Pulmonary veins stenosis
Overview
Pulmonary vein stenosis is a condition in which there is an obstruction (blockage) in the blood vessels that bring oxygen-rich blood from the lungs back to the heart. Stenosis occurs due to an abnormal process that thickens and narrows the walls in the veins. Pulmonary vein stenosis frequently progresses. As a result, total obstruction or partial loss of flow to a vessel or vessels may occur. This condition may occur as a complicating feature of complex congenital heart disease, but it may also occur in infants with otherwise normal hearts. When pulmonary vein stenosis occurs in children without congenital heart defects, it occurs in early infancy and usually progresses very rapidly. Infants with this disease may seem well for weeks before they develop difficulty breathing and low oxygen levels. They may become quite ill quickly. The effects of the disease vary in children with co-existing congenital heart defects.
Symptoms
* shortness of breath, rapid breathing * fatigue * poor feeding * paleness or cyanosis * fast heartbeat
Causes
Progressive pulmonary vein stenosis is thought to result from the uncontrolled growth of connective tissue cells, which causes the blockage in the pulmonary veins. The cells are thought to be myofibroblast-like. These cells are normally in our bodies and function in wound healing. The cells are also known to grow abnormally in several different disease processes in both children and adults. It appears that this cell type plays a role in the development of pulmonary vein stenosis. Current treatment focuses upon removal of the obstruction and prevention of progression and recurrence of this abnormal cell growth.
Prognosis
Prognosis of Pulmonary veins stenosis: death usually occurs from 5 months to 10 years
Treatment
The goals of treatment are to control thrombus development, prevent complications, relieve pain, and prevent recurrence of the disorder. Symptomatic measures include bed rest, with elevation of the affected arm or leg; warm, moist soaks to the affected area; and analgesics. After the acute episode of DVT subsides, the patient may resume activity while wearing antiembolism stockings that were applied before he got out of bed. Treatment also includes anticoagulants (initially, heparin; later, warfarin) to prolong clotting time. Low-molecular-weight (LMW) heparin has been shown to be effective in treating DVT. Although LMW heparin is more expensive, it doesn’t require monitoring for its anticoagulant effect. Full anticoagulant doses must be discontinued during any operative period because of the risk of hemorrhage. After some types of surgery, especially major abdominal or pelvic operations, prophylactic doses of anticoagulants may reduce the risk of DVT and pulmonary embolism. For lysis of acute, extensive DVT, treatment should include streptokinase. Rarely, DVT may cause complete venous occlusion, which necessitates venous interruption through simple ligation to vein plication, or clipping. Embolectomy and insertion of a vena caval umbrella or filter may also be done. Therapy for severe superficial thrombophlebitis may include an anti-inflammatory drug such as indomethacin, antiembolism stockings, warm soaks, and elevation of the leg.