Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Review Article
  • Published:

Primary pulmonary vein stenosis during infancy: state of the art review

Journal of Perinatology volume 41, pages 1528–1539 (2021)Cite this article

Subjects

Abstract

Primary pulmonary vein stenosis (PPVS) is an emerging problem among infants. In contrast to acquired disease, PPVS is the development of stenosis in the absence of preceding intervention. While optimal care approaches remain poorly characterized, over the past decade, understanding of potential pathophysiological mechanisms and development of novel therapeutic strategies are increasing. A multidisciplinary team of health care providers was assembled to review the available evidence and provide a common framework for the diagnosis, management, and treatment of PPVS during infancy. To address knowledge gaps, institutional and multi-institutional approaches must be employed to generate knowledge specific to ex-premature infants with PPVS. Within individual institutions, creation of a team comprised of dedicated health care providers from diverse backgrounds is critical to accelerate clinical learning and provide care for infants with PPVS. Multi-institutional collaborations, such as the PVS Network, provide the infrastructure and statistical power to advance knowledge for this rare disease.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Fig. 1: Echocardiographic images of pulmonary vein stenosis.
Fig. 2: Computed topography angiography (CTA) image depicting stenoses of the left upper and lower pulmonary veins.
Fig. 3: Anteroposterior and lateral angiograms of a 6-month-old patient with stenosis of the common left pulmonary vein.

Similar content being viewed by others

References

  1. Latson LA, Prieto LR. Congenital and acquired pulmonary vein stenosis. Circulation. 2007;115:103–8.

    Article  PubMed  Google Scholar 

  2. Breinholt JP, Hawkins JA, Minich LA, Tani LY, Orsmond GS, Ritter S, et al. Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome. Ann Thorac Surg. 1999;68:164–8.

    Article  CAS  PubMed  Google Scholar 

  3. Drossner DM, Kim DW, Maher KO, Mahle WT. Pulmonary vein stenosis: prematurity and associated conditions. Pediatrics. 2008;122:e656–61.

    Article  PubMed  Google Scholar 

  4. Devaney EJ, Ohye RG, Bove EL. Pulmonary vein stenosis following repair of total anomalous pulmonary venous connection. Semin Thorac Cardiovasc Surg Pediatr Cardiac Surg Annu. 2006;9:51–5.

  5. Backes CH, Nealon E, Armstrong AK, Cua CL, Mitchell C, Krishnan U, et al. Pulmonary vein stenosis in infants: a systematic review, meta-analysis, and meta-regression. J Pediatr. 2018;198:36–45 e33.

    Article  PubMed  Google Scholar 

  6. Krishnan U, Feinstein JA, Adatia I, Austin ED, Mullen MP, Hopper RK, et al. Evaluation and management of pulmonary hypertension in children with bronchopulmonary dysplasia. J Pediatr. 2017;188:24–34 e21.

    Article  PubMed  Google Scholar 

  7. Levy PT, Jain A, Nawaytou H, Teitel D, Keller R, Fineman J, et al. Risk assessment and monitoring of chronic pulmonary hypertension in premature infants. J Pediatr. 2020;217:199–209 e194.

    Article  PubMed  Google Scholar 

  8. Mahgoub L, Kaddoura T, Kameny AR, Lopez Ortego P, Vanderlaan RD, Kakadekar A, et al. Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort. Pediatr Pulmonol. 2017;52:1063–70.

    Article  PubMed  Google Scholar 

  9. Prosnitz AR, Leopold J, Irons M, Jenkins K, Roberts AE. Pulmonary vein stenosis in patients with Smith-Lemli-Opitz syndrome. Congenit Heart Dis. 2017;12:475–83.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Callahan R, Kieran MW, Baird CW, Colan SD, Gauvreau K, Ireland CM, et al. Adjunct targeted biologic inhibition agents to treat aggressive multivessel intraluminal pediatric pulmonary vein stenosis. J Pediatr. 2018;198:29–35 e25.

    Article  PubMed  Google Scholar 

  11. DiLorenzo MP, Santo A, Rome JJ, Zhang H, Faerber JA, Mercer-Rosa L, et al. Pulmonary vein stenosis: outcomes in children with congenital heart disease and prematurity. Semin Thorac Cardiovasc Surg. 2019;31:266–73.

    Article  PubMed  Google Scholar 

  12. Kalfa D, Belli E, Bacha E, Lambert V, di Carlo D, Kostolny M, et al. Primary pulmonary vein stenosis: outcomes, risk factors, and severity score in a multicentric study. Ann Thorac Surg. 2017;104:182–9.

    Article  PubMed  Google Scholar 

  13. Seale AN, Webber SA, Uemura H, Partridge J, Roughton M, Ho SY, et al. Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study. Heart. 2009;95:1944–9.

    Article  CAS  PubMed  Google Scholar 

  14. Gowda S, Bhat D, Feng Z, Chang CH, Ross RD. Pulmonary vein stenosis with Down syndrome: a rare and frequently fatal cause of pulmonary hypertension in infants and children. Congenit Heart Dis. 2014;9:E90–7.

    Article  PubMed  Google Scholar 

  15. van de Laar I, Wessels M, Frohn-Mulder I, Dalinghaus M, de Graaf B, van Tienhoven M, et al. First locus for primary pulmonary vein stenosis maps to chromosome 2q. Eur Heart J. 2009;30:2485–92.

    Article  PubMed  CAS  Google Scholar 

  16. Braun S, Platzek I, Zophel K, Weise M, Kolditz M, Halank M, et al. Haemoptysis due to pulmonary venous stenosis. Eur Respir Rev. 2014;23:170–9.

    Article  PubMed  Google Scholar 

  17. Heching HJ, Turner M, Farkouh-Karoleski C, Krishnan U. Pulmonary vein stenosis and necrotising enterocolitis: is there a possible link with necrotising enterocolitis? Arch Dis Child Fetal Neonatal Ed. 2014;99:F282–5.

    Article  PubMed  Google Scholar 

  18. Kotani Y, Zhu J, Grosse-Wortmann L, Honjo O, Coles JG, Van Arsdell GS, et al. Anatomical risk factors, surgical treatment, and clinical outcomes of left-sided pulmonary vein obstruction in single-ventricle patients. J Thorac Cardiovasc Surg. 2015;149:1332–8.

    Article  PubMed  Google Scholar 

  19. Swier NL, Richards B, Cua CL, Lynch SK, Yin H, Nelin LD, et al. Pulmonary vein stenosis in neonates with severe bronchopulmonary dysplasia. Am J Perinatol. 2016;33:671–7.

    Article  PubMed  Google Scholar 

  20. Kovach AE, Magcalas PM, Ireland C, McEnany K, Oliveira AM, Kieran MW, et al. Paucicellular fibrointimal proliferation characterizes pediatric pulmonary vein stenosis: clinicopathologic analysis of 213 samples from 97 patients. Am J Surg Pathol. 2017;41:1198–204.

    Article  PubMed  Google Scholar 

  21. Pogoriler JE, Kulik TJ, Casey AM, Baird CW, Mullen MP, Jenkins KJ, et al. Lung pathology in pediatric pulmonary vein stenosis. Pediatr Dev Pathol. 2016;19:219–29.

    Article  PubMed  Google Scholar 

  22. Sadr IM, Tan PE, Kieran MW, Jenkins KJ. Mechanism of pulmonary vein stenosis in infants with normally connected veins. Am J Cardiol. 2000;86:577–9, A510.

    Article  CAS  PubMed  Google Scholar 

  23. Blom NA, Gittenberger-de Groot AC, Jongeneel TH, DeRuiter MC, Poelmann RE, Ottenkamp J. Normal development of the pulmonary veins in human embryos and formulation of a morphogenetic concept for sinus venosus defects. Am J Cardiol. 2001;87:305–9.

    Article  CAS  PubMed  Google Scholar 

  24. DeRuiter MC, Gittenberger-De Groot AC, Wenink AC, Poelmann RE, Mentink MM. In normal development pulmonary veins are connected to the sinus venosus segment in the left atrium. Anat Rec. 1995;243:84–92.

    Article  CAS  PubMed  Google Scholar 

  25. Tasaka H, Krug EL, Markwald RR. Origin of the pulmonary venous orifice in the mouse and its relation to the morphogenesis of the sinus venosus, extracardiac mesenchyme (spina vestibuli), and atrium. Anat Rec. 1996;246:107–13.

    Article  CAS  PubMed  Google Scholar 

  26. Webb S, Brown NA, Wessels A, Anderson RH. Development of the murine pulmonary vein and its relationship to the embryonic venous sinus. Anat Rec. 1998;250:325–34.

    Article  CAS  PubMed  Google Scholar 

  27. Webb S, Kanani M, Anderson RH, Richardson MK, Brown NA. Development of the human pulmonary vein and its incorporation in the morphologically left atrium. Cardiol Young. 2001;11:632–42.

    Article  CAS  PubMed  Google Scholar 

  28. Peng T, Tian Y, Boogerd CJ, Lu MM, Kadzik RS, Stewart KM, et al. Coordination of heart and lung co-development by a multipotent cardiopulmonary progenitor. Nature. 2013;500:589–92.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  29. Degenhardt K, Singh MK, Aghajanian H, Massera D, Wang Q, Li J, et al. Semaphorin 3d signaling defects are associated with anomalous pulmonary venous connections. Nat Med. 2013;19:760–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Bleyl SB, Saijoh Y, Bax NA, Gittenberger-de Groot AC, Wisse LJ, Chapman SC. et al. Dysregulation of the PDGFRA gene causes inflow tract anomalies including TAPVR: integrating evidence from human genetics and model organisms. Hum Mol Genet. 2010;19:1286–301.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Douglas YL, Jongbloed MR, den Hartog WC, Bartelings MM, Bogers AJ, Ebels T, et al. Pulmonary vein and atrial wall pathology in human total anomalous pulmonary venous connection. Int J Cardiol. 2009;134:302–12.

    Article  PubMed  Google Scholar 

  32. Mommersteeg MT, Soufan AT, de Lange FJ, van den Hoff MJ, Anderson RH, Christoffels VM, et al. Two distinct pools of mesenchyme contribute to the development of the atrial septum. Circ Res. 2006;99:351–3.

    Article  CAS  PubMed  Google Scholar 

  33. Riedlinger WF, Juraszek AL, Jenkins KJ, Nugent AW, Balasubramanian S, Calicchio ML, et al. Pulmonary vein stenosis: expression of receptor tyrosine kinases by lesional cells. Cardiovasc Pathol. 2006;15:91–9.

    Article  CAS  PubMed  Google Scholar 

  34. Nasr VG, Callahan R, Wichner Z, Odegard KC, DiNardo JA. Intraluminal pulmonary vein stenosis in children: a “new” lesion. Anesth Analg. 2019;129:27–40.

    Article  PubMed  Google Scholar 

  35. Chakrabarti S, Mittal R, Gnanapragasam JP, Martin RP. Acquired stenosis of normally connected pulmonary veins. Cardiol Young. 2007;17:322–7.

    Article  PubMed  Google Scholar 

  36. del Cerro MJ, Moledina S, Haworth SG, Ivy D, Al Dabbagh M, Banjar H, et al. Cardiac catheterization in children with pulmonary hypertensive vascular disease: consensus statement from the Pulmonary Vascular Research Institute, Pediatric and Congenital Heart Disease Task Forces. Pulm Circ. 2016;6:118–25.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  37. del Cerro MJ, Sabate Rotes A, Carton A, Deiros L, Bret M, Cordeiro M, et al. Pulmonary hypertension in bronchopulmonary dysplasia: clinical findings, cardiovascular anomalies and outcomes. Pediatr Pulmonol. 2014;49:49–59.

    Article  PubMed  Google Scholar 

  38. Holt DB, Moller JH, Larson S, Johnson MC. Primary pulmonary vein stenosis. Am J Cardiol. 2007;99:568–72.

    Article  PubMed  Google Scholar 

  39. Benjamin JT, Hamm CR, Zayek M, Eyal FG, Carlson S, Manci E. Acquired left-sided pulmonary vein stenosis in an extremely premature infant: a new entity? J Pediatr. 2009;154:459–459 e451.

    Article  PubMed  Google Scholar 

  40. Laux D, Rocchisani MA, Boudjemline Y, Gouton M, Bonnet D, Ovaert C. Pulmonary hypertension in the preterm infant with chronic lung disease can be caused by pulmonary vein stenosis: a must-know entity. Pediatr Cardiol. 2016;37:313–21.

    Article  PubMed  Google Scholar 

  41. Quinonez LG, Gauvreau K, Borisuk M, Ireland C, Marshall AM, Mayer JE, et al. Outcomes of surgery for young children with multivessel pulmonary vein stenosis. J Thorac Cardiovasc Surg. 2015;150:911–7.

    Article  PubMed  Google Scholar 

  42. Bussmann N, Breatnach C, Levy PT, McCallion N, Franklin O, El-Khuffash A. Early diastolic dysfunction and respiratory morbidity in premature infants: an observational study. J Perinatol. 2018;38:1205–11.

    Article  PubMed  Google Scholar 

  43. Lewandowski AJ, Augustine D, Lamata P, Davis EF, Lazdam M, Francis J, et al. Preterm heart in adult life: cardiovascular magnetic resonance reveals distinct differences in left ventricular mass, geometry, and function. Circulation. 2013;127:197–206.

    Article  PubMed  Google Scholar 

  44. Martin T, Smith A, Breatnach CR, Kent E, Shanahan I, Boyle M, et al. Infants born with Down syndrome: burden of disease in the early neonatal period. J Pediatr. 2018;193:21–6.

    Article  PubMed  Google Scholar 

  45. Shah PS, Hellmann J, Adatia I. Clinical characteristics and follow up of Down syndrome infants without congenital heart disease who presented with persistent pulmonary hypertension of newborn. J Perinat Med. 2004;32:168–70.

    Article  PubMed  Google Scholar 

  46. Smith AM, Levy PT, Franklin O, Molloy E, El-Khuffash A. Pulmonary hypertension and myocardial function in infants and children with Down syndrome. Arch Dis Child. 2020;105:1031–4.

  47. Lo Rito M, Gazzaz T, Wilder TJ, Vanderlaan RD, Van Arsdell GS, Honjo O, et al. Pulmonary vein stenosis: severity and location predict survival after surgical repair. J Thorac Cardiovasc Surg. 2016;151:657–6 e652.

    Article  PubMed  Google Scholar 

  48. Minich LL, Tani LY, Breinholt JP, Tuohy AM, Shaddy RE. Complete follow-up echocardiograms are needed to detect stenosis of normally connecting pulmonary veins. Echocardiography. 2001;18:589–92.

    Article  CAS  PubMed  Google Scholar 

  49. Rosenblum JM, Altin HF, Gillespie SE, Bauser-Heaton H, Kanter KA, Sinha R, et al. Management outcomes of primary pulmonary vein stenosis. J Thorac Cardiovasc Surg. 2020;159:1029–36 e1021.

    Article  PubMed  Google Scholar 

  50. Viola N, Alghamdi AA, Perrin DG, Wilson GJ, Coles JG, Caldarone CA. Primary pulmonary vein stenosis: the impact of sutureless repair on survival. J Thorac Cardiovasc Surg. 2011;142:344–50.

    Article  PubMed  Google Scholar 

  51. Yun TJ, Coles JG, Konstantinov IE, Al-Radi OO, Wald RM, Guerra V, et al. 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. 2005;129:167–74.

    Article  PubMed  Google Scholar 

  52. Feltes TF, Bacha E, Beekman RH 3rd, Cheatham JP, Feinstein JA, Gomes AS, et al. Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. Circulation. 2011;123:2607–52.

    Article  PubMed  Google Scholar 

  53. Humpl T, Fineman J, Qureshi AM. The many faces and outcomes of pulmonary vein stenosis in early childhood. Pediatr Pulmonol. 2020;56:649–55.

  54. Driscoll DJ, Hesslein PS, Mullins CE. Congenital stenosis of individual pulmonary veins: clinical spectrum and unsuccessful treatment by transvenous balloon dilation. Am J Cardiol. 1982;49:1767–72.

    Article  CAS  PubMed  Google Scholar 

  55. Peng LF, Lock JE, Nugent AW, Jenkins KJ, McElhinney DB. Comparison of conventional and cutting balloon angioplasty for congenital and postoperative pulmonary vein stenosis in infants and young children. Catheter Cardiovasc Inter. 2010;75:1084–90.

    Google Scholar 

  56. Prieto LR, Schoenhagen P, Arruda MJ, Natale A, Worley SE. Comparison of stent versus balloon angioplasty for pulmonary vein stenosis complicating pulmonary vein isolation. J Cardiovasc Electrophysiol. 2008;19:673–8.

    Article  PubMed  Google Scholar 

  57. Balasubramanian S, Marshall AC, Gauvreau K, Peng LF, Nugent AW, Lock JE, et al. Outcomes after stent implantation for the treatment of congenital and postoperative pulmonary vein stenosis in children. Circ Cardiovasc Inter. 2012;5:109–17.

    Article  Google Scholar 

  58. Furukawa T, Kishiro M, Fukunaga H, Ohtsuki M, Takahashi K, Akimoto K, et al. Drug-eluting stents ameliorate pulmonary vein stenotic changes in pigs in vivo. Pediatr Cardiol. 2010;31:773–9.

    Article  PubMed  Google Scholar 

  59. Khan A, Qureshi AM, Justino H. Comparison of drug eluting versus bare metal stents for pulmonary vein stenosis in childhood. Catheter Cardiovasc Inter. 2019;94:233–42.

    Article  Google Scholar 

  60. Cory MJ, Ooi YK, Kelleman MS, Vincent RN, Kim DW, Petit CJ. Reintervention is associated with improved survival in pediatric patients with pulmonary vein stenosis. JACC Cardiovasc Inter. 2017;10:1788–98.

    Article  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  62. van Son JA, Danielson GK, Puga FJ, Edwards WD, Driscoll DJ. Repair of congenital and acquired pulmonary vein stenosis. Ann Thorac Surg. 1995;60:144–50.

    Article  PubMed  Google Scholar 

  63. Pacifico AD, Mandke NV, McGrath LB, Colvin EV, Bini RM, Bargeron LM Jr. Repair of congenital pulmonary venous stenosis with living autologous atrial tissue. J Thorac Cardiovasc Surg. 1985;89:604–9.

    Article  CAS  PubMed  Google Scholar 

  64. Najm HK, Caldarone CA, Smallhorn J, Coles JG. A sutureless technique for the relief of pulmonary vein stenosis with the use of in situ pericardium. J Thorac Cardiovasc Surg. 1998;115:468–70.

    Article  CAS  PubMed  Google Scholar 

  65. Lacour-Gayet F, Rey C, Planche C. Pulmonary vein stenosis. Description of a sutureless surgical procedure using the pericardium in situ. Arch Mal Coeur Vaiss. 1996;89:633–6.

    CAS  PubMed  Google Scholar 

  66. Lacour-Gayet F. Surgery for pulmonary venous obstruction after repair of total anomalous pulmonary venous return. Semin Thorac Cardiovasc Surg Pediatr Cardiac Surg Annu. 2006;89:45–50.

  67. Lacour-Gayet F, Zoghbi J, Serraf AE, Belli E, Piot D, Rey C, et al. Surgical management of progressive pulmonary venous obstruction after repair of total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg. 1999;117:679–87.

    Article  CAS  PubMed  Google Scholar 

  68. McElhinney DB, Reddy VM, Moore P, Hanley FL. Revision of previous Fontan connections to extracardiac or intraatrial conduit cavopulmonary anastomosis. Ann Thorac Surg. 1996;62:1276–82.

    Article  CAS  PubMed  Google Scholar 

  69. Kreutzer J, Keane JF, Lock JE, Walsh EP, Jonas RA, Castaneda AR, et al. Conversion of modified Fontan procedure to lateral atrial tunnel cavopulmonary anastomosis. J Thorac Cardiovasc Surg. 1996;111:1169–76.

    Article  CAS  PubMed  Google Scholar 

  70. O’Donnell CP, Lock JE, Powell AJ, Perry SB. Compression of pulmonary veins between the left atrium and the descending aorta. Am J Cardiol. 2003;91:248–51.

    Article  PubMed  Google Scholar 

  71. Baird C. Anatomic-based considerations in the surgical approach to PVS. 2020.

  72. Bibevski S, Ruzmetov M, Fortuna RS, Turrentine MW, Brown JW, Ohye RG. Performance of SynerGraft decellularized pulmonary allografts compared with standard cryopreserved allografts: results from multiinstitutional data. Ann Thorac Surg. 2017;103:869–74.

    Article  PubMed  Google Scholar 

  73. Feins EN, Ireland CM, Chavez M, Gauvreau K, Callahan R, Jenkins KJ, et al. Pulmonary vein stenosis management & outcomes - a large single center retrospective review [Abstract]. Proceedings of the 100th Annual Meeting of the American Association for Thoracic Surgery; 2020.

  74. Rehman M, Jenkins KJ, Juraszek AL, Connor JA, Gauvreau K, Muneeb M, et al. A prospective phase II trial of vinblastine and methotrexate in multivessel intraluminal pulmonary vein stenosis in infants and children. Congenit Heart Dis. 2011;6:608–23.

    Article  PubMed  Google Scholar 

  75. Masaki N, Adachi O, Katahira S, Saiki Y, Horii A, Kawamoto S, et al. Progression of vascular remodeling in pulmonary vein obstruction. J Thorac Cardiovasc Surg. 2020;160:777–90 e775.

    Article  PubMed  Google Scholar 

  76. Callahan R, Esch JJ, Wang G, Ireland CM, Gauvreau K, Jenkins KJ. Systemic sirolimus to prevent in-stent stenosis in pediatric pulmonary vein stenosis. Pediatr Cardiol. 2020;41:282–9.

    Article  PubMed  Google Scholar 

  77. Lacro RV, Dietz HC, Sleeper LA, Yetman AT, Bradley TJ, Colan SD, et al. Atenolol versus losartan in children and young adults with Marfan’s syndrome. N Engl J Med. 2014;371:2061–71.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  78. Kato H, Fu YY, Zhu J, Wang L, Aafaqi S, Rahkonen O, et al. Pulmonary vein stenosis and the pathophysiology of “upstream” pulmonary veins. J Thorac Cardiovasc Surg. 2014;148:245–53.

    Article  CAS  PubMed  Google Scholar 

  79. Zhu J, Ide H, Fu YY, Teichert AM, Kato H, Weisel RD, et al. Losartan ameliorates “upstream” pulmonary vein vasculopathy in a piglet model of pulmonary vein stenosis. J Thorac Cardiovasc Surg. 2014;148:2550–7.

    Article  CAS  PubMed  Google Scholar 

  80. Vanderlaan RD, Honjo O. Commentary: it takes a village: Changing the trajectory of pulmonary vein stenosis outcomes. J Thorac Cardiovasc Surg. 2020;159:1037–8.

    Article  PubMed  Google Scholar 

  81. Vanderlaan RD, Rome J, Hirsch R, Ivy D, Caldarone CA. Pulmonary vein stenosis: treatment and challenges. J Thorac Cardiovasc Surg. 2020. (in press, corrected proof).

  82. Vanderlaan RD, Caldarone CA. PVS Network. 2017. http://www.pvsnetwork.org. Accessed November 1st, 2020.

Download references

Funding

This work was funded by Ohio Perinatal Research Network within the Abigail Wexner Research Institute at Nationwide Children’s Hospital. CHB received funding from the National Heart, Lung, and Blood Institute of the US National Institutes of Health (R01HL145032).

Author information

Authors and Affiliations

  1. Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    David B. Frank

  2. Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    David B. Frank

  3. Division of Newborn Medicine, Boston Children’s Hospital, Boston, MA, USA

    Philip T. Levy

  4. Department of Pediatrics, Harvard Medical School, Boston, MA, USA

    Philip T. Levy, Christopher W. Baird & Ryan M. Callahan

  5. The Heart Center, Nationwide Children’s Hospital, Columbus, OH, USA

    Corey A. Stiver, Brian A. Boe & Carl H. Backes

  6. Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, USA

    Corey A. Stiver, Brian A. Boe & Carl H. Backes

  7. Department of Cardiac Surgery, Boston Children’s Hospital, Boston, MA, USA

    Christopher W. Baird

  8. Department of Cardiology, Boston Children’s Hospital, Boston, MA, USA

    Ryan M. Callahan

  9. Center for Developmental Therapeutics, Seattle Children’s Research Institute, University of Washington School of Medicine, Seattle, WA, USA

    Charles V. Smith

  10. Department of Thoracic Surgery, New York Presbyterian Morgan Stanley Children’s Hospital, New York, NY, USA

    Rachel D. Vanderlaan

  11. Division of Neonatology, The Ohio State University Wexner Medical Center, Columbus, OH, USA

    Carl H. Backes

  12. Center for Perinatal Research, The Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, USA

    Carl H. Backes

Authors
  1. David B. Frank
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Philip T. Levy
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Corey A. Stiver
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Brian A. Boe
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Christopher W. Baird
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Ryan M. Callahan
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Charles V. Smith
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Rachel D. Vanderlaan
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Carl H. Backes
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Carl H. Backes.

Ethics declarations

Conflict of interest

The authors declare no competing interests.

Additional information

Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Frank, D.B., Levy, P.T., Stiver, C.A. et al. Primary pulmonary vein stenosis during infancy: state of the art review. J Perinatol 41, 1528–1539 (2021). https://doi.org/10.1038/s41372-021-01008-7

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/s41372-021-01008-7

This article is cited by

Search

Advanced search

Quick links