d'Udekem Yves, Alphonso Nelson, Nørgaard Martin A, Cochrane Andrew D, Grigg Leeanne E, Wilkinson James L, Brizard Christian P
Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.
J Thorac Cardiovasc Surg. 2005 Dec;130(6):1496-502. doi: 10.1016/j.jtcvs.2005.07.034.
We sought to evaluate the contribution of unifocalization procedures in the management of patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.
From 1975 through 1995, 82 consecutive patients were entered in a multistage approach and had 189 sternotomies and thoracotomies to perform 119 shunts, 130 major aortopulmonary collateral artery transplantations, and 76 major aortopulmonary collateral artery ligations. The serial angiographies and the follow-up of these patients were reviewed.
The concurrent follow-up rate was 80%. The hospital mortality of the preliminary procedures was 4% (7/189). Fifty-three (65%) patients had a complete repair. The hospital mortality of the repair was 8% (4/53), and 9 late deaths occurred after repair, all of which were cardiac related. The overall survival of all patients to the age of 30 years was 58% +/- 7%. Survival 12 years after complete repair was 51% +/- 14%. On angiography, central shunts promoted growth of central pulmonary arteries in all cases (29 patients). Sixty unifocalized major aortopulmonary collateral arteries were identified in 31 patients. After a mean of 3.2 +/- 4 years, 26 thrombosed, and 12 presented with a stenosis of greater than 50%. Serial measurements of 29 major aortopulmonary collateral arteries showed no signs of growth (P = .25).
Long-term survival into adulthood of patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries has been achieved with a multistage approach. However, late survival depends exclusively on the growth of the native pulmonary circulation. The few unifocalized major aortopulmonary collateral arteries that did not thrombose failed to grow.
我们试图评估单源化手术在肺动脉闭锁、室间隔缺损及主要体肺侧支动脉患者治疗中的作用。
1975年至1995年，82例连续患者采用多阶段治疗方法，接受了189次胸骨切开术和开胸手术，以进行119次分流术、130次主要体肺侧支动脉移植术和76次主要体肺侧支动脉结扎术。对这些患者的系列血管造影及随访情况进行了回顾。
同期随访率为80%。初期手术的院内死亡率为4%（7/189）。53例（65%）患者接受了完全修复。修复手术的院内死亡率为8%（4/53），修复后发生9例晚期死亡，均与心脏相关。所有患者至30岁时的总体生存率为58%±7%。完全修复后12年的生存率为51%±14%。血管造影显示，所有病例（29例患者）中的中心分流均促进了中心肺动脉的生长。31例患者中发现60条单源化的主要体肺侧支动脉。平均3.2±4年后，26条发生血栓形成，12条出现大于50%的狭窄。对29条主要体肺侧支动脉的系列测量未显示生长迹象（P = 0.25）。
采用多阶段治疗方法已使肺动脉闭锁、室间隔缺损及主要体肺侧支动脉患者存活至成年期。然而，晚期存活完全取决于自身肺循环的生长。少数未发生血栓形成的单源化主要体肺侧支动脉未能生长。
