De Troyer A, Yernault J C, Englert M
Circulation. 1977 Oct;56(4 Pt 1):647-51. doi: 10.1161/01.cir.56.4.647.
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.
对10例先天性肺动脉瓣狭窄的成年患者的肺功能进行了研究。这些患者的肺部明显小于年龄和身高相当的健康对照者；在任何给定的测量肺总量百分比下，肺弹性回缩压均正常，表明出生后肺实质损伤不是肺部较小的原因。一氧化碳肺弥散量降低，反映了肺血管床的解剖学改变。最后，最大流量-静态回缩曲线显示气道尺寸有固定（而非动态）减小：可塌陷限流段的临界跨壁压（Ptm'）正常，但S段的传导性降低。这些异常很可能反映了肺部发育不足，并提示肺动脉压可能是出生后肺生长的一个重要决定因素。
