Pulmonary vein stenosis presents as hemoptysis in a 17-month old

Pulmonary vein stenosis (PVS) is a rare and serious condition causing pulmonary hypertension leading to significant morbidity and mortality. PVS can be isolated to a single pulmonary vein, but all four of the pulmonary veins may be simultaneously affected. The primary form of the disease may be related to inadequate embryological connections between the pulmonary venous system, common pulmonary vein, and left atrium. The secondary (or acquired) form of PVS is seen most commonly after surgical repair of anomalous pulmonary venous connection. In these circumstances, the obstruction may occur at the site of anastomosis (i.e. between the left atrium and the pulmonary venous confluence), or it may involve stenosis extending into the individual pulmonary veins. Surgical procedures to widen the narrowed veins and cardiac catheterization to stretch the affected vessel(s) are only short-term solutions because the rate of re-stenosis is high. The timing and severity of symptoms in pediatric patients with PVS depend largely on the number of pulmonary veins involved and the severity of obstruction. Most patients present in the first months to years of life with a history of unexplained respiratory symptoms (e.g. tachypnea, recurrent pneumonias). As the disease progresses, signs and symptoms of pulmonary hypertension and pulmonary edema become increasingly prominent. Hemoptysis is often seen in the older pediatric population. The following case report involves a 17-month old male who presents with sudden onset hemoptysis, hypoxia, and respiratory failure requiring intubation.