Presbitero P, Bull C, Haworth S G, de Leval M R
Br Heart J. 1984 Aug;52(2):178-85. doi: 10.1136/hrt.52.2.178.
Of 12 patients with angiographically absent pulmonary artery, 11 were investigated surgically. The previously occult pulmonary artery was found in 10 patients, in five of whom a vestige of an intrapericardial artery was present and in five the artery was patent only at the hilus, a gap existing between the main pulmonary artery and the hilar vessel, and no artery was found in one. All patients with an intrapericardial artery had right ventricular outflow tract obstruction and a ductus descending vertically from underneath the aortic arch. In those without an intrapericardial vessel the occult artery was on the side opposite the aortic arch, and there was evidence of a ductus coming from the innominate artery on the side of the interruption. The occult pulmonary artery, where identified at operation, was usually joined initially to the systemic circulation. Ultimately, continuity between the hilar and main pulmonary artery may be established surgically. Where no intrapericardial vessel exists, however, a conduit may be required to bridge the gap. It seems advisable to search for the occult artery as early in life as is feasible in the hope that providing a blood supply will ensure development of the vessel and normal lung growth.
在12例血管造影显示肺动脉缺如的患者中，11例接受了手术探查。10例患者发现了先前隐匿的肺动脉，其中5例存在心包内动脉遗迹，5例动脉仅在肺门处通畅，主肺动脉与肺门血管之间存在间隙，1例未发现动脉。所有有心包内动脉的患者均有右心室流出道梗阻，且动脉导管从主动脉弓下方垂直下行。在那些没有心包内血管的患者中，隐匿动脉位于主动脉弓对侧，且有证据表明动脉导管来自中断侧的无名动脉。术中发现的隐匿肺动脉最初通常与体循环相连。最终，可通过手术建立肺门与主肺动脉之间的连续性。然而，在没有心包内血管的情况下，可能需要使用管道来弥合间隙。尽早在可行的情况下寻找隐匿动脉似乎是可取的，希望提供血液供应能确保血管发育和肺正常生长。
