Mery Carlos M, Molina Kimberly M, Krishnamurthy Rajesh, Fraser Charles D, Justino Henri
Division of Congenital Heart Surgery, Texas Children's Hospital; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Tex.
Section of Pediatric Cardiology, Primary Children's Medical Center, University of Utah, Salt Lake City, Utah.
J Thorac Cardiovasc Surg. 2014 Nov;148(5):2235-2244.e1. doi: 10.1016/j.jtcvs.2013.11.041. Epub 2013 Dec 31.
Ductal origin of a pulmonary artery (DOPA) is commonly misdiagnosed as agenesis of a pulmonary artery (PA), which may result in inadequate treatment. The objective is to describe the results of resuscitation of unilateral DOPA.
This study is a retrospective review of all patients with unilateral DOPA who underwent PA resuscitation at Texas Children's Hospital from 1993 to 2012. Patients with other cardiac or contralateral lung anomalies were excluded.
Ten patients, median age 2 years (range, 3 days to 9 years), with unilateral DOPA were included. Symptoms were present in 6 patients. Cardiac catheterization was performed in all and showed a patent duct or a ductal stump in most patients and a small PA on wedge angiography of the pulmonary veins. Two patients underwent single-stage centralization. The other 8 underwent ductal stenting (n=2) or a systemic-to-PA shunt (n=6) as the first stage before centralization. The 2 patients with ductal stenting developed pulmonary edema. The 2 patients with a cryopreserved vein shunt developed early thrombosis requiring reintervention. Nine patients have undergone centralization. Six patients have required further interventional procedures. There have been no deaths. Symptoms and lung hypoplasia have improved in all patients. Median relative lung perfusion at follow-up was 26% (range, 12%-46%) with significant improvement in the size of the affected PA.
PA resuscitation is effective at restoring flow to the affected lung resulting in improved diameter of the PA, lung growth, and resolution of symptoms. PA resuscitation should be considered in all children with DOPA, including those beyond infancy.
肺动脉导管起源（DOPA）常被误诊为肺动脉缺如（PA），这可能导致治疗不充分。目的是描述单侧DOPA复苏的结果。
本研究是对1993年至2012年在德克萨斯儿童医院接受PA复苏的所有单侧DOPA患者进行的回顾性研究。排除有其他心脏或对侧肺异常的患者。
纳入10例单侧DOPA患者，中位年龄2岁（范围3天至9岁）。6例患者有症状。所有患者均进行了心导管检查，大多数患者显示动脉导管未闭或动脉导管残端，肺静脉楔入造影显示小PA。2例患者接受了一期根治术。其他8例在根治术前作为第一阶段接受了动脉导管支架置入术（n = 2）或体肺分流术（n = 6）。2例接受动脉导管支架置入术的患者发生了肺水肿。2例接受冷冻保存静脉分流术的患者发生早期血栓形成，需要再次干预。9例患者接受了根治术。6例患者需要进一步的介入治疗。无死亡病例。所有患者的症状和肺发育不全均有改善。随访时中位相对肺灌注为26%（范围12%至46%），患侧PA大小有显著改善。
PA复苏可有效恢复患侧肺的血流，导致PA直径增大、肺生长和症状缓解。所有DOPA患儿，包括婴儿期以后的患儿，均应考虑进行PA复苏。
