Three Cases of Congenital Pulmonary Vein Stenosis

Abstract

We report three cases of congenital pulmonary vein stenosis （CPVS）. All 3 cases had some cardiac anomalies, such as atrial septal defect, persistent left superior vena cava, patent ductus arteriosus, and patent foramen ovale. Patients experienced tachypnea and retractive breath in early infancy. We could easily diagnose CPVS used by Doppler echocardiography and contrast three-dimensional CT scan. One of the patients underwent sutureless repair for CPVS, but he had re-stenotic changes at the operative site. Pathological examination of two patients showed myofibroblast proliferation in the pulmonary veins. Imatinib administration failed to regress the myofibroblast proliferation in a child with postoperative pulmonary vein stenosis. All three patients died of progressive pulmonary venous stenosis.
CPVS is a progressive disease, and the prognosis for infants with bilateral CPVS is very poor. Postnatal progression of CPVS may be caused by abnormal myofibroblast proliferation in the pulmonary veins.