Ovseyevitz J, Attie F, Buendia A
Int J Cardiol. 1982;1(3-4):263-71. doi: 10.1016/0167-5273(82)90088-2.
We describe the clinical and surgical findings of a patient with absence of the pulmonary valve leaflets and complete transposition of the great arteries. The main clinical features were a loud, single second heart sound, a loud pansystolic murmur over the entire precordial area, but no diastolic murmurs were heard. The electrocardiogram suggested right ventricular hypertrophy and right bundle branch block. The roentgenographic study showed cardiomegaly with dilatation of both branches of the pulmonary artery. Cardiac catheterization and angiocardiography showed systemic arterial desaturation, discordant ventriculo-arterial connections and absence of the pulmonary valve leaflets. The patient underwent surgical correction by the Rastelli technique with a favorable clinical course.
我们描述了一名肺动脉瓣叶缺如并完全性大动脉转位患者的临床及手术发现。主要临床特征为响亮的单一第二心音、整个心前区响亮的全收缩期杂音，但未闻及舒张期杂音。心电图提示右心室肥厚及右束支传导阻滞。X线检查显示心脏增大伴肺动脉分支扩张。心导管检查及心血管造影显示体循环动脉血氧饱和度降低、心室-动脉连接不一致以及肺动脉瓣叶缺如。该患者接受了Rastelli手术矫正，临床过程良好。
