Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Infundibular Pulmonary Stenosis Clinical Presentation

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

History

Infundibular pulmonary stenosis (IPS) manifestations depend on the severity of obstruction and presence or absence of associated cardiac anomalies. They may include the following:

  • Most children grow well and are asymptomatic, even when stenosis is moderate or severe.
  • The murmur is discovered on routine auscultation, usually at birth, although cyanosis may lead to discovery of maximum obstruction.
  • Symptoms are rare in infants, with the notable exception of patients with critical stenosis.
  • Subjective complaints tend to increase with age.
  • Dyspnea and fatigue are the most common symptoms.
  • Exertion may provoke syncope or even death.
  • Precordial pain is common, and epigastric pain is often present.
  • Frank right-sided heart failure occasionally occurs in infancy or early childhood.
  • Squatting is extremely rare in children with isolated PS (compared with tetralogy of Fallot)
Next

Physical

Physical examination findings may include the following:

  • Growth and development are usually normal. Frank heart failure is rarely evident.
  • Chest asymmetry occasionally accompanies severe stenosis, but precordial bulge is uncommon.
  • Jugular venous pulse shows larger a waves as the degree of obstruction increases. These presystolic pulsations may be felt during palpation of the liver, even without evidence of cardiac failure.
  • Prominent left parasternal heave occurs if PS is significant.
  • A systolic thrill is present at the second and third left intercostal space near the sternum. Occasionally, the thrill may disappear with onset of failure.

Auscultation

The first heart sound is normal.

The pulmonic valve component (P-2) of the second heart sound is soft and delayed in moderate-to-severe stenosis. Note the following features:

  • The degree of split is proportionate to the severity of the obstruction; the greater the obstruction, the longer the RV takes to empty and the wider the split.
  • P-2 decreases in intensity in proportion to the pressure in the PA. The lower the pressure, the softer the P-2. P-2 may be inaudible with maximal obstruction.
  • In severe stenosis with unchanging cardiac output, the split may be fixed. A loud pansystolic crescendo-decrescendo murmur (ejection type), with its maximal intensity at mid systole or later (indistinguishable from that of isolated pulmonary valve stenosis [PVS]), is heard at the left sternal border and is well-conducted to the precordium, neck, and back.

A third heart sound is audible in the presence of an associated atrial septal defect (ASD) or anomalous pulmonary vein.

A fourth heart sound is heard at the lower left sternal border in severe cases. This fourth heart sound is associated with a large a wave in the RA and usually indicates a severe lesion.

Note the absence of the ejection click that characterizes valvar PS.

A loud, long, systolic crescendo-decrescendo murmur (ejection type), with its maximal intensity at mid systole or later (indistinguishable from that of isolated PVS), is heard at the left sternal border and is well-conducted to the precordium, neck, and back. Note the following features:

  • The murmur, although louder at the second and third left intercostal space, may be heard well at the low left sternal border.
  • The later the peak intensity of the murmur occurs, the greater the obstruction.
  • Although murmur loudness does not necessarily increase with severity, murmurs of less than grade 3/6 usually occur with mild stenosis. With moderate-to-severe stenosis, murmurs are usually systolic and grade 4/6 or louder.
  • The length of the murmur depends on duration of RV systole that, in turn, depends on severity of the stenosis. Thus, mild stenosis is associated with a short murmur, with its peak earlier than mid systole. In moderate stenosis, the murmur ends at or slightly after the aortic component of the second heart sound, which remains audible. With marked-to-severe obstruction, the murmur extends beyond the aortic component, which may be obscured.

Critical stenosis

Infants with critical stenosis present with variable findings, including the following:

  • Heart failure is prominent.
  • A small infant with maximal obstruction may have minimal murmur (sometimes overlooked) and cyanosis.
  • An additional systolic murmur is heard in the lower left parasternal region from the tricuspid regurgitation (TR).
  • Absence of P-2 along with the presence of cardiomegaly and the holosystolic murmur of TR highly suggests a critical PS diagnosis.
Previous
Next

Causes

See Pathophysiology.

Previous
 
 
Contributor Information and Disclosures
Author

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.

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. 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].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.