主动脉瓣上狭窄的外科治疗

Surgical Management of Supravalvular Aortic Stenosis

报告１３例主动脉瓣上狭窄（ｓｕｐｒａｖａｌｖｕｌａｒａｏｒｔｉｃｓｔｅｎｏｓｉｓ，ＳＶＡＳ）病例的治疗。其中局限型１１例，弥漫型２例。合并的心血管畸形包括：主动脉瓣狭窄５例，主动脉瓣反流２例，主动脉瓣环发育不全２例，冠状动脉畸形６例，动脉导管未闭１例，多发肺动脉狭窄１例，肺动脉瓣上隔膜１例，头臂动脉畸形５例。１３例病人中，９例行狭窄解除术，１例用同种升主动脉行心尖至胸主动脉架桥术，１例单纯动脉导管结扎术，２例未手术。结果手术早期死亡２例，晚期死亡１例。作者认为ＳＶＡＳ手术治疗的要点为：（１）充分解除主动脉瓣上狭窄；（２）避免一切影响冠状动脉灌注的因素；（２）妥善处理并存的主动脉瓣病变。

Aim:To review the surgical experience of supravalvular aortic stenosis.Clinical material and method:Thirteen patients with supravalvular aortic stenosis (SVAS) were treated in our hospital.Eleven patients had localized SVAS and 2 had diffused SVAS.The associated cardiovascular anomalies included aoritc valve stenosis in 5,aortic regurgitation in 2,hypoplasia of aortic annulus in 2,abnormalities of coronary artery in 6,patent duct arteriosus in 1,membranous supravalvular pulmonary stenosis in 1,multiple stenoses of pulmonary artery in 1 and abnormalities of the brachiocephalic arteries in 5.Eleven patients underwent surgery including aortoplasty in 9,apicoaortic conduit of aortic homograft in 1 and ligation of patent ductus arteriosus alone in 1.Two patients did not receive operation.Result:There were 2 operative deaths with a hospital mortality of 18%.Late death occurred in one patient 16 months after the operation.Conclusion:For localized SVAS,patch aortoplasty is the choice of the treatment.Coronary perfusion during operation is closely related to surgical outcome.The associated aortic valvular abnormalities should be carefully corrected.