Shimazaki Y, Tokuan Y, Lio M, Nakano S, Matsuda H, Blackstone E H, Kirklin J W, Shirakura R, Ogawa M, Kawashima Y
First Department of Surgery, Osaka University Medical School, Japan.
J Thorac Cardiovasc Surg. 1990 Sep;100(3):425-40.
Twenty-one patients underwent elective hemodynamic and cineangiographic studies 1 to 9 years after repair of tetralogy of Fallot and pulmonary atresia. They were a subset of 41 patients who underwent complete repair between Jan. 1, 1973, and 1988. The mean and systolic pulmonary arterial pressure, as well as the pulmonary vasculature resistance, were abnormally high in 60% of the patients. Thirty-three percent of the patients were found to have pulmonary arterial segments that were not connected to the central and unbranched hilar portions of the right and left pulmonary arteries. The mean pulmonary artery pressure and the pulmonary vasculature resistance were correlated (inversely) with the number of centrally connected pulmonary arterial segments. The pulmonary vasculature resistance per pulmonary arterial segment was also inversely correlated with the number of centrally connected pulmonary arterial segments. Inferences: The finding that abnormalities in the pulmonary vasculature of centrally connected pulmonary arterial segments become more severe as the number of centrally connected pulmonary arterial segments decreased has implications as to hemodynamic effectiveness of unifocalization operations. Since others have found these abnormalities in the pulmonary vasculature to be age related, the findings support beginning the appropriate surgical treatment of patients with tetralogy of Fallot and pulmonary atresia very early in life.
21例患者在法洛四联症和肺动脉闭锁修复术后1至9年接受了选择性血流动力学和血管造影研究。他们是1973年1月1日至1988年间接受完全修复的41例患者的一个子集。60%的患者平均肺动脉压和收缩期肺动脉压以及肺血管阻力异常升高。33%的患者发现肺动脉段未与左右肺动脉的中央和无分支肺门部分相连。平均肺动脉压和肺血管阻力与中央相连肺动脉段的数量（呈负）相关。每个肺动脉段的肺血管阻力也与中央相连肺动脉段的数量呈负相关。推论：随着中央相连肺动脉段数量的减少，中央相连肺动脉段的肺血管异常变得更加严重，这一发现对单灶化手术的血流动力学效果具有重要意义。由于其他人发现这些肺血管异常与年龄有关，这些发现支持在生命早期就对法洛四联症和肺动脉闭锁患者进行适当的手术治疗。
