Muster A J, Paul M H, Van Grondelle A, Conway J J
Am J Cardiol. 1976 Sep;38(3):352-61. doi: 10.1016/0002-9149(76)90178-8.
Pulmonary angiograms, radionuclide lung images and chest roentgenograms were evaluated regarding the incidence, magnitude and natural evolution of maldistribution of the pulmonary blood flow between the lungs in 63 patients with dextrotransposition of the great arteries. Approximately half of these patients had some degree of greater perfusion of the right relative to the left lung. A significant correlation was demonstrated between the incidence of this maldistribution of blood flow and the angulation between the main and the right pulmonary arteries. For any given angulation between these vessels, additional pulmonary stenosis increased the incidence of disparity in perfusion. Our observations suggest the following developmental mechanisms: The maldistribution in flow results from the abnormal rightward inclination of the main pulmonary artery in the transposition malformation which straightens the flow axis from the main to the right pulmonary artery. Under these circumstances the momentum of the blood in the main pulmonary artery carries the blood preferentially into the right pulmonary artery. This momentum is increased when there is stenosis of the left ventricular outflow tract. Consequent differences in the mechanical properties of the two pulmonary vascular beds can increase this maldistribution. The disparity in perfusion between the lungs is not present in newborns with d-transposition, appears to be progressive in severity and in time may result in almost complete cessation of effective perfusion of the left lung. The effect of the Mustard operation on this abnormality of flow is discussed.
对63例大动脉右转位患者的肺血管造影、放射性核素肺显像和胸部X线片进行了评估，以了解两肺间肺血流分布不均的发生率、程度和自然演变情况。这些患者中约有一半存在右肺灌注相对于左肺某种程度的增加。血流分布不均的发生率与主肺动脉和右肺动脉之间的夹角之间存在显著相关性。对于这些血管之间的任何给定夹角，额外的肺动脉狭窄会增加灌注差异的发生率。我们的观察结果提示了以下发育机制：血流分布不均是由于转位畸形中主肺动脉异常向右倾斜，使从主肺动脉到右肺动脉的血流轴变直所致。在这种情况下，主肺动脉内血液的动量优先将血液带入右肺动脉。当左心室流出道狭窄时，这种动量会增加。两肺血管床力学特性的差异会加剧这种血流分布不均。大动脉右转位新生儿不存在两肺灌注差异，其严重程度似乎呈进行性发展，最终可能导致左肺几乎完全停止有效灌注。文中还讨论了Mustard手术对这种血流异常的影响。
