title: [A case report of definitive repair for pulmonary atresia and intact ventricular septum associated with aortic valve regurgitation].
abstract: A successful definitive repair for a 10-year-old girl with pulmonary atresia and intact ventricular septum (PA.IVS) associated with aortic valve regurgitation is described. The Fontan type repair was not indicated in this case because of the left ventricular dysfunction due to aortic valve regurgitation and inadequate size of the pulmonary artery. Therefore, right ventricular outflow tract reconstruction, Glen shunt and aortic valve replacement were performed despite severe hypoplastic right ventricle (RVEDVI; 33% of normal) and restrictive tricuspid valve (TVD; 48% of normal). Postoperatively, good result was obtained. There is general agreement that biventricular repair could be safely performed using Glenn shunt, when RVEDVI is above 40% of normal and TVD is above 50% of normal in a patient with PA.IVS. Moreover recently including our case, several successful repairs for PA.IVS with more hypoplastic right ventricle and tricuspid valve have been reported. So it is suggested that the right ventricular outflow tract reconstruction and Glenn shunt can be reliably applied for PA.IVS with more hypoplastic right ventricle and more restrictive tricuspid valve. To our knowledge, this is the first successful report of definitive repair (right ventricular outflow tract reconstruction, Glenn shunt and AVR) for PA.IVS associated with AR.
authors: Department of Cardiovascular Surgery, Kanto Teishin Hospital, Tokyo, Japan.
authors: M
authors: M
authors: Nagashima
authors: Y
authors: Y
authors: Takeuchi
authors: A
authors: A
authors: Gomi
authors: Y
authors: Y
authors: Okamura
authors: H
authors: H
authors: Mori
mesh_terms: D001021:Aortic Valve / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000150:complications / Q000601:surgery*; D002648:Child; D005260:Female; D006346:Heart Septum; D006350:Heart Valve Prosthesis; D006801:Humans; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000601:surgery