Fouron J C, Favreau-Ethier M, Marion P, Davignon A
Can Med Assoc J. 1967 Apr 15;96(15):1084-94.
Sixteen cases of peripheral pulmonary stenosis have been studied clinically and by cardiac catheterization. Diagnosis has been proved in all cases by manometric measurements and/or cineangiocardiography. All patients except two were below 2 years of age. Ten cases were of type I, i.e. the stenosis was localized to the pulmonary trunk or its main branches. Six patients were of type III, i.e. they had diffuse stenosis of the pulmonary arterial tree. The physical findings, which in many cases are typical and include the presence of a systolic murmur over both lung fields, should alert the physician to the diagnosis at the bedside. At cardiac catheterization the configuration of the pressure tracing in the main pulmonary artery is typical, showing an abrupt rise and fall of the systolic wave followed by a low situated dicrotic notch. There is no doubt that in the past many cases of peripheral pulmonary stenosis have been wrongly diagnosed as “essential pulmonary hypertension”.
我们对16例周围性肺动脉狭窄患者进行了临床及心导管检查研究。所有病例均通过压力测量和/或心血管造影证实诊断。除2例患者外，所有患者年龄均在2岁以下。10例为I型，即狭窄局限于肺动脉干或其主要分支。6例为III型，即肺动脉树弥漫性狭窄。体格检查结果在许多情况下具有典型性，包括双肺野出现收缩期杂音，这应提醒医生在床边即可做出诊断。在心导管检查中，主肺动脉压力曲线形态典型，收缩波突然上升和下降，随后出现低位的重搏波切迹。毫无疑问，过去许多周围性肺动脉狭窄病例被误诊为“原发性肺动脉高压”。
