Pulmonary Vein Stenosis: Case Report and Literature Review

Abstract

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.

Congenital pulmonary vein stenosis at an African tertiary care centre over a 25-year period

“…(6) Congenital PVS is thought to result from abnormal incorporation of the common pulmonary vein into the left atrium. (1)(2)(3) One or more of the 4 pulmonary veins may be involved, and may be unilateral on the left or right or bilateral and involve either lung. (1)(2)(3)(4)(5) All the cases of PVS in our study were unilateral and right sided, and only one or 2 pulmonary veins were an older age, (8) which may be the reason for the later presentation and diagnosis in our patients -all of whom had involvement of 1 -2 pulmonary veins (Table 1).…”

“…Congenital PVS is caused by a progressive neoproliferative process and may not be present at birth. (2,3) It is also possible that the study patients have a more benign disease process, which progresses more slowly, and hence the late presentation and diagnosis.…”

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INTRODUCTIONCongenital pulmonary vein stenosis (PVS) is a rare condition, resulting from abnormal incorporation of the common pulmonary vein into the left atrium. (1)(2)(3) One or more of the 4 pulmonary veins may be involved and it is commonly associated with other congenital heart defects (CHD), but can occur in isolation. (2)(3)(4) Commonly, patients present with respiratory symptoms with varying degrees of pulmonary hypertension.

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“…(1)(2)(3) One or more of the 4 pulmonary veins may be involved and it is commonly associated with other congenital heart defects (CHD), but can occur in isolation. (2)(3)(4) Commonly, patients present with respiratory symptoms with varying degrees of pulmonary hypertension. (5,6) Surgical results are generally poor with a high risk of recurrence and high mortality.(3) Reports are limited to small case series with little data on outcomes.…”

Pulmonary-Vein Stenosis Can Mimic Massive Pulmonary Embolism After Radiofrequency Ablation for Atrial Fibrillation

“…Pulmonary-vein stenosis and pulmonary embolism share similar nonspecific symptoms, and these patients range from asymptomatic to highly symptomatic, with cough, shortness of breath, chest pain, fatigue, and/or hemoptysis. 7,9 Also, V /Q mismatch or perfusion defect of a certain lobe on V /Q scan occurs in 80% of pulmonary-vein-stenosis patients, and a higher percentage in pulmonary-embolism patients. [10][11][12][13][14] Thus, it is hard to distinguish between pulmonary-vein stenosis and pulmonary embolism based on symptoms and V /Q scan.…”