Pulmonary vein stenosis is a rare cardiovascular disorder. It is recognized as being the stenosis of one or more of the four pulmonary veins that return blood from the lungs to the left atrium of the heart. In congenital cases, it is associated with poor prognosis and high mortality rate. In some people, pulmonary vein stenosis occurs after pulmonary vein ablation for the treatment of atrial fibrillation.[1] Some recent research has indicated that it may be genetically linked in congenital cases.[2][3]
| Pulmonary vein stenosis | |
|---|---|
 | |
| Pulmonary veins | |
| Specialty | Cardiovascular |
Signs and symptoms
editPulmonary vein stenosis usually manifests during the initial years of life.[4] Pneumonia recurrentis and tachypnea are common presentation symptoms rather than the typical early pulmonary hypertension symptoms.[5] Patients may experience a new murmur, right ventricular heart failure, pulmonary edema, failure to thrive, or hemoptysis as the disease worsens and pulmonary hypertension becomes more noticeable.[4]
Causes
editPulmonary vein stenosis can be congenital or acquired.[6]
A rare abnormality that accounts for 0.4% of congenital heart diseases, congenital pulmonary vein stenosis results from the common right or left pulmonary vein failing to integrate into the left atrium (LA) during the vessel's embryonic development, obliterating the pulmonary veins partially or completely on one or both sides.[7]
The main cause of pulmonary vein stenosis is radiofrequency ablation for atrial fibrillation.[6]
Pneumonia resulting from sarcoidosis may be caused by granulomatous involvement or extrinsic compression due to lymphadenopathies.[8][9] Uncontrolled fibrosis surrounding the afflicted mediastinal lymph nodes is the hallmark of fibrosing mediastinitis, a rare consequence of tuberculosis and Histoplasma capsulatum infection that can cause invasion as well as obstruction of the surrounding pulmonary veins.[10] Neoplasms next to the pulmonary veins have the potential to compress or infiltrate, resulting in stenosis.[11][12]
In the pediatric population, the most common cause of clinically significant pulmonary vein stenosis is complete anomalous pulmonary venous return repair.[13][14] Localized obliteration can occur further into the center of the vessel or at the point where the pulmonary vein anastomoses into the left atrium.[6] The literature contains isolated reports of lung transplantation,[15] suture repair of a pulmonary vein cannulation site,[16] and pulmonary vein injury resulting in obstruction following myxoma resection.[17]
Diagnosis
editPulmonary vein stenosis may be identified by noninvasive procedures such as magnetic resonance imaging, multidetector CT angiography, radionuclide quantitative pulmonary flow imaging, and echocardiography with Doppler ultrasonography.[5][18]
Treatment
editAlthough clinical and imaging surveillance is recommended because the disease can change over time, mild and asymptomatic pulmonary vein stenosis may not require intervention. When it comes to the majority of pulmonary vein stenosis cases, whether congenital or acquired, surgery is the recommended course of action.[6]
Systemic Sirolimus therapy has shown promising results for the treatment of pulmonary vein stenosis. With one trial managing to achieve a 100% survival rate in a small cohort of patients with severe disease.
References
edit- ^ Latson, Larry; Prieto, Lourdes (2007). "Congenital and Acquired Pulmonary Vein Stenosis". Circulation. 115 (1). American Heart Association: 103–8. doi:10.1161/CIRCULATIONAHA.106.646166. PMID 17200453. Retrieved 3 November 2014.
- ^ Chen, Ming (April 1, 2014). "Clinical and Genetic Features of Pulmonary Vein Stenosis". Journal of the American College of Cardiology. 63 (12): A524. doi:10.1016/S0735-1097(14)60524-6. Retrieved November 2, 2014.
- ^ Laar, Ingrid (July 4, 2009). "First locus for primary pulmonary vein stenosis maps to chromosome 2q". European Heart Journal. 30 (20): 2485–92. doi:10.1093/eurheartj/ehp271. PMID 19578166.
- ^ a b Amin, R; Kwon, S; Moayedi, Y; Sweezey, N (2009). "Pulmonary Vein Stenosis: Case Report and Literature Review". Canadian Respiratory Journal. 16 (6). Hindawi Limited: e77–e80. doi:10.1155/2009/791653. ISSN 1198-2241. PMC 2807798. PMID 20011721.
- ^ a b Latson, Larry A.; Prieto, Lourdes R. (January 2, 2007). "Congenital and Acquired Pulmonary Vein Stenosis". Circulation. 115 (1). Ovid Technologies (Wolters Kluwer Health): 103–108. doi:10.1161/circulationaha.106.646166. ISSN 0009-7322. PMID 17200453.
- ^ a b c d Pazos-López, Pablo (2016). "Pulmonary vein stenosis: Etiology, diagnosis and management". World Journal of Cardiology. 8 (1). Baishideng Publishing Group Inc.: 81–88. doi:10.4330/wjc.v8.i1.81. ISSN 1949-8462. PMC 4728109. PMID 26839659.
- ^ Porres, Diego Varona; Morenza, Óscar Persiva; Pallisa, Esther; Roque, Alberto; Andreu, Jorge; Martínez, Manel (2013). "Learning from the Pulmonary Veins". RadioGraphics. 33 (4). Radiological Society of North America (RSNA): 999–1022. doi:10.1148/rg.334125043. ISSN 0271-5333. PMID 23842969.
- ^ Padia, Siddharth A.; Budev, Marie; Farver, Carol F.; Mohammed, Tan-Lucien H. (2007). "Intravascular Sarcoidosis Presenting as Pulmonary Vein Occlusion". Journal of Thoracic Imaging. 22 (3). Ovid Technologies (Wolters Kluwer Health): 268–270. doi:10.1097/rti.0b013e3180437e3f. ISSN 0883-5993. PMID 17721340.
- ^ Gomes, Matthieu; Bendaoud, Sofiane; Wemeau-Stervinou, Lidwine; Faivre, Jean-Baptiste; Duhamel, Alain; Wallaert, Benoit; Remy, Jacques; Remy-Jardin, Martine (2015). "Prevalence of Venoatrial Compression by Lymphadenopathy in Sarcoidosis". Journal of Thoracic Imaging. 30 (4). Ovid Technologies (Wolters Kluwer Health): 268–273. doi:10.1097/rti.0000000000000134. ISSN 0883-5993. PMID 25730555. S2CID 22138786.
- ^ Albers, Erin L.; Pugh, Meredith E.; Hill, Kevin D.; Wang, Li; Loyd, James E.; Doyle, Thomas P. (April 5, 2011). "Percutaneous Vascular Stent Implantation as Treatment for Central Vascular Obstruction Due to Fibrosing Mediastinitis". Circulation. 123 (13). Ovid Technologies (Wolters Kluwer Health): 1391–1399. doi:10.1161/circulationaha.110.949180. ISSN 0009-7322. PMC 3095436. PMID 21422386.
- ^ Hamzeh, Ihab; Rashid, Abdul; Shaib, Fidaa; Dawn, Buddhadeb (January 25, 2011). "Pulmonary Vein Stenosis Due to a Compressive Malignant Tumor Detected by Transesophageal Echocardiography". Circulation. 123 (3). Ovid Technologies (Wolters Kluwer Health): 349–350. doi:10.1161/circulationaha.110.958082. ISSN 0009-7322. PMID 21263008.
- ^ Morjaria, J B; Choong, C K; Amsha, K; Stewart, S; Wells, F C; Rintoul, R C (August 28, 2009). "Small cell lung cancer mimicking a pulmonary venous angiosarcoma". Thorax. 64 (9). BMJ: 827–828. doi:10.1136/thx.2008.109306. ISSN 0040-6376. PMID 19717720.
- ^ Caldarone, Christopher A; Najm, Hani K; Kadletz, Margit; Smallhorn, Jeffrey F; Freedom, Robert M; Williams, William G; Coles, John G (1998). "Relentless pulmonary vein stenosis after repair of total anomalous pulmonary venous drainage". The Annals of Thoracic Surgery. 66 (5). Elsevier BV: 1514–1519. doi:10.1016/s0003-4975(98)00952-7. ISSN 0003-4975. PMID 9875744.
- ^ Hancock Friesen, Camille L.; Zurakowski, David; Thiagarajan, Ravi R.; Forbess, Joseph M.; del Nido, Pedro J.; Mayer, John E.; Jonas, Richard A. (2005). "Total Anomalous Pulmonary Venous Connection: An Analysis of Current Management Strategies in a Single Institution". The Annals of Thoracic Surgery. 79 (2). Elsevier BV: 596–606. doi:10.1016/j.athoracsur.2004.07.005. ISSN 0003-4975. PMID 15680843.
- ^ Pazos-López, Pablo; Piñeiro-Portela, Miriam; Bouzas-Mosquera, Alberto; Peteiro-Vázquez, Jesús; Vázquez-Gonzalez, Nicolás; Rueda-Nuñez, Fernando; Duro-Tacón, José; Fernández-Prado, Ricardo; Martínez-Sapiña, María José; Castro-Beiras, Alfonso (December 21, 2010). "Pulmonary Vein Stenosis After Lung Transplantation Successfully Treated With Stent Implantation". Circulation. 122 (25). Ovid Technologies (Wolters Kluwer Health): 2745–2747. doi:10.1161/circulationaha.110.973370. ISSN 0009-7322. PMID 21173363.
- ^ Booher, Anna M.; Bach, David S. (2010). "Acquired Pulmonary Vein Stenosis: One Problem, Two Mechanisms". Journal of the American Society of Echocardiography. 23 (8). Elsevier BV: 904.e1–904.e3. doi:10.1016/j.echo.2009.12.015. ISSN 0894-7317. PMID 20138470.
- ^ Rosetti, Mihaela; Tighe, Dennis A.; Chandok, Dinesh; Gammie, James S.; Griffith, Bartley P.; Folland, Edward D. (June 28, 2006). "An Unusual Cause of Pulmonary Vein Stenosis: A Case Report and Review of the Literature". Echocardiography. 23 (8). Wiley: 685–688. doi:10.1111/j.1540-8175.2006.00293.x. ISSN 0742-2822. PMID 16970720. S2CID 887689.
- ^ Holt, D. Byron; Moller, James H.; Larson, Sarah; Johnson, Mark C. (2007). "Primary Pulmonary Vein Stenosis". The American Journal of Cardiology. 99 (4). Elsevier BV: 568–572. doi:10.1016/j.amjcard.2006.09.100. ISSN 0002-9149. PMID 17293205.
Further reading
edit- McLennan, Daniel I.; Solano, Elyan C. Ruiz; Handler, Stephanie S.; Lincoln, Joy; Mitchell, Michael E.; Kirkpatrick, Edward C. (October 5, 2021). "Pulmonary Vein Stenosis: Moving From Past Pessimism to Future Optimism". Frontiers in Pediatrics. 9. Frontiers Media SA. doi:10.3389/fped.2021.747812. ISSN 2296-2360. PMC 8524035.