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Repair of congenital and acquired pulmonary vein stenosis
J A van Son1, G K Danielson, F J Puga
1Division of Thoracic and Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905, USA.
Insights
Surgical relief of pulmonary vein stenosis, a serious condition, can be rewarding. Prompt intervention improves patient outcomes, offering a better prognosis for those with congenital or acquired forms.
Area of Science:
- Cardiology
- Thoracic Surgery
- Pediatric Cardiology
Background:
- Pulmonary vein stenosis (PVS) is a rare but serious condition causing obstructed pulmonary venous blood flow with high mortality.
- Both congenital and acquired forms of PVS present significant clinical challenges.
Purpose of the Study:
- To evaluate the outcomes of surgical intervention for pulmonary vein stenosis.
- To assess the efficacy of surgical relief in improving functional status and survival.
Main Methods:
- Retrospective analysis of eight patients (3 months to 43 years) undergoing surgical correction of PVS.
- Patients included those with congenital PVS, acquired PVS post-total anomalous pulmonary venous connection repair, and PVS associated with mediastinal fibrosis.
Main Results:
- One infant mortality occurred post-correction of acquired PVS.
- At a median follow-up of 6.5 years (up to 16 years), 6 patients achieved New York Heart Association functional class I, and 1 patient was in class II.
Conclusions:
- Untreated pulmonary vein stenosis has a poor natural history.
- Prompt surgical intervention for PVS can lead to favorable long-term functional outcomes and is a potentially rewarding undertaking.
Background:
Congenital pulmonary vein stenosis is a rare cause of obstruction of pulmonary venous blood flow with a high mortality. Acquired pulmonary vein stenosis is an equally serious condition.
Methods:
Eight patients (age range, 3 months to 43 years; median age, 1.5 years) underwent surgical relief of pulmonary vein stenosis. Two had congenital pulmonary vein stenosis, 5 had pulmonary vein stenosis that was acquired after surgical treatment of total anomalous pulmonary venous connection, and 1 had pulmonary vein stenosis associated with idiopathic mediastinal fibrosis and calcification.
Results:
One infant died 2 months after correction of acquired pulmonary vein stenosis. At follow-up extending to 16 years (median follow-up, 6.5 years), 6 patients are in New York Heart Association functional class I, and 1 patient is in class II.
Conclusions:
In view of the dismal natural history of untreated pulmonary vein stenosis, prompt surgical relief of the stenosis may be a rewarding undertaking.