillansky D, Schneider HE, Rome JJ, Kreutzer J. Role of high-pressure balloon valvotomy for resistant pulmonary valve stenosis. Congenit Heart Dis. 2010 Mar-Apr. 5(2):134-40. [Medline].
Abels JE. Balloon catheters and transluminal dilatation: technical considerations. Am J Roentgenol. 1980. 135:901.
Rao PS. Balloon angioplasty and valvuloplasty in infants, children, and adolescents. Curr Probl Cardiol. 1989 Aug. 14(8):417-97. [Medline].
Walls JT, Lababidi Z, Curtis JJ, Silver D. Assessment of percutaneous balloon pulmonary and aortic valvuloplasty. J Thorac Cardiovasc Surg. 1984 Sep. 88(3):352-6. [Medline].
Ettedgui JA, Ho SY, Tynan M, Jones OD, Martin RP, Baker EJ. The pathology of balloon pulmonary valvoplasty. Int J Cardiol. 1987 Sep. 16(3):285-93. [Medline].
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. 1988 Apr. 11(2):111-6. [Medline].
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].
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. 1988 Aug. 12(2):476-9. [Medline].
Rao PS. Balloon pulmonary valvuloplasty: a review. Clin Cardiol. 1989 Feb. 12(2):55-74. [Medline].
Rao PS. Pulmonary valve in children. Sigwart U, Bertrand M, Serruys PW, eds. Handbook of Cardiovascular Interventions. New York, NY: Churchill Livingstone; 1996. 273-310.
Rao PS, Galal O, Patnana M. Results of three to 10 year follow up of balloon dilatation of the pulmonary valve. Heart. 1998 Dec. 80(6):591-5. [Medline].
Fontes VF, Esteves CA, Sousa JE, et al. Regression of infundibular hypertrophy after pulmonary valvuloplasty for pulmonic stenosis. Am J Cardiol. 1988 Nov 1. 62(13):977-9. [Medline].
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.
Johnson AM. Hypertonic infundibular stenosis complicating simple pulmonary valve stenosis. Br Heart J. 1959. 21:429.
Gilbert JW, Morrow AG, Talbert JL. The surgical significance of hypertophic infundibular obstruction accompanying valvular pulmonic stenosis. J Thorac Cardiovasc Surg. 1963 Oct. 46:457-67. [Medline].
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. 1999 Jul-Aug. 42(1):59-74. [Medline].
Rao PS, Solymar L. Electrocardiographic changes following balloon dilatation of valvar pulmonic stenosis. J Intervent Cardiol. 1988. 1:189.
Rao PS. Value of echo-Doppler studies in the evaluation of the results of balloon pulmonary valvuloplasty. J Cardiovasc Ultrasonography. 1986. 309:
Rao PS, Thapar MK, Kutayli F, Carey P. Causes of restenosis after balloon valvuloplasty for valvular pulmonary stenosis. Am J Cardiol. 1988 Nov 1. 62(13):979-82. [Medline].
McCrindle BW. Independent predictors of long-term results after balloon pulmonary valvuloplasty. Valvuloplasty and Angioplasty of Congenital Anomalies (VACA) Registry Investigators. Circulation. 1994 Apr. 89(4):1751-9. [Medline].
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. 1996 Aug. 132(2 Pt 1):403-7. [Medline].
Galian-Gay L, Gordon B, Marsal JR, et al. Determinants of long-term outcome of repaired pulmonary valve stenosis [English, Spanish]. Rev Esp Cardiol (Engl Ed). 2020 Feb. 73 (2):131-8. [Medline]. [Full Text].
Merino-Ingelmo R, Santos-de Soto J, Coserria-Sánchez F, Descalzo-Señoran A, Valverde-Pérez I. Long-term results of percutaneous balloon valvuloplasty in pulmonary valve stenosis in the pediatric population. Rev Esp Cardiol (Engl Ed). 2014 May. 67(5):374-9. [Medline].
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. 1992 Jul. 20(1):169-73. [Medline].
Voet A, Rega F, de Bruaene AV, Troost E, Gewillig M, Van Damme S, et al. Long-term outcome after treatment of isolated pulmonary valve stenosis. Int J Cardiol. 2012 Apr 5. 156(1):11-5. [Medline].
Rao PS. Balloon valvuloplasty in the neonate with critical pulmonary stenosis. J Am Coll Cardiol. 1996 Feb. 27(2):479-80. [Medline].
Jureidini SB, Rao PS. Critical Pulmonary Stenosis in the Neonate: Role of Transcatheter Management. J Invasive Cardiol. 1996 Sep. 8(7):326-331. [Medline].
Tabatabaei H, Boutin C, Nykanen DG, Freedom RM, Benson LN. Morphologic and hemodynamic consequences after percutaneous balloon valvotomy for neonatal pulmonary stenosis: medium-term follow-up. J Am Coll Cardiol. 1996 Feb. 27(2):473-8. [Medline].
Sellors TH. Surgery of pulmonary stenosis; a case in which the pulmonary valve was successfully divided. Lancet. 1948 Jun 26. 1(6513):988. [Medline].
Brock RC. Pulmonary valvotomy for relief of congenital stenosis: report of 3 cases. Br Med J. 1948. 1:1121.
Castenada AR, Jonas RA, Meyer JE. Surgery for infants with congenital heart defects. Cardiac Surgery of the Neonate and Infant. 1993. 1st ed: 1013-35.
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. 1982 Sep. 50(3):560-8. [Medline].
Rao PS, Liebman J, Borkat G. Right ventricular growth in a case of pulmonic stenosis with intact ventricular septum and hypoplastic right ventricle. Circulation. 1976 Feb. 53(2):389-94. [Medline].
Freedom RM. Pulmonary Atresia with Intact Ventricular Septum. Mount Kisco, NY: Futura Publishing, Co; 1989. 1-257.
Rao PS. Comprehensive management of pulmonary atresia with intact ventricular septum. Ann Thorac Surg. 1985 Oct. 40(4):409-13. [Medline].
Kopecky SL, Gersh BJ, McGoon MD, Mair DD, Porter CJ, Ilstrup DM, et al. Long-term outcome of patients undergoing surgical repair of isolated pulmonary valve stenosis. Follow-up at 20-30 years. Circulation. 1988 Nov. 78(5 Pt 1):1150-6. [Medline].
[Guideline] Bonow RO, Carabello BA, Chatterjee K. guideline for the management of patients with valvular heart disease. J Am Coll Cardiol. 2006. 48(3):e1-148.
Ho VB, Yucel EK, Khan A, et al. Suspected congenital heart disease in the adult. American College of Radiology. 2007. [Full Text].
[Guideline] Vahanian A, Baumgartner H, Bax J, 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. 2007 Jan. 28(2):230-68. [Medline].

Media Gallery

Valvar Pulmonary Stenosis. 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 far ahead of 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 systole and the murmur reaches the A2, and the second heart sound is widely split with a 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.
Valvar Pulmonary Stenosis. Posteroanterior chest roentgenogram in a patient with valvar pulmonic stenosis showing a normal-sized heart with normal pulmonary vascular markings. Note prominent main pulmonary artery. Reproduced with permission from Rao PS: Diagnosis and management of acyanotic heart disease: Part I – Obstructive lesions. Indian J Pediatr. 2005; 72: 495-502.
Valvar Pulmonary Stenosis. 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 the 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 the 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.
Valvar Pulmonary Stenosis. Right ventricular (RV) cineangiogram in the lateral view of 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.
Valvar Pulmonary Stenosis. 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.
Valvar Pulmonary Stenosis. Selected frames from the 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 mmHg 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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