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Francisco J. Puga
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Ann Thorac Surg 1995;60:144-150
© 1995 The Society of Thoracic Surgeons

Repair of Congenital and Acquired Pulmonary Vein Stenosis

Jacques A. M. van Son, MD, PhD, Gordon K. Danielson, MD, Francisco J. Puga, MD, William D. Edwards, MD, David J. Driscoll, MD

Divisions of Thoracic and Cardiovascular Surgery and Anatomic Pathology and Section of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota

Accepted for publication March 15, 1995.

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 pulmonaryvein 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.




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