Skip to main content
SpringerLink
Log in

Pulmonary Vein Stenosis

  • Living reference work entry
  • First Online:
Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care

  • 53 Accesses

Abstract

Pulmonary vein stenosis (PVS) is a relatively rare congenital heart condition that is characterized by narrowing or obstruction of one or more of the pulmonary veins. Involvement of multiple veins and early age of diagnosis is associated with a guarded prognosis, as children develop secondary pulmonary hypertension and right heart failure resulting in death. Surgical, catheter-based interventions and experimental therapies can be effective in some children, however survival following intervention is 60% in children with multivessel involvement. There are two broad categories of pulmonary vein stenosis: (1) Primary (congenital) PVS involves stenosis in normally connected pulmonary veins and can be associated with other congenital heart diseases. (2) Post-repair pulmonary vein stenosis (PR-PVS) typically occurs in 10–15% of children after repair of total anomalous pulmonary venous return (TAPVR). This chapter will discuss current advances in clinical care for children with PVS and outline medical, surgical, and interventional management along with contemporary outcomes.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

Similar content being viewed by others

References

  1. Quinonez LG, Gauvreau K, Borisuk M, Ireland C, Marshall AM, Mayer JE, Jenkins KJ, Fynn-Thompson FE, Baird CW (2015) Outcomes of surgery for young children with multivessel pulmonary vein stenosis. J Thorac Cardiovasc Surg 150(4):911–917

    Article  PubMed  Google Scholar 

  2. Hickey EJ, Caldarone CA (2011) Surgical management of post-repair pulmonary vein stenosis. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 14(1):101–108

    Article  PubMed  Google Scholar 

  3. Larson WJ (1993) Human embryology. Churchill Livingstone, New York

    Google Scholar 

  4. Sadler TW (1995) Langman’s medical embryology, 7th edn. Williams & Wilkins, Baltimore

    Google Scholar 

  5. Geva T, Van Praagh S (2007) Anomalies of the pulmonary veins. In: Allen HD, Driscoll DJ et al (eds) Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adults, 7th edn. Lippincott Williams and Wilkins, Philadelphia, pp 761–792

    Google Scholar 

  6. Sadr IM, Tan PE, Kieran MW, Jenkins KJ (2000) Mechanism of pulmonary vein stenosis in infants with normally connected veins. Am J Cardiol 86:577–579

    Article  CAS  PubMed  Google Scholar 

  7. Riedlinger WF, Juraszek AL, Jenkins KJ, Nugent AW, Balasubramanian S, Calicchio ML, Kieran MW, Collins T (2006) Pulmonary vein stenosis: expression of receptor tyrosine kinases by lesional cells. Cardiovasc Pathol 15:91–99

    Article  CAS  PubMed  Google Scholar 

  8. Seale AN, Webber SA, Uemura H, Partridge J, Roughton M, Ho SY, McCarthy KP, Jones S, Shaughnessy L, Sunnegardh J, Hanseus K, Rigby ML, Keeton BR, Daubeney PE, British Congenital Cardiac Association (2009) Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study. Heart 95(23):1944–1949

    Article  CAS  PubMed  Google Scholar 

  9. Drossner DM, Kim DW, Maher KO, Mahle WT (2008) Pulmonary vein stenosis: prematurity and associated conditions. Pediatrics 122:e656–e661

    Article  PubMed  Google Scholar 

  10. Shuhaiber J, Rehman M, Jenkins K, Fynn-Thompson F, Bacha E (2011) The role of surgical therapy for pulmonary vein atresia in childhood. Pediatr Cardiol 32(5):639–645

    Article  CAS  PubMed  Google Scholar 

  11. Balasubramanian S, Rehman M, Gauvreau K, Jenkins KJ (2012) Bilateral disease and early age at presentation are associated with shorter survival in patients with congenital heart disease and intraluminal pulmonary vein stenosis. Congenit Heart Dis 7:378–386

    Article  PubMed  Google Scholar 

  12. Mahgoub L, Kaddoura T, Kameny AR, Lopez Ortego P, Vanderlaan RD, Kakadekar A, Dicke F, Rebeyka I, Calderone CA, Redington A, Del Cerro MJ, Fineman J, Adatia I (2017) Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort. Pediatr Pulmonol 52(8):1063–1070

    Article  PubMed  Google Scholar 

  13. Bando K, Turrentine MW, Ensing GJ et al (1996) Surgical management of total anomalous pulmonary venous connection. Thirty-year trends. Circulation 94:II12–II16

    CAS  PubMed  Google Scholar 

  14. Seale AN, Uemura H, Webber SA, Partridge J, Roughton M, Ho SY, McCarthy KP, Jones S, Shaughnessy L, Sunnegardh J, Hanseus K, Berggren H, Johansson S, Rigby ML, Keeton BR, Daubeney PE, British Congenital Cardiac Association (2010) Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation 122(25):2718–2726

    Article  PubMed  Google Scholar 

  15. Shi G, Zhu Z, Chen J, Ou Y, Hong H, Nie Z, Zhang H, Liu X, Zheng J, Sun Q, Liu J, Chen H, Zhuang J (2017) Total anomalous pulmonary venous connection: the current management strategies in a pediatric cohort of 768 patients. Circulation 135(1):48–58

    Article  PubMed  Google Scholar 

  16. Karamlou T, Gurofsky R, Al Sukhni E, Coles JG, Williams WG, Caldarone CA, Van Arsdell GS, McCrindle BW (2007) Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection. Circulation 115(12):1591–1598

    Article  PubMed  Google Scholar 

  17. Seale AN, Uemura H, Webber SA, Partridge J, Roughton M, Ho SY, McCarthy KP, Jones S, Shaughnessy L, Sunnegardh J, Hanseus K, Berggren H, Johansson S, Rigby ML, Keeton BR, Daubeney PE, British Congenital Cardiac Association (2013) Total anomalous pulmonary venous connection: outcome of postoperative pulmonary venous obstruction. J Thorac Cardiovasc Surg 145(5):1255–1262

    Article  PubMed  Google Scholar 

  18. Kalfa D, Belli E, Bacha E, Lambert V, di Carlo D, Kostolny M, Nosal M, Horer J, Salminen J, Rubay J, Yemets I, Hazekamp M, Maruszewski B, Sarris G, Berggren H, Ebels T, Baser O, Lacour-Gayet F, European Congenital Heart Surgeons Association (2018) Outcomes and prognostic factors for postsurgical pulmonary vein stenosis in the current era. J Thorac Cardiovasc Surg 156(1):278–286

    Article  PubMed  Google Scholar 

  19. Honjo O, Atlin CR, Hamilton BC, Al-Radi O, Viola N, Coles JG, Van Arsdell GS, Caldarone CA (2010) Primary sutureless repair for infants with mixed total anomalous pulmonary venous drainage. Ann Thorac Surg 90(3):862–868

    Article  PubMed  Google Scholar 

  20. Manzar S (2007) Congenital pulmonary vein stenosis. J Coll Physicians Surg Pak 17:374–375

    PubMed  Google Scholar 

  21. Omasa M et al (2004) A case of congenital pulmonary vein stenosis in an adult. Respiration 71:92–94

    Article  PubMed  Google Scholar 

  22. Minich LL et al (2001) Complete follow-up echocardiograms are needed to detect stenosis of normally connecting pulmonary veins. Echocardiography 18:589–592

    Article  CAS  PubMed  Google Scholar 

  23. Smallhorn JF et al (1987) Pulsed Doppler echocardiographic assessment of extraparenchymal pulmonary vein flow. J Am Coll Cardiol 9:573–579

    Article  CAS  PubMed  Google Scholar 

  24. Agata Y et al (1994) Changes in pulmonary venous flow pattern during early neonatal life. Br Heart J 71:182–186

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Roman KS (2005) How is pulmonary arterial blood flow affected by pulmonary venous obstruction in children? A phase-contrast magnetic resonance study. Pediatr Radiol 35:580–586

    Article  PubMed  Google Scholar 

  26. Grosse-Wortmann L, Al-Otay A, Goo HW, Macgowan CK, Coles JG, Benson LN, Redington AN, Yoo SJ (2007) Anatomical and functional evaluation of pulmonary veins in children by magnetic resonance imaging. J Am Coll Cardiol 49(9):993–1002

    Article  PubMed  Google Scholar 

  27. Peng LF, Lock JE, Nugent AW, Jenkins KJ, McElhinney DB (2010) Comparison of conventional and cutting balloon angioplasty for congenital and postoperative pulmonary vein stenosis in infants and young children. Catheter Cardiovasc Interv 75(7):1084–1090

    PubMed  Google Scholar 

  28. Balasubramanian S, Marshall AC, Gauvreau K, Peng LF, Nugent AW, Lock JE, McElhinney DB (2012) Outcomes after stent implantation for the treatment of congenital and postoperative pulmonary vein stenosis in children. Circ Cardiovasc Interv 5(1):109–117

    Article  PubMed  Google Scholar 

  29. Cory MJ, Ooi YK, Kelleman MS, Vincent RN, Kim DW, Petit CJ (2017) Reintervention is associated with improved survival in pediatric patients with pulmonary vein stenosis. JACC Cardiovasc Interv 10(17):1788–1798

    Article  PubMed  Google Scholar 

  30. Drubach LA, Jenkins KJ, Stamoulis C, Palmer EL 3rd, Lee EY (2015) Evaluation of primary pulmonary vein stenosis in children: comparison of radionuclide perfusion lung scan and angiography. AJR Am J Roentgenol 205(4):873–877

    Article  PubMed  Google Scholar 

  31. Kawashima Y, Ueda T, Naito Y, Morikawa E, Manabe H (1971) Stenosis of pulmonary veins: report of a patient corrected surgically. Ann Thorac Surg 12:196–202

    Article  CAS  PubMed  Google Scholar 

  32. Lacour-Gayet F, Zoghbi J, Serraf AE et al (1999) Surgical management of progressive pulmonary venous obstruction after repair of total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 117:679–687

    Article  CAS  PubMed  Google Scholar 

  33. Lacour-Gayet F (2006) Surgery for pulmonary venous obstruction after repair of total anomalous pulmonary venous return. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 9:45–50

    Article  Google Scholar 

  34. Song MK, Bae EJ, Jeong SI, Kang IS, Kim NK, Choi JY, Kim SJ, Kim YH, Kim WH (2012) Clinical characteristics and prognostic factors of primary pulmonary vein stenosis or atresia in children. Ann Thorac Surg 25:10016–10023

    Google Scholar 

  35. Lacour-Gayet F, Rey C, Planche C (1996) Pulmonary vein stenosis. Description of a sutureless surgical procedure using the pericardium in situ. Arch Mal Coeur Vaiss 89:633–636

    CAS  PubMed  Google Scholar 

  36. Najm HK, Caldarone CA, Smallhorn J, Coles JG (1998) A sutureless technique for the relief of pulmonary vein stenosis with the use of in situ pericardium. J Thorac Cardiovasc Surg 115(2):468–470

    Article  CAS  PubMed  Google Scholar 

  37. Tissot C, Corbelli R, Aggoun Y, Beghetti M, da Cruz E (1998) Bronchoscopic diagnosis of asymptomatic unilateral pulmonary vein atresia in an infant. Pediatr Cardiol 29:976–979

    Article  Google Scholar 

  38. Bharat A, Epstein DJ, Grady M, Faro A, Michelson P, Sweet SC, Huddleston CB (2013) Lung transplant is a viable treatment option for patients with congenital and acquired pulmonary vein stenosis. J Heart Lung Transplant 32(6):621–625

    Article  PubMed  Google Scholar 

  39. Feins EN, Callahan R, Baird CW (2021) Pulmonary vein stenosis-evolving surgical management of a challenging disease. Children (Basel) 8(8):631

    PubMed  Google Scholar 

  40. Byrne RA, Stone GW, Ormiston J, Kastrati A (2017) Coronary balloon angioplasty, stents, and scaffolds. Lancet 390(10096):781–792

    Article  PubMed  Google Scholar 

  41. Muller MJ, Krause U, Paul T, Schneider HE (2011) Serum levels after everolimus-stent implantation and paclitaxel-balloon angioplasty in an infant with recurrent pulmonary vein obstruction after repaired total anomalous pulmonary venous connection. Pediatr Cardiol 32(7):1036–1039

    Article  PubMed  PubMed Central  Google Scholar 

  42. Rehman M, Jenkins KJ, Juraszek AL, Connor JA, Gauvreau K, Muneeb M, Sena LM, Colan SD, Saia T, Kieran MW (2011) A prospective phase II trial of vinblastine and methotrexate in multivessel intraluminal pulmonary vein stenosis in infants and children. Congenit Heart Dis 6(6):608–623

    Article  PubMed  Google Scholar 

  43. Callahan R, Kieran MW, Baird CW, Colan SD, Gauvreau K, Ireland CM, Marshall AC, Sena LM, Vargas SO, Jenkins KJ (2018) Adjunct targeted biologic inhibition agents to treat aggressive multivessel intraluminal pediatric pulmonary vein stenosis. J Pediatr 198:29–35.e5

    Article  PubMed  Google Scholar 

  44. Zhu J, Ide H, Fu YY, Teichert AM, Kato H, Weisel RD, Maynes JT, Coles JG, Caldarone CA (2014) Losartan ameliorates “upstream” pulmonary vein vasculopathy in a piglet model of pulmonary vein stenosis. J Thorac Cardiovasc Surg 148(6):2550–2557

    Article  CAS  PubMed  Google Scholar 

  45. Patel JD, Briones M, Mandhani M, Jones S, Suthar D, Gray R, Pettus J, McCracken C, Thomas A, Petit CJ (2021) Systemic sirolimus therapy for infants and children with pulmonary vein stenosis. J Am Coll Cardiol 77(22):2807–2818

    Article  CAS  PubMed  Google Scholar 

  46. Yun TJ, Coles JG, Konstantinov IE, Al-Radi OO, Wald RM, Guerra V et al (2005) Conventional and sutureless techniques for management of the pulmonary veins: evolution of indications from postrepair pulmonary vein stenosis to primary pulmonary vein anomalies. J Thorac Cardiovasc Surg 129:167–174

    Article  PubMed  Google Scholar 

  47. Devaney EJ, Chang AC, Ohye RG, Bove EL (2006) Management of congenital and acquired pulmonary vein stenosis. Ann Thorac Surg 81:992–996

    Article  PubMed  Google Scholar 

  48. Viola N, Alghamdi AA, Perrin DG, Wilson GJ, Coles JG, Caldarone CA (2011) Primary pulmonary vein stenosis: the impact of sutureless repair on survival. J Thorac Cardiovasc Surg 142:344–350

    Article  PubMed  Google Scholar 

  49. Backes CH, Nealon E, Armstrong AK, Cua CL, Mitchell C, Krishnan U, Vanderlaan RD, Song MK, Viola N, Smith CV, McConnell PI, Rivera BK, Bridge J (2018) Pulmonary vein stenosis in infants: a systematic review, meta-analysis, and meta-regression. J Pediatr 198(36–45):e33

    Google Scholar 

  50. Kalfa D, Belli E, Bacha E, Lambert V, di Carlo D, Kostolny M, Salminen J, Nosal M, Poncelet A, Horer J, Berggren H, Yemets I, Hazekamp M, Maruszewski B, Sarris G, Pozzi M, Ebels T, Lacour-Gayet F, European Congenital Heart Surgeons Association (2017) Primary pulmonary vein stenosis: outcomes, risk factors, and severity score in a multicentric study. Ann Thorac Surg 104(1):182–189

    Article  PubMed  Google Scholar 

  51. Vanderlaan RD, Rome J, Hirsch R, Ivy D, Caldarone CA (2021) Pulmonary vein stenosis: treatment and challenges. J Thorac Cardiovasc Surg 161(6):2169–2176

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Cardiac Surgery, University of Toronto, Toronto, ON, Canada

    Rachel D. Vanderlaan

  2. Congenital Cardiac Surgery, Texas Children’s Hospital, Houston, TX, USA

    Christopher A. Caldarone

  3. Children’s Hospital at Montefiore, Albert Einstein College of Medicine, New York, NY, USA

    François Lacour-Gayet & Leo Lopez

  4. Cardiovascular and Thoracic Surgery, Montefiore Medical Center, Bronx, NY, USA

    François Lacour-Gayet & Leo Lopez

  5. The Heart Institute, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Denver, School of Medicine, Aurora, CO, USA

    Jesse Davidson & Eduardo M. da Cruz

Authors
  1. Rachel D. Vanderlaan
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Christopher A. Caldarone
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. François Lacour-Gayet
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Leo Lopez
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Jesse Davidson
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Eduardo M. da Cruz
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Christopher A. Caldarone .

Editor information

Editors and Affiliations

  1. Children’s Hospital Colorado University of Colorado Denver, School of, Aurora, CO, USA

    Eduardo M. da Cruz

  2. Children's Hospital Colorado University of Colorado School of Medicin, Aurora, CO, USA

    Dunbar Ivy

  3. Department of Cardiac Surgery, German Pediatric Heart Center, Sankt Augustin, Germany

    Viktor Hraska

  4. Pediatric Cardiac Surgery, Children's Hospital Colorado University of Colorado, Aurora, CO, USA

    James Jaggers

Section Editor information

  1. No affiliation provided

    Eduardo M. da Cruz

  2. No affiliation provided

    James Jaggers

  3. No affiliation provided

    Dunbar Ivy

  4. No affiliation provided

    John Kim

Rights and permissions

Reprints and permissions

Copyright information

© 2023 Springer-Verlag London Ltd., part of Springer Nature

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Vanderlaan, R.D., Caldarone, C.A., Lacour-Gayet, F., Lopez, L., Davidson, J., da Cruz, E.M. (2023). Pulmonary Vein Stenosis. In: da Cruz, E.M., Ivy, D., Hraska, V., Jaggers, J. (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer, London. https://doi.org/10.1007/978-1-4471-4999-6_44-2

Download citation

  • DOI: https://doi.org/10.1007/978-1-4471-4999-6_44-2

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-4999-6

  • Online ISBN: 978-1-4471-4999-6

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation