The structure of the pulmonary circulation in tetralogy of Fallot with pulmonary atresia. A quantitative cineangiographic study.
The structure of the pulmonary circulation in tetralogy of Fallot with pulmonary atresia. A quantitative cineangiographic study.
复制标题
法洛四联症伴肺闭锁的肺循环结构。
DOI：
10.1016/s0022-5223(19)35674-0
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发表时间：
1988
期刊：
影响因子：
--
通讯作者：
L. Bargeron
中科院分区：
文献类型：
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作者：
Y. Shimazaki;T. Maehara;E. Blackstone;J. Kirklin;L. Bargeron
对172例法洛四联症合并肺动脉闭锁患者的血管造影进行了回顾性分析。调查结果：28例（16%）患者先天性缺乏左肺动脉或右肺动脉或两者的中央和无分支肺门部分，因此先天性肺动脉不融合。另外5例（3%）原本融合的肺动脉由于端对端外科分流而变得不融合，另外7例（4%）由于一条肺动脉血栓形成而变得不融合。在132例左右肺动脉融合的患者中，70例（53%）一个或两个肺动脉分支不完全（分布），23例（82%）先天性肺动脉不融合（P = 0.005）。后一组中有10个（36%）与中央肺动脉连续的肺血管段少于10个（正常为20个），而前一组中有4个（3%）。所有组的左肺动脉和右肺动脉近端较小，但大小不同（中位值为1.05 McGoon比率），但肺动脉汇合的患者最大。79例（60%）左右肺动脉汇合的患者有较大的肺动脉侧支动脉，而先天性肺动脉不汇合的患者100%有较大的肺动脉侧支动脉（P < 0.0001）。肺动脉大支数目与左、右肺动脉分支的完整性呈负相关（P < 0.0001）。推论：大多数四联症和肺动脉闭锁患者的肺血管存在大体形态学异常，在这方面与四联症和肺动脉狭窄患者存在定量差异。各种类型的这些异常的患病率是相当相关的。
Cineangiograms of 172 patients with tetralogy of Fallot and pulmonary atresia were qualitatively and quantitatively analyzed retrospectively. Findings: Twenty-eight (16%) patients had congenital absence of the central and unbranched hilar portions of the left or right pulmonary artery or both, and thus had congenitally nonconfluent pulmonary arteries. Five additional patients (3%) with originally confluent pulmonary arteries had nonconfluent ones because of an end-to-end surgical shunt, as did seven others (4%) because of thrombosis of one pulmonary artery. Among the 132 patients with confluent right and left pulmonary arteries, 70 (53% of 132) had incomplete arborization (distribution) of one or both, as did 23 (82% of 28) of those with congenitally nonconfluent pulmonary arteries (P for difference = 0.005). Ten (36% of 28) of the latter group had fewer than 10 pulmonary vascular segments (normal is 20) in continuity with central pulmonary arteries, compared with four (3%) of the former group. The proximal left and right pulmonary arteries were small but variable in size in all groups (median value 1.05 McGoon ratio), but were largest in the patients with confluent pulmonary arteries. Seventy-nine (60% of 132) patients with confluent left and right pulmonary arteries had large aortopulmonary collateral arteries, as did 100% of those with congenitally nonconfluent pulmonary arteries (P < 0.0001). The number of large aortopulmonary collateral arteries correlated inversely with the completeness of arborization of the left and right pulmonary arteries (P < 0.0001). Inferences: Most patients with tetralogy and pulmonary atresia have gross morphologic abnormalities in the pulmonary vasculature and differ quantitatively in this regard from those with tetralogy and pulmonary stenosis. The prevalences of the various types of these abnormalities are considerably interrelated.