Soquet Jerome, Liava'a Matthew, Eastaugh Lucas, Konstantinov Igor E, Brink Johann, Brizard Christian P, d'Udekem Yves
Department of Cardiac Surgery, the Royal Children's Hospital, Parkville, Victoria, Australia.
Department of Cardiology, the Royal Children's Hospital, Parkville, Victoria, Australia; Murdoch Childrens Research Institute, Parkville, Victoria, Australia.
Ann Thorac Surg. 2017 May;103(5):1519-1526. doi: 10.1016/j.athoracsur.2016.08.113. Epub 2016 Dec 20.
A strategy of rehabilitation for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) comprises repetitive shunting and patching procedures of the central pulmonary arteries. We wanted to determine the feasibility and limitations of a strategy of rehabilitation.
The outcomes of 37 consecutive patients operated from June 2003 to December 2014 for PA/VSD/MAPCAs were reviewed. The patients were directed to a rehabilitation strategy, except when they presented in heart failure with very large collaterals.
Four patients with very large MAPCAs underwent a one-stage repair with unifocalization of collateral vessels at a median age of 8.6 months. There was no mortality in this group after a median follow-up of 4.6 years. Following a strategy of staged rehabilitation, 33 patients had 2.01 ± 0.9 procedures before repair. Median age at primary shunting was 3.3 weeks (0.4 to 31.9 weeks). Repair rate was 73% (22 patients), at a median age of 1.7 years. Three patients (10%) were left palliated and 3 patients (10%) died. Median follow-up in this group was 4.5 years. Complementary procedures to the rehabilitation strategy consisted in pulmonary artery reconstruction in 25 patients (76%) and MAPCAs ligation in 7 patients (21%). Pulmonary balloon angioplasty was required in 12 patients (36%) and MAPCAs coil occlusion in 8 patients (24%).
A strategy of rehabilitation can be implemented in almost 90% of the cases, with a low mortality rate. Following this strategy, 73% of the patients can be successfully repaired.
肺动脉闭锁、室间隔缺损及大量主-肺动脉侧支血管（PA/VSD/MAPCAs）的康复治疗策略包括对中央肺动脉进行反复分流和修补手术。我们想要确定康复治疗策略的可行性和局限性。
回顾了2003年6月至2014年12月间连续接受手术治疗的37例PA/VSD/MAPCAs患者的治疗结果。除了那些出现心力衰竭且伴有非常粗大侧支血管的患者外，其余患者均采用康复治疗策略。
4例伴有非常粗大MAPCAs的患者在平均年龄8.6个月时接受了一期修复手术，同时对侧支血管进行了单灶化处理。该组患者在中位随访4.6年后无死亡病例。按照分期康复治疗策略，33例患者在修复前平均接受了2.01±0.9次手术。初次分流时的中位年龄为3.3周（0.4至31.9周）。修复率为73%（22例患者），中位年龄为1.7岁。3例患者（10%）接受姑息治疗，3例患者（10%）死亡。该组患者的中位随访时间为4.5年。康复治疗策略的辅助手术包括25例患者（76%）进行肺动脉重建，7例患者（21%）进行MAPCAs结扎。12例患者（36%）需要进行肺动脉球囊血管成形术，8例患者（24%）需要进行MAPCAs弹簧圈封堵术。
在几乎90%的病例中可以实施康复治疗策略，死亡率较低。按照该策略，73%的患者能够成功修复。
