O'Toole S J, Karamanoukian H L, Glick P L
Buffalo Institute of Fetal Therapy, Childrens Hospital of Buffalo, NY 14222, USA.
J Perinatol. 1996 Mar-Apr;16(2 Pt 2 Su):S34-9.
The cardiopulmonary consequences of CDH can be devastating, and despite intensive research the pathophysiologic makeup of this condition is not fully understood. It seems likely that its overall prognosis is related to the size of the pulmonary vascular bed and its capacity to accept the cardiac output at birth. The ability of the lungs to perform this task is impaired by a surfactant deficiency and by pulmonary vessels that respond excessively to normal stimuli of vasoconstriction. Understanding the mechanisms through which these pathophysiological abnormalities are occurring should provide us with therapeutic insights into how we may recruit all the available alveoli in these lungs, prevent ventilation-induced lung injury, optimize pulmonary blood flow, and maximize cardiac output without causing right or left heart compromise. Failure to achieve all or any of these goals can feed back and exacerbate the underlying pathophysiological condition.
先天性膈疝的心肺后果可能是毁灭性的，尽管进行了深入研究，但这种病症的病理生理构成仍未完全明了。其总体预后似乎与肺血管床的大小及其在出生时接受心输出量的能力有关。肺执行这项任务的能力因表面活性剂缺乏以及肺血管对正常血管收缩刺激反应过度而受损。了解这些病理生理异常发生的机制，应该能为我们提供治疗思路，即如何在这些肺中募集所有可用的肺泡、预防通气诱导的肺损伤、优化肺血流量以及在不导致右心或左心功能受损的情况下使心输出量最大化。未能实现所有或任何这些目标可能会产生反馈并加剧潜在的病理生理状况。
