LUNG HYPOPLASIA IN CONGENITAL PULMONARY VALVE STENOSIS
LUNG HYPOPLASIA IN CONGENITAL PULMONARY VALVE STENOSIS
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DOI：
10.1161/01.cir.56.4.647
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发表时间：
1977-01-01
期刊：
影响因子：
37.8
通讯作者：
ENGLERT, M
中科院分区：
文献类型：
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作者：
DETROYER, A;YERNAULT, JC;ENGLERT, M
对十名成人先天性肺瓣膜狭窄患者的肺功能进行了调查。患者的肺部明显比同等年龄和身高的健康对照者要小；在测量的总肺容量的任何给定百分比下，肺弹性反冲压力都是正常的，这表明出生后实质损伤不是小肺的原因。肺一氧化碳弥散能力降低，反映了肺血管床的解剖学改变。最后，最大流量-静态反冲曲线显示气道尺寸固定（非动态）减小：可折叠限流段（Ptm'）中的临界跨壁压正常，但S段的电导降低。这些异常很可能反映了肺部发育不足，并表明肺血压可能是产后肺部生长的重要决定因素。
The pulmonary function of ten adult patients with congenital pulmonary valvular stenosis was investigated. The patients clearly showed smaller lungs than healthy control subjects of equivalent age and height; lung elastic recoil pressure was normal at any given percentage of measured total lung capacity, indicating that postnatal parenchymal damage is not the cause of the small lungs. The lung diffusing capacity for carbon monoxide was reduced, reflecting the anatomical alterations of the pulmonary vascular bed. Finally, the maximal flow-static recoil curves showed a fixed (not dynamic) reduction of airway dimensions: the critical transmural pressure in the collapsible flow-limiting segment (Ptm') was normal, but the conductance of the S segment was lowered. These abnormalities most likely reflect inadequate development of the lung and suggest that pulmonary blood pressure may be an important determinant of lung growth in the postnatal period.