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右肺动脉起源于主动脉。两例病例回顾及报告(作者译)

[Aortic origin of the right pulmonary artery. Review and report of two cases (author's transl)].

作者信息

Vázquez-Pérez J, Pereda Pérez R A, Frontera Izquierdo P

出版信息

An Esp Pediatr. 1976 Nov-Dec;9(6):584-93.

PMID:1030930
Abstract

Two cases of aortic origin of the right pulmonary artery are reported. This is a congenital heart disease with every low incidence. To our knowledge only 62 cases have been previously reported. Patent ductus arteriosus is the usual associated anomaly. The first of our cases is associated with patent ductus arteriosus and pulmonary hypertension with severe pulmonary vascular bed changes; surgical closure was considered unadvisable. Aortic-pulmonic window is associated in the second case; cardiac surgery was carried out and the patient died posoperatively. The previously reported cases are reviewed and are compared with anatomic, physiopathologic, clinical, angiohemodiamic and therapeutic features of our cases. Congestive heart failure and inespecific left-to-right shunt clinical features were present early in life, mostly in the first month. Anatomic and funtional evaluation is only possible by cardiac catheterization and angiocardiography, because pulmonary hypertension appears at a very early stage and once fixed makes surgical treatment unadvisable.

摘要

报告了两例右肺动脉起源于主动脉的病例。这是一种发病率极低的先天性心脏病。据我们所知,此前仅报告过62例。动脉导管未闭是常见的相关异常。我们的第一例病例伴有动脉导管未闭和肺动脉高压,伴有严重的肺血管床改变;手术闭合被认为不可取。第二例病例伴有主肺动脉窗;进行了心脏手术,患者术后死亡。对先前报告的病例进行了回顾,并与我们病例的解剖、生理病理、临床、血管血液动力学和治疗特征进行了比较。充血性心力衰竭和非特异性左向右分流的临床特征在生命早期出现,大多在第一个月。解剖和功能评估只能通过心导管检查和心血管造影进行,因为肺动脉高压在很早阶段就出现,一旦固定,手术治疗就不可取。

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