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Pulmonary vein stenosis

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Medical condition
Pulmonary vein stenosis
Pulmonary veins
SpecialtyCardiovascular

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. Some recent research has indicated that it may be genetically linked in congenital cases.

Signs and symptoms

Pulmonary vein stenosis usually manifests during the initial years of life. Pneumonia recurrentis and tachypnea are common presentation symptoms rather than the typical early pulmonary hypertension symptoms. 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.

Causes

Pulmonary vein stenosis can be congenital or acquired.

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.

The main cause of pulmonary vein stenosis is radiofrequency ablation for atrial fibrillation.

Pneumonia resulting from sarcoidosis may be caused by granulomatous involvement or extrinsic compression due to lymphadenopathies. 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. Neoplasms next to the pulmonary veins have the potential to compress or infiltrate, resulting in stenosis.

In the pediatric population, the most common cause of clinically significant pulmonary vein stenosis is complete anomalous pulmonary venous return repair. Localized obliteration can occur further into the center of the vessel or at the point where the pulmonary vein anastomoses into the left atrium. The literature contains isolated reports of lung transplantation, suture repair of a pulmonary vein cannulation site, and pulmonary vein injury resulting in obstruction following myxoma resection.

Diagnosis

Pulmonary 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.

Treatment

Although 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.

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

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  2. 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.
  3. 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.
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  11. 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.
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  13. 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.
  14. 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.
  15. 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.
  16. 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.
  17. 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.
  18. 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

External links

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