Felmly L Mac, Mainwaring Richard D, Collins R Thomas, Lechich Kirstie, Martin Elisabeth, Ma Michael, Hanley Frank L
Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Lucile Packard Children's Hospital Stanford, Stanford, Calif.
Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Lucile Packard Children's Hospital Stanford, Stanford, Calif.
J Thorac Cardiovasc Surg. 2023 Apr;165(4):1493-1502.e2. doi: 10.1016/j.jtcvs.2022.07.037. Epub 2022 Aug 10.
Peripheral pulmonary artery stenosis (PPAS) is a relatively rare form of congenital heart disease often associated with Williams syndrome, Alagille syndrome, and elastin arteriopathy. This disease is characterized by stenoses at nearly all lobar and segmental ostia and results in systemic-level right ventricular pressures. The current study summarizes our experience with the surgical treatment of PPAS.
This was a retrospective review of 145 patients who underwent surgical repair of PPAS. This included 43 patients with Williams syndrome, 39 with Alagille syndrome, and 21 with elastin arteriopathy. Other diagnoses include tetralogy of Fallot with PPAS (n = 21), truncus arteriosus (n = 5), transposition (n = 3), double-outlet right ventricle (n = 2), arterial tortuosity syndrome (n = 3), and other (n = 8).
The median preoperative right ventricle to aortic peak systolic pressure ratio was 1.01 (range, 0.50-1.60) which was reduced to 0.30 (range, 0.17-0.60) postoperatively. The median number of ostial repairs was 17 (range, 6-34) and median duration of cardiopulmonary bypass was 398 minutes (range, 92-844). There were 3 in-hospital deaths (2.1%). The median duration of follow-up was 26 months (range, 1-220) with 4 late deaths (2.9%). Eighty-two patients have subsequently undergone catheterization and 74 had a pressure ratio <0.50.
The surgical treatment of PPAS resulted in a 70% reduction in right ventricular pressures. At 3 years, freedom from death was 94% and 90% of those evaluated maintained low pressures. These results suggest that the surgical treatment of PPAS is highly effective in most patients.
外周肺动脉狭窄（PPAS）是一种相对罕见的先天性心脏病，常与威廉姆斯综合征、阿拉吉列综合征和弹性蛋白动脉病相关。这种疾病的特征是几乎所有叶和段的开口处均有狭窄，并导致体循环水平的右心室压力。本研究总结了我们对外周肺动脉狭窄手术治疗的经验。
这是一项对145例行外周肺动脉狭窄手术修复患者的回顾性研究。其中包括43例威廉姆斯综合征患者、39例阿拉吉列综合征患者和21例弹性蛋白动脉病患者。其他诊断包括合并外周肺动脉狭窄的法洛四联症（n = 21）、动脉干（n = 5）、大动脉转位（n = 3）、右心室双出口（n = 2）、动脉迂曲综合征（n = 3）以及其他（n = 8）。
术前右心室与主动脉收缩压峰值比值的中位数为1.01（范围0.50 - 1.60），术后降至0.30（范围0.17 - 0.60）。开口修复的中位数为17次（范围6 - 34），体外循环的中位数持续时间为398分钟（范围92 - 844）。有3例住院死亡（2.1%）。随访的中位数持续时间为26个月（范围1 - 220），有4例晚期死亡（2.9%）。82例患者随后接受了心导管检查，74例的压力比值<0.50。
外周肺动脉狭窄的手术治疗使右心室压力降低了70%。3年时，免于死亡的比例为94%，90%接受评估的患者维持低压。这些结果表明，外周肺动脉狭窄的手术治疗对大多数患者非常有效。
