title: [Right branch pulmonary artery stenosis with supravalvar aortic stenosis as a complication of Lecompte maneuver for tetralogy of Fallot associated with absent pulmonary valve].
abstract: The patient was diagnosed with tetralogy of Fallot associated with absent pulmonary valve syndrome and a low birth weight of 1,912 g. He suffered from respiratory distress on day 14 and received non-invasive positive pressure ventilation. At 5 months of age and 4.1 kg, he underwent intracardiac repair including right ventricular outflow repair with a monocusp patch, patch closure of the ventricular septum defect and right pulmonary transposition to the anterior of the ascending aorta following the Lecompte maneuver for airway decompression. He was subsequently discharged to home and exhibited an uneventful clinical course with non-invasive positive pressure ventilation for 5 months postoperatively. However, right pulmonary artery and supra-aortic stenosis was noted 2 years after the operation. Computed tomography (CT) and angiography showed ascending aorta strangulation by the right pulmonary artery with right ventricular outflow regurgitation. Right pulmonary artery reconstruction using polytetrafluoroethylene graft interposition and repeat right ventricular outflow repair with bicuspid hand-sewn valves was therefore performed;the postoperative course was uneventful. Pre- and post-operative management using non-invasive positive pressure ventilation and airway decompression with pulmonary artery translocation is a useful strategy in patients exhibiting symptomatic tetralogy of Fallot associated with absent pulmonary valve syndrome in the neonatal period.
authors.affiliation: Department of Second Surgery, University of Yamanashi, Chuo, Japan.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Honda
authors.forename: Shoji
authors.initials: S
authors.lastname: Suzuki
authors.forename: Shigeaki
authors.initials: S
authors.lastname: Kaga
authors.forename: Yukiyo
authors.initials: Y
authors.lastname: Yoshida
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Kimura
authors.forename: Kentaro
authors.initials: K
authors.lastname: Kamiya
authors.forename: Kenji
authors.initials: K
authors.lastname: Sakakibara
authors.forename: Masatake
authors.initials: M
authors.lastname: Katsu
mesh_terms: D001020:Aortic Stenosis, Subvalvular / Q000209:etiology / Q000601:surgery*; D006801:Humans; D021621:Imaging, Three-Dimensional; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery / Q000601:surgery*; D011664:Pulmonary Valve / Q000002:abnormalities / Q000601:surgery*; D011666:Pulmonary Valve Stenosis / Q000601:surgery*; D019651:Plastic Surgery Procedures; D012086:Reoperation; D014057:Tomography, X-Ray Computed