An Infant with Bilateral Isolated Pulmonary Vein Stenosis

Abstract

Congenital pulmonary vein stenosis is rare condition, and an isolated form of this condition characterized by no intra-cardiac anomalies is extremely rare. We report here a four-month-old male infant with bilateral pulmonary vein stenosis, who finally received a percutaneous stent implantation due to recurrent pulmonary vein stenosis after repeated surgical repair. Congenital isolated pulmonary vein stenosis is frequently lethal because of worsening pulmonary hypertension and cardiac failure. Surgical or transcatheter intervention is not a definite therapy due to recurrent stenosis. Although lung or cardiopulmonary transplantation might be recommended, there are limited cases in Japan. At present, percutaneous pulmonary vein stent implantation may be considered an important therapy as a bridge to cardiac transplantation.