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特发性肺动脉扩张是指肺动脉主干的扩张,较少见,已证实为一种无心内分流的心血管畸形。由Wessler和Jaches于1923年首先描述。1933年Oppenheimer提出“特发性扩张”的命名。Abbott报告发病率为0.6％。我院1979～1985年间发现4例,均初诊为房缺,3例经手术探查,1例经右心导管、X线及随访5年证实为本病。本病国内尚未见有报告,有关本病的详细描述不多。
Idiopathic pulmonary artery dilatation refers to the expansion of the pulmonary artery trunk, less common, has proved to be a cardiac distraction without heart and heart. Described first by Wessler and Jaches in 1923. 1933 Oppenheimer proposed “idiopathic expansion” of the name. Abbott reported a prevalence of 0.6%. Four cases were found in our hospital from 1979 to 1985, all were newly diagnosed as atrial septal defect, 3 cases were surgically investigated, 1 case was confirmed by right heart catheterization, X-ray and follow-up for 5 years. The disease has not been reported in the country, the detailed description of the disease is limited.