Relationship between Clinical Features and Histopathologic Findings in Patients with Pulmonary Vein Stenosis

  • Y. Mykychak Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
  • D. Kozhokar Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
  • I. Yusifli Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
  • O. Yachnik Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
  • V. Zakharova National M. M. Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine, Kyiv, Ukraine
  • I. Yemets Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
Keywords: pulmonary vein stenosis, pulmonary hypertension, total anomalous pulmonary venous connection, intimal proliferation


Pulmonary vein stenosis (PVS) is a rare but serious condition characterized by obstruction of extrapulmonary segments of pulmonary veins which leads to progressive pulmonary hypertension.

Objective. In this study we aimed to determine relationship between clinical features and histopathologic findings in patients with PVS.

Material and methods. We retrospectively reviewed 34 consecutive patients who underwent PV stenosis repair. Surgical wedge biopsy specimens were collected intraoperatively in 11 patients and reviewed using light microscopy.

Results. Affected pulmonary veins in patients with primary PVS were characterized by diffuse stenosis extending into the lung parenchyma. In post-surgical group stenosis was found in a limited segment of pulmonary vein at its ostium. Microscopically, abnormal intimal proliferation was identified in both patient groups. Scaring was predominant finding in patients with post-surgical PVS.

Conclusion. In patients with PVS, pathophysiological mechanism influences the severity and extent of clinical manifestations. A comprehensive understanding of this mechanism may improve results of the treatment.


  1. Bernstein J, Noke AC, Reed JO. Extrapulmonic stenosis of the pulmonary veins. Circulation. 1959;19:891–7.
  2. 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 Sep;41(9):1198–204.
  3. Caldarone CA, Najm HK, Kadletz M, Smallhorn JF, Freedom RM, Williams WG, et al. Relentless pulmonary vein stenosis after repair of total anomalous pulmonary venous drainage. Ann Thorac Surg. 1998;66:1514–20.
  4. Seale AN, Uemura H, Webber SA, Partridge J, Roughton M, Ho SY, et al. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation. 2010;122:2718–26.
  5. 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.
  6. Riedlinger WFJ, 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.
  7. 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 Jul;104(1):182–9.
  8. Kalfa D, Belli E, Bacha E, Lambert V, di Carlo D, Kostolny M, et al. Outcomes and Prognostic Factors for Post-Surgical Pulmonary Vein Stenosis in the Current Era. J Thorac Cardiovasc Surg. 2018 Jul;156(1):278–86.
  9. 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(6):608–23.
  10. CallahanR, Kieran M, Baird C, Colan S, Gauvreau K, Ireland C, et al. Adjunct targeted biological inhibition agents to treat aggressive multi-vessel intraluminal pediatric pulmonary vein stenosis. J Pediatr. 2018;198:29–35.
  11. 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 Jul;148(1):245–53.
  12. Mykychak Y. Complex treatment of recurrent pulmonary vein stenosis. Herald Cardiovascular Surgery. 2018 Nov;4(33):94–6.
How to Cite
Mykychak, Y., Kozhokar, D., Yusifli, I., Yachnik, O., Zakharova, V., & Yemets, I. (2019). Relationship between Clinical Features and Histopathologic Findings in Patients with Pulmonary Vein Stenosis. Ukrainian Journal of Cardiovascular Surgery, (2 (35), 61-63.