Infundibular Pulmonary Stenosis 

  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCP(Glasg), FRCPCH; Chief Editor: Stuart Berger, MD   more...
 
Updated: Jun 23, 2010
 

Background

First described by Elliotson, infundibular pulmonary stenosis (IPS) refers to obstruction of outflow from the right ventricle (RV) within the body of the RV, as opposed to obstruction at the pulmonary valve, pulmonary artery (PA), or its branches. IPS often occurs in association with other intracardiac anomalies; isolated IPS is rare. In this article, IPS refers to isolated infundibular pulmonary stenosis with an intact ventricular septum.

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Pathophysiology

IPS has 2 forms. In the more common type, stenosis of the proximal portion of the infundibulum is due to a fibrous or muscle band at the junction of the main cavity of the RV and the infundibulum. The band divides the cavity into 2 chambers. The second type is associated with a thickened muscular infundibulum that forms a narrow outlet to the RV. The infundibulum appears shrunken. In this second type, the narrowed area may be short or long and may be located immediately below the pulmonary valve or lower into the outflow tract.

IPS is often associated with a ventricular septal defect (VSD). Common opinion holds that the VSD closes, leaving behind an isolated IPS. Whether some children have excessive muscle without ever having had a VSD is not known.

The hemodynamic consequence of the obstruction is elevated pressure within the RV cavity. The degree of elevation depends on the severity of obstruction. When severe, the resulting RV systolic pressure may exceed that of the left ventricle (LV). This high pressure is limited to the portion proximal to the infundibulum. Lower or even normal pressures are present beyond the obstruction site. Reactive RV hypertrophy follows.

Subsequent elevation of end-diastolic pressure and decreased compliance of the RV, consequent to the hypertrophy, lead to elevated right atrial (RA) pressure and dilatation of that chamber. Greater RA pressures are required to fill the ventricle, and relative right and left atrial pressures may be reversed, favoring persistent patency of the foramen ovale and right-to-left shunting. This gives rise to central cyanosis. Such reversal and cyanosis can occur when the RV is hypoplastic, even with less severe PS.

In neonates with severe PS, the pulmonary blood flow depends on the patency of the ductus arteriosus. The RV becomes hypertrophic and maintains cardiac output to a very advanced stage. With exercise, the requirement for cardiac output increases, and RV pressure proportionately rises until it exceeds the capacity of the RV muscle.

Deformity and malfunctioning of the LV occur in proportion to RV hypertension and can be demonstrated using sophisticated techniques. These LV problems have little practical consequence because they improve with relief of the RV hypertension.

Reduced LV end-diastolic compliance has been chiefly ascribed to displacement of the interventricular septum (IVS) into the LV cavity. The mechanisms responsible for systolic dysfunction are less clear.

Patients with isolated valvular PS may have a reactive infundibular hypertrophy that could elicit a reactive infundibular obstruction. This obstruction might persist for a variable period following relief of the valvar obstruction.

IPS can cause anatomic and, possibly, functional changes in the developing pulmonary vascular bed. Experimental construction of the pulmonary trunk in fetal lambs is accompanied by relatively thin-walled PA resistance vessels. Some researchers postulate this decreased medial muscular layer is caused by a higher oxygen tension of the blood perfusing the fetal pulmonary circulation.

IPS has been noted to develop in a fetus following twin-to-twin transfusion, and authors have, in that case, postulated that the elevated blood volume/pressure may be a factor in the pathogenesis.[1] Inversin, the product of the inv locus, may have a specific role in cardiac morphogenesis (especially in the development of IPS) in addition to its contribution to situs determination.

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Epidemiology

Frequency

United States

The relative frequency of IPS among all obstructive lesions of the RV outflow tract is 2-10%.

International

No reliable figures have been reported.

Mortality/Morbidity

Severe IPS that leads to atresia can result in neonatal death when the ductus arteriosus constricts. Heart failure secondary to IPS is rare in childhood beyond early infancy. Exercise may provoke syncope and even sudden death in severe stenosis. The stenosis may remain asymptomatic but may progress to require intervention in adulthood.

Sex

IPS has no gender predilection.

Age

IPS is present from birth, although most patients are asymptomatic. The severity of stenosis can progress with age.

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Contributor Information and Disclosures
Author

Poothirikovil Venugopalan, MBBS, MD, FRCP(Glasg), FRCPCH,  Consulting Staff, Department of Child Health, University Hospital of North Tees and Hartlepool, UK

Poothirikovil Venugopalan, MBBS, MD, FRCP(Glasg), FRCPCH, is a member of the following medical societies: British Cardiac Society, Paediatrician with Cardiology Expertise Special Interest Group, Royal College of Paediatrics and Child Health, and Royal College of Physicians and Surgeons of Glasgow

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.

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 College of Cardiology, American Heart Association, American Pediatric Society, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, and Western Society for Pediatric Research

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. 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. Jun 2001;21(6):452-6. [Medline].

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

  3. 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. Sep 1989;19(3):851-8. [Medline].

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

  5. 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. Jul 21 2008;127(3):e129-31. [Medline].

  6. 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. Jul-Sep 2008;9(3):188-9. [Medline].

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

  8. 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. Nov 1980;21(6):883-90. [Medline].

  9. 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. Sep-Oct 1986;38(5):425-8. [Medline].

  10. 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. Sep 2006;82(3):909-16. [Medline].

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

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

  13. 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. Feb 1999;15(2):143-9. [Medline].

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

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

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

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

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

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

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

  21. 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. Jan 1977;126(1):11-4. [Medline].

  22. 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. Jul 2001;79(2-3):143-9. [Medline].

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

  24. 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. Apr 1986;55(4):381-4. [Medline].

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

  26. 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. May 1986;25(2):195-8. [Medline].

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

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

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

  30. 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].

  31. 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. Apr 1992;103(4):706-23. [Medline].

  32. 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].

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

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

  35. 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. Feb 1991;(2):53-5. [Medline].

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

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

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

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

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

  41. 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. Jun 1983;31(6):936-9. [Medline].

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

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

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

  45. 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. Oct 1982;50(4):776-80. [Medline].

  46. 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. Aug 1990;64(2):163-5. [Medline].

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

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

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

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

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

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

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

  54. 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. Sep 1991;11-4. [Medline].

  55. 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].

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

  57. 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. Jun 1989;37(6):1197-201. [Medline].

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

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

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

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

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

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

 
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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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