Shimazaki Y, Maehara T, Blackstone E H, Kirklin J W, Bargeron L M
Department of Surgery, University of Alabama, Birmingham 35294.
J Thorac Cardiovasc Surg. 1988 Jun;95(6):1048-58.
Cineangiograms of 172 patients with tetralogy of Fallot and pulmonary atresia were qualitatively and quantitatively analyzed retrospectively.
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 less than 0.001). The number of large aortopulmonary collateral arteries correlated inversely with the completeness of arborization of the left and right pulmonary arteries (P less than 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.
对172例法洛四联症合并肺动脉闭锁患者的心血管造影进行回顾性定性和定量分析。
28例（16%）患者先天性左或右肺动脉或两者的中央及未分支肺门部分缺失，因此患有先天性非融合性肺动脉。另外5例（3%）原本融合性肺动脉的患者因端到端手术分流而出现非融合性肺动脉，还有7例（4%）因一侧肺动脉血栓形成而出现非融合性肺动脉。在132例左右肺动脉融合的患者中，70例（132例中的53%）一侧或双侧存在不完全分支（分布），先天性非融合性肺动脉患者中有23例（28例中的82%）也是如此（差异P值=0.005）。后一组中有10例（28例中的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例（3%）。所有组中左右肺动脉近端较小但大小不一（中位麦高恩比例值为1.05），但在肺动脉融合的患者中最大。132例左右肺动脉融合的患者中有79例（60%）存在大的主肺动脉侧支动脉，先天性非融合性肺动脉患者中100%存在大的主肺动脉侧支动脉（P值小于0.001）。大的主肺动脉侧支动脉数量与左右肺动脉分支的完整性呈负相关（P值小于0.0001）。推论：大多数法洛四联症合并肺动脉闭锁患者的肺血管系统存在明显形态学异常，在这方面与法洛四联症合并肺动脉狭窄患者在数量上有所不同。这些异常的各种类型的患病率密切相关。
