Kucera V, Fiser B, Tůma S, Hucin B
Thorac Cardiovasc Surg. 1982 Jun;30(3):152-8.
Nineteen cases of unilateral absence of the pulmonary artery (UAPA) were found in a group of 2,960 patients investigated consecutively by cardiac catheterization, angiocardiography, and pulmonary perfusion scintigraphy. The incidence of UAPA was 0,6%. In 8 cases (40%) UAPA was found in isolation, and in 11 cases (60%) it was combined with other congenital heart defects (CHD). In 30% of our patients the diagnosis of UAPA was suggested by clinical symptoms and plain chest X-ray. Pulmonary perfusion scan was made in 2,600 children before catheterization. Though it can provide strong evidence of the diagnosis, this should be confirmed by cardiac catheterization and selective angiography. Eight patients were operated upon. Systemic-pulmonary shunt operations were performed in 6, and ligation of PDA in 2 patients. In one of the latter severe hemoptysis occurred following ligation of the PDA. The pathophysiological and diagnostic aspects of UAPA are discussed.
在一组2960例经心导管检查、心血管造影和肺灌注闪烁扫描连续检查的患者中，发现19例单侧肺动脉缺如（UAPA）。UAPA的发生率为0.6%。8例（40%）UAPA为孤立性，11例（60%）合并其他先天性心脏病（CHD）。30%的患者UAPA诊断由临床症状和平片胸片提示。2600例儿童在导管检查前行肺灌注扫描。虽然它能为诊断提供有力证据，但仍需通过心导管检查和选择性血管造影来确诊。8例患者接受了手术。6例行体肺分流术，2例行动脉导管未闭结扎术。后者中有1例在结扎动脉导管后发生严重咯血。本文讨论了UAPA的病理生理和诊断方面。
