title: [Anesthetic management for an infant with tetralogy of fallot with absent pulmonary valve].
abstract: Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is a rare disease, and characterized by massive dilatation of pulmonary artery. Bronchial compression by the aneurysm-like dilated pulmonary artery sometimes results in severe respiratory compromise. Surgery including closure of VSD and right ventricular outflow construction is often needed in infancy. But, even after the surgical correction, patients often suffer from right ventricular failure due to right ventriculotomy and poor development of pulmonary capillaries. Therefore, preventing pulmonary hypertension is important during the perioperative period. We report an infant with TOF/APV. This 3 month-old boy developed respiratory distress suddenly. After the induction of anesthesia with sevoflurane, fentanyl, and vecuronium, continuous infusion of alprostadil and olprinone was started. Cardiopulmonary bypass (CPB) was instituted smoothly. VSD was closed and right ventricular outflow tract was reconstructed. Dilated pulmonary arteries were resected and plicated. Weaning from CPB was easy and postoperative course was uneventful with mild pulmonary hypertension. In the cases predisposed to right ventricular failure like TOF/APV, olprinone may be useful.
authors.affiliation: Department of Anesthesia, Kyoto University Hospital, Kyoto 606-8507.
authors.forename: Tomoharu
authors.initials: T
authors.lastname: Tanaka
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yamashita
authors.forename: Hisanari
authors.initials: H
authors.lastname: Ishii
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Fukuda
mesh_terms: D000527:Alprostadil / Q000008:administration & dosage; D000758:Anesthesia / Q000379:methods*; D006348:Cardiac Surgical Procedures / Q000379:methods; D002315:Cardiopulmonary Bypass; D002316:Cardiotonic Agents / Q000008:administration & dosage; D005283:Fentanyl; D006801:Humans; D006976:Hypertension, Pulmonary / Q000517:prevention & control; D007093:Imidazoles / Q000008:administration & dosage; D007223:Infant; D008297:Male; D008738:Methyl Ethers; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000601:surgery*; D011728:Pyridones / Q000008:administration & dosage; D000077149:Sevoflurane; D013771:Tetralogy of Fallot / Q000150:complications / Q000601:surgery*; D014673:Vecuronium Bromide
chemical_list: D002316:Cardiotonic Agents; D007093:Imidazoles; D008738:Methyl Ethers; D011728:Pyridones; D000077149:Sevoflurane; C059498:olprinone; D014673:Vecuronium Bromide; D000527:Alprostadil; D005283:Fentanyl