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Case Reports
. 2009 Nov-Dec;16(6):e77-80.
doi: 10.1155/2009/791653.

Pulmonary vein stenosis: Case report and literature review

R Amin  1 , S Kwon, Y Moayedi, N Sweezey
Affiliations
Case Reports

Pulmonary vein stenosis: Case report and literature review

R Amin et al. Can Respir J. 2009 Nov-Dec.

Abstract
in English, French

Background: Pulmonary vein stenosis is a rare cause of pulmonary hypertension, with variable onset and presentation. One or more of the four pulmonary veins can be primarily or secondarily affected. A five-month-old girl presented with respiratory distress, lethargy and cyanosis requiring intubation.

Methods: Echocardiography showed right ventricular dilation, a right ventricular systolic pressure of 97 mmHg, decreased ejection fraction and turbulent flow at the left atrium. Cardiac catheterization revealed stenosis of the left-sided pulmonary veins, for which she underwent a Coles procedure.

Results: Postoperatively, there was a transient improvement in the patient's pulmonary hypertension but she subsequently deteriorated. Her prognosis was considered bleak, and a decision was made with the family to withdraw care.

Conclusion: Pulmonary vein stenosis is a rare cause of pulmonary hypertension, and is associated with significant morbidity and mortality. Surgical intervention may be of benefit in selected cases. It stands to reason that any treatment will have the best chance of success if completed before the pulmonary hypertension becomes fixed. Early diagnosis depends on a high index of clinical suspicion.

HISTORIQUE :: La sténose des veines pulmonaires est une cause rare d’hypertension pulmonaire, à l’apparition et à la présentation variables. Au moins l’une des quatre veines pulmonaires peut faire l’objet d’une atteinte primaire ou secondaire. Une fillette de cinq mois a consulté en raison d’une détresse respiratoire, d’une léthargie et d’une cyanose exigeant une intubation.

MÉTHODOLOGIE :: L’échocardiographie a révélé une dilatation ventriculaire droite, une pression systolique ventriculaire droite de 97 mmHg, une fraction d’éjection réduite et un débit turbulent à l’oreillette droite. Le cathétérisme cardiaque a signalé une sténose des veines pulmonaires gauches, pour laquelle la patiente a subi une intervention de Coles.

RÉSULTATS :: Après l’opération, on a constaté une amélioration transitoire de l’hypertension de la patiente, dont l’état s’est ensuite détérioré. Son pronostic était considéré comme peu réjouissant, et il a été entendu avec la famille de suspendre les soins.

CONCLUSION :: La sténose des veines pulmonaires est une cause rare d’hypertension pulmonaire et s’associe à une morbidité et une mortalité importantes. L’intervention chirurgicale peut être bénéfique dans certains cas. Il va sans dire que le traitement aura de meilleures chances de réussir s’il est administré avant que l’hypertension pulmonaire se soit fixée. Le diagnostic précoce dépend d’un fort indice de présomption clinique.

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Figures

Figure 1)
Figure 1)
Echocardiogram showing a dilated right ventricle compressing the left ventricle and a bowed interventricular septum
Figure 2)
Figure 2)
Computed tomography scan of the chest performed after the patient deteriorated. A Increased bilateral reticular markings in the lung fields. B Enlarged pulmonary artery
See this image and copyright information in PMC

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