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Infundibular Pulmonary Stenosis Medication

  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCPCH; Chief Editor: Stuart Berger, MD  more...
Updated: Feb 21, 2014

Medication Summary

Surgery is the mainstay of treatment for significant stenosis. Antibiotics for endocarditis prophylaxis are given to patients with infundibular pulmonary stenosis (IPS) before performing procedures that may cause bacteremia. Endocarditis prophylaxis is recommended throughout life, even after surgical relief. For more information, see Antibiotic Prophylactic Regimens for Endocarditis. Heart failure therapy is indicated only as a temporary measure for patients with heart failure. Ensure adequate hydration before administering diuretics because the cardiac output depends on adequate preload; if necessary, use beta-adrenergic blockers to relax the infundibular muscles. Alprostadil minimizes cyanosis in neonates with ductal-dependent pulmonary circulation.


Beta-adrenergic blocking agents

Class Summary

These agents inhibit beta1- and beta2-adrenergic receptors. They inhibit chronotropic, inotropic and vasodilatory responses to beta-adrenergic stimulation. Their exact mechanism of benefit is uncertain, although it is believed to relieve infundibular spasm.

Propranolol (Inderal)


Inhibits beta1- and beta2-adrenergic receptors.



Class Summary

These agents are used to eliminate retained fluid and lower preload. They promote excretion of water and electrolytes by the kidneys. They are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention has resulted in edema or ascites.

Furosemide (Lasix)


Increases water excretion by interfering with Cl-binding cotransport system, which, in turn, inhibits NA and Cl reabsorption in ascending Henle loop and distal renal tubule. When administered IV, also has a venodilation action; thus lowering the preload even before diuresis sets in. DOC for acute heart failure and for exacerbations of chronic heart failure; used for long-term management of chronic heart failure.

Spironolactone (Aldactone)


Competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.

Amiloride (Midamor)


Potassium-sparing diuretic acting directly on the distal renal tubule. Often combined with a nonsparing-potassium diuretic.



Class Summary

These agents are used to maintain patency of ductus arteriosus in patients with duct-dependent circulation.

Alprostadil IV (Prostin VR Pediatric)


Acts on the smooth muscles of the ductus arteriosus and prevents its closure in response to elevation of arterial oxygen saturation. First-line medication used as palliative therapy to temporarily maintain patency of the ductus arteriosus prior to surgery. Beneficial in infants with congenital defects that restrict pulmonary or systemic blood flow and who depend on a patent ductus arteriosus to get adequate oxygenation and lower body perfusion. Produces vasodilation and increases cardiac output. Each 1-mL ampule contains 500 mcg/mL. Used to maintain patency of ductus arteriosus when cyanotic lesion (critical pulmonary stenosis/atresia) or interrupted aortic arch presents in newborns.

Contributor Information and Disclosures

Poothirikovil Venugopalan, MBBS, MD, FRCPCH Consultant Pediatrician with Cardiology Expertise, Department of Child Health, Brighton and Sussex University Hospitals, NHS Trust; Honorary Senior Clinical Lecturer, Brighton and Sussex Medical School, UK

Poothirikovil Venugopalan, MBBS, MD, FRCPCH is a member of the following medical societies: Royal College of Paediatrics and Child Health, Paediatrician with Cardiology Expertise Special Interest Group, British Congenital Cardiac Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Hugh D Allen, MD Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, Western Society for Pediatric Research, American College of Cardiology, American Heart Association, American Pediatric Society

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College 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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

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 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, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, Texas Pediatric Society, Cardiac Electrophysiology Society

Disclosure: Nothing to disclose.

  1. Marton T, Hajdu J, Papp C, et al. Pulmonary stenosis and reactive right ventricular hypertrophy in the recipient fetus as a consequence of twin-to-twin transfusion. Prenat Diagn. 2001 Jun. 21(6):452-6. [Medline].

  2. Matsuo S, Sato Y, Higashida R, Shiraishi S, Asai T, Nakae I, et al. A giant main pulmonary artery aneurysm associated with infundibular pulmonary stenosis. Cardiovasc Revasc Med. 2008 Jul-Sep. 9(3):188-9. [Medline].

  3. Moon JI, Jeong YJ, Lee G, Choi JH, Lee JW. Isolated left ventricular apical hypoplasia with infundibular pulmonary and aortic stenosis: a rare combination. Korean J Radiol. 2013 Nov-Dec. 14(6):874-7. [Medline].

  4. Joy MV, Subramonium R, Venkitachalam CG, Balakrishnan KG. Two dimensional and Doppler echocardiographic evaluation of double chambered right ventricle. Indian Heart J. 1992 May-Jun. 44(3):159-63. [Medline].

  5. Nakajima T, Arakaki Y, Kamiya T, et al. Doppler echocardiographic estimates of pressure gradients in various types of stenoses: usefulness and limitations [in Japanese]. J Cardiol. 1989 Sep. 19(3):851-8. [Medline].

  6. Martín M, Luyando LH, Morís C. Pulmonary infundibular stenosis and ventricular septum defect: usefulness of cardiac CT. Acta Cardiol. 2009 Apr. 64(2):269-70. [Medline].

  7. Sahin T, Bildirici U, Kandemir C, Celikyurt U, Ural D, Komsuoglu B. Infective endocarditis in the setting of infundibular-valvular pulmonary stenosis with incomplete cor triatriatum dextrum and patent foramen ovale. Int J Cardiol. 2008 Jul 21. 127(3):e129-31. [Medline].

  8. Antal AD, Cikirikcioglu M, Myers PO, Didier D, Kalangos A. Respiratory distress after surgery of RVOT pathologies: a word of caution on pseudoaneurysm development. Thorac Cardiovasc Surg. 2010 Sep. 58(6):356-8. [Medline].

  9. Alekian BG, Petrosian IuS, Podzolkov VP, et al. Catheter therapy of congenital cardiovascular defects [in Russian]. Vestn Rentgenol Radiol. 1995 Mar-Apr. (2):16-26. [Medline].

  10. Alipour MS, Shadkhoo G, Tarbiat C. Congenital pulmonary valvular and infundibular stenosis complicating Ebstein's anomaly of the tricuspid valve. A case report. Jpn Heart J. 1980 Nov. 21(6):883-90. [Medline].

  11. Bhandari S, Dev V, Shrivastava S, Bhatia ML. Echocardiographic demonstration of pulmonary valve vegetation in a child with infundibular pulmonic stenosis and closed VSD. Indian Heart J. 1986 Sep-Oct. 38(5):425-8. [Medline].

  12. Brown JW, Ruzmetov M, Rodefeld MD, et al. Valved bovine jugular vein conduits for right ventricular outflow tract reconstruction in children: an attractive alternative to pulmonary homograft. Ann Thorac Surg. 2006 Sep. 82(3):909-16. [Medline].

  13. Carlson KM, Neish SR, Justino H, et al. Use of cutting balloon for palliative treatment in tetralogy of Fallot. Catheter Cardiovasc Interv. 2005 Apr. 64(4):507-12. [Medline].

  14. Caspi J, Coles JG, Benson LN, et al. Management of neonatal critical pulmonic stenosis in the balloon valvotomy era. Ann Thorac Surg. 1990 Feb. 49(2):273-8. [Medline].

  15. Conte S, Jashari R, Eyskens B, et al. Homograft valve insertion for pulmonary regurgitation late after valveless repair of right ventricular outflow tract obstruction. Eur J Cardiothorac Surg. 1999 Feb. 15(2):143-9. [Medline].

  16. Daskalopoulos DA, Pieroni DR, Gingell RL, et al. Closed transventricular pulmonary valvotomy in infants. J Thorac Cardiovasc Surg. 1982 Aug. 84(2):187-91. [Medline].

  17. Delhaas T, Prinzen FW. Sequelae of DDD pacing in patients with pulmonary infundibular stenosis [letter]. Am J Cardiol. 1999 Nov 1. 84(9):1142. [Medline].

  18. Fawzy ME, Awad M, Galal O, et al. Long-term results of pulmonary balloon valvulotomy in adult patients. J Heart Valve Dis. 2001 Nov. 10(6):812-8. [Medline].

  19. Freed MD, Heymann MA, Lewis AB, et al. Prostaglandin E1 infants with ductus arteriosus-dependent congenital heart disease. Circulation. 1981 Nov. 64(5):899-905. [Medline].

  20. Galal O, Kalloghlian A, Pittappilly BM, Dzimiri N. Phentolamine improves clinical outcome after balloon valvoplasty in neonates with severe pulmonary stenosis. Cardiol Young. 1999 Mar. 9(2):127-8. [Medline].

  21. Gibbs JL, Uzun O, Blackburn ME, et al. Right ventricular outflow stent implantation: an alternative to palliative surgical relief of infundibular pulmonary stenosis. Heart. 1997 Feb. 77(2):176-9. [Medline]. [Full Text].

  22. Glen S, Burns J, Bloomfield P. Prevalence and development of additional cardiac abnormalities in 1448 patients with congenital ventricular septal defects. Heart. 2004 Nov. 90(11):1321-5. [Medline]. [Full Text].

  23. Goebel N, Gander MP. [Echinococcus of the heart. Infundibular pulmonary stenosis due to a large solitary cyst of the ventricular septum (author's transl)]. Rofo. 1977 Jan. 126(1):11-4. [Medline].

  24. Gupta D, Saxena A, Kothari SS, Juneja R. Factors influencing late course of residual valvular and infundibular gradients following pulmonary valve balloon dilatation. Int J Cardiol. 2001 Jul. 79(2-3):143-9. [Medline].

  25. Harinck E, Becker AE, Groot AC, et al. The left ventricle in congenital isolated pulmonary valve stenosis. A morphological study. Br Heart J. 1977 Apr. 39(4):429-35. [Medline]. [Full Text].

  26. Houston AB, Simpson IA, Sheldon CD, et al. Doppler ultrasound in the estimation of the severity of pulmonary infundibular stenosis in infants and children. Br Heart J. 1986 Apr. 55(4):381-4. [Medline].

  27. Ilbawi M, Cua C, DeLeon S, et al. Repair of complete atrioventricular septal defect with tetralogy of Fallot. Ann Thorac Surg. 1990 Sep. 50(3):407-12. [Medline].

  28. Imafuku T, Ogihara T, Kudo H, et al. Kartagener's syndrome associated with infundibular pulmonic stenosis, chronic renal failure and azoospermia: a report of a case. Jpn J Med. 1986 May. 25(2):195-8. [Medline].

  29. Isomura T, Hisatomi K, Hirano A, et al. Ruptured aneurysms of the sinus of Valsalva. J Cardiovasc Surg (Torino). 1994 Apr. 35(2):135-8. [Medline].

  30. Jackson F. The heart at high altitude. Br Heart J. 1968 May. 30(3):291-4. [Medline].

  31. Keith A. Malformation of the heart. Lancet. 1909. 2:359.

  32. Khikmatov AA. Changes in intracardiac hemodynamics during of the natural course of isolated stenoses of pulmonary artery and the right ventricular outflow tract [in Russian]. Ter Arkh. 2001. 73(9):73-6. [Medline].

  33. Kirklin JW, Blackstone EH, Jonas RA, et al. Morphologic and surgical determinants of outcome events after repair of tetralogy of Fallot and pulmonary stenosis. A two-institution study. J Thorac Cardiovasc Surg. 1992 Apr. 103(4):706-23. [Medline].

  34. 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].

  35. Kraiem S, Hmem M, Longo S, et al. Double chamber right ventricle. About 3 cases [in French]. Tunis Med. 2006 May. 84(5):316-20. [Medline].

  36. Kronik G, Fitscha P, Slany J, Mösslacher H. [Echocardiographic diagnosis of subpulmonic obstruction in hypertrophic cardiomyopathy (a case report) (author's transl)]. Z Kardiol. 1979 Jun. 68(6):415-8. [Medline].

  37. Latsis AT, Iagmanis MIa, Kreile IE, Trushelis E. Correction of a partially patent atrioventricular canal with a common atrium, infundibular and valvular stenosis of the pulmonary artery and a single (left) superior vena cava [in Russian]. Grud Serdechnososudistaia Khir. 1991 Feb. (2):53-5. [Medline].

  38. Levin DL, Heymann MA, Rudolph AM. Morphological development of the pulmonary vascular bed in experimental pulmonic stenosis. Circulation. 1979 Jan. 59(1):179-82. [Medline].

  39. Lucas RV, Varco RL, Lillehei CW, et al. Anomalous muscle bundle of the right ventricle. Hemodynamic consequences and surgical considerations. Circulation. 1962 Mar. 25:443-55. [Medline].

  40. Mantri RR, Bajaj R, Shrivastava S. Multiple anomalies of caval veins in a patient with pulmonic stenosis. Int J Cardiol. 1994 Sep. 46(2):172-4. [Medline].

  41. McQuinn TC, Miga DE, Mjaatvedt CH, et al. Cardiopulmonary malformations in the inv/inv mouse. Anat Rec. 2001 May 1. 263(1):62-71. [Medline].

  42. Mehan VK, Meier B. Interventional cardiology: state of the art. Presse Med. 1994 Feb 19. 23(7):339-44. [Medline].

  43. Mori F, Tsuboi H, Ohmi M, et al. Noonans syndrome associated with atrial septal defect, ventricular septal defect and infundibular pulmonary stenosis--a case report [in Japanese]. Nippon Kyobu Geka Gakkai Zasshi. 1983 Jun. 31(6):936-9. [Medline].

  44. Moura C, Carrico A, Baptista MJ, et al. Balloon pulmonary valvotomy performed in the first year of life. Rev Port Cardiol. 2004 Jan. 23(1):55-63. [Medline].

  45. Patel R, Astley R. Right ventricular obstruction due to anomalous muscle bands. Br Heart J. 1973 Sep. 35(9):890-3. [Medline].

  46. Pogrebniak VV, Tereshchenko VP, Zin'kovskii MF, Zurnadzhi IuN, Chumak OS. [Morphogenetic patterns of isolated infundibular stenosis of the pulmonary artery]. Vrach Delo. 1989 Sep. 44-6. [Medline].

  47. Pongiglione G, Freedom RM, Cook D, Rowe RD. Mechanism of acquired right ventricular outflow tract obstruction in patients with ventricular septal defect: an angiocardiographic study. Am J Cardiol. 1982 Oct. 50(4):776-80. [Medline].

  48. Qureshi SA, Parsons JM, Tynan M. Percutaneous transcatheter myectomy of subvalvar pulmonary stenosis in tetralogy of Fallot: a new palliative technique with an atherectomy catheter. Br Heart J. 1990 Aug. 64(2):163-5. [Medline].

  49. Rao PS. Transcatheter management of cyanotic congenital heart defects: a review. Clin Cardiol. 1992 Jul. 15(7):483-96. [Medline].

  50. Rao PS, Wilson AD, Thapar MK, Brais M. Balloon pulmonary valvuloplasty in the management of cyanotic congenital heart defects. Cathet Cardiovasc Diagn. 1992 Jan. 25(1):16-24. [Medline].

  51. Raut NB, Norton JB, Patil AA. Bourneville's tuberous sclerosis associated with double outlet right ventricle and infundibular pulmonary stenosis. J Assoc Physicians India. 1992 Jul. 40(7):469-70. [Medline].

  52. Rowland TW, Rosenthal A, Castaneda AR. Double-chamber right ventricle: experience with 17 cases. Am Heart J. 1975 Apr. 89(4):455-62. [Medline].

  53. Rummeny E, Hausen W, Lorbacher P, Willems D. Acquired infundibular pulmonary stenosis. Possible late complication following radiotherapy of Hodgkin disease [in German]. Z Kardiol. 1984 Oct. 73(10):641-5. [Medline].

  54. Schrope DP. Primary pulmonic infundibular stenosis in 12 cats: natural history and the effects of balloon valvuloplasty. J Vet Cardiol. 2008 Jun. 10(1):33-43. [Medline].

  55. Shenoy AR, Padmakumar P, Subashchandra V. Right ventricular outflow tract stenting in tetrology of fallot with restrictive ventricular septal defect. J Invasive Cardiol. 2006 Jan. 18(1):E59-60. [Medline].

  56. Shimada Y, Yaku H, Kawata M, et al. Surgical repair of primary infundibular stenosis in a 72-year-old man. ANZ J Surg. 2001 Aug. 71(8):498-9. [Medline].

  57. Shishkov BV, Nikoliuk AP, Garibian VA, et al. Surgical tactics in critical stenosis and atresia of the pulmonary artery with intact interventricular septum in infants under 1 year of age [in Russian]. Grud Serdechnososudistaia Khir. 1991 Sep. 11-4. [Medline].

  58. 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].

  59. Simpson WF Jr, Sade RM, Crawford FA, et al. Double-chambered right ventricle. Ann Thorac Surg. 1987 Jul. 44(1):7-10. [Medline].

  60. Takahashi T, Sakakibara T, Nomura F, et al. A case report of isolated infundibular pulmonary stenosis with pouch of the infundibular chamber [in Japanese]. Nippon Kyobu Geka Gakkai Zasshi. 1989 Jun. 37(6):1197-201. [Medline].

  61. Tanner K, Sabrine N, Wren C. Cardiovascular malformations among preterm infants. Pediatrics. 2005 Dec. 116(6):e833-8. [Medline].

  62. Tereshchenko VP, Pogrebniak VV, Ishchenko VE, et al. The structure of isolated infundibular stenosis of the pulmonary artery [in Russian]. Vrach Delo. 1990 Jun. (6):31-2. [Medline].

  63. Wu MH, Wu JM, Chang CI, et al. Implication of aneurysmal transformation in isolated perimembranous ventricular septal defect. Am J Cardiol. 1993 Sep 1. 72(7):596-601. [Medline].

  64. Yamagishi M, Nakamura Y, Kanazawa T, Kawada N. Extracardiac direct total cavopulmonary connection. Ann Thorac Surg. 1997 Dec. 64(6):1817-9; discussion 1819-20. [Medline].

  65. Yamamoto T, Habuchi Y, Morikawa J. Single coronary artery with infundibular pulmonary stenosis. Heart. 1997 Aug. 78(2):205-6. [Medline].

  66. Zacherl S, Feyertag C, Salzer-Muhar U, Wimmer M. Bacterial endocarditis in childhood [in German]. Klin Padiatr. 1996 Mar-Apr. 208(2):47-52. [Medline].

Chest radiograph of a 2-year-old boy with severe pulmonary stenosis (infundibular). Note the mild cardiomegaly, reduced pulmonary vascularity, and absence of poststenotic dilatation of pulmonary artery.
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