title: Normalization of pulmonary vascular resistance long after successful primary sutureless repair for congenital pulmonary vein stenosis.
abstract: A 3-month-old infant with perimembranous ventricular septal defect (VSD) and pulmonary hypertension presented at our center. Cardiac catheterization showed a systolic pulmonary-to-systemic arterial pressure ratio of 0.99 and a pulmonary vascular resistance of 9.32 μm(2). Pulmonary angiography showed focal orifice stenosis in the right lower and left lower and upper pulmonary veins, whereas the right upper pulmonary vein was atretic. Sutureless pericardial marsupialization concomitant with VSD patch closure was used to repair the three stenotic veins. Administration of home oxygen therapy and sildenafil citrate was continued after surgical repair. Postoperative catheterization 1.5 years after surgery showed patency of the three repaired veins and normalization of pulmonary vascular resistance.
authors.affiliation: Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, 5-7-1, Fujishirodai, Suita, Osaka, 565-8565, Japan. thoashi@surg1.med.osaka-u.ac.jp
authors.forename: Takaya
authors.initials: T
authors.lastname: Hoashi
authors.forename: Koji
authors.initials: K
authors.lastname: Kagisaki
authors.forename: Heima
authors.initials: H
authors.lastname: Sakaguchi
authors.forename: Hajime
authors.initials: H
authors.lastname: Ichikawa
mesh_terms: D003251:Constriction, Pathologic; D006345:Heart Septal Defects, Ventricular; D006801:Humans; D007223:Infant; D011667:Pulmonary Veins / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery*; D011859:Radiography; D013997:Time Factors; D014655:Vascular Resistance