Shimazaki Y, Nakano S, Iio M, Ikawa S, Tokuan Y, Matsuda H, Kawashima Y, Morimoto S, Arisawa J, Sano T
First Department of Surgery, Osaka University Medical School.
Kokyu To Junkan. 1991 Aug;39(8):801-5.
Twenty-two patients with tetralogy of Fallot and pulmonary atresia underwent cardiac catheterization and angiocardiography late after repair. In ten patients, the disease was not associated with the major aortopulmonary collateral artery (MAPCA), but in 12 patients, the disease was associated with it. Three patients died after restudy, two because of pulmonary hypertension and one because of pneumonia. Pulmonary artery mean pressure was 15-92 (28 +/- 21) mmHg, being higher in 75% of the patients with MAPCA than the normal range. The number of pulmonary artery subsegments connected to the central pulmonary arteries was determined from pulmonary arteriography, being 22-42 (38 +/- 6). Pulmonary artery mean pressure and pulmonary vasculature resistance (PVR), 0.8-35.2 (10 +/- 9), inversely correlated with the number of the centrally connected pulmonary artery subsegments (r = -0.81, p less than 0.001 vs PAP, r = -0.85, p less than 0.001 vs PVR). PVR per subsegment also inversely correlated with them (r = -0.81, p less than 0.001). These findings suggest that pulmonary vasculature resistance is abnormally high in this disease, and increase as the number of the centrally connected pulmonary artery subsegments decrease.
22例法洛四联症合并肺动脉闭锁患者在修复术后晚期接受了心导管检查和心血管造影。10例患者的疾病与主要体肺侧支动脉（MAPCA）无关，但12例患者的疾病与之相关。3例患者在复查后死亡，2例死于肺动脉高压，1例死于肺炎。肺动脉平均压为15 - 92（28±21）mmHg，75%合并MAPCA的患者高于正常范围。通过肺动脉造影确定与中央肺动脉相连的肺动脉亚段数量为22 - 42（38±6）。肺动脉平均压和肺血管阻力（PVR）为0.8 - 35.2（10±9），与中央相连的肺动脉亚段数量呈负相关（与肺动脉平均压相比，r = -0.81，p < 0.001；与肺血管阻力相比，r = -0.85，p < 0.001）。每个亚段的肺血管阻力也与它们呈负相关（r = -0.81，p < 0.001）。这些发现表明，该疾病中肺血管阻力异常高，且随着与中央相连的肺动脉亚段数量减少而增加。
