Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Lucile Packard Children's Hospital Stanford, Stanford, Calif.
Division of Cardiology, Stanford University School of Medicine, Lucile Packard Children's Hospital Stanford, Stanford, Calif.
J Thorac Cardiovasc Surg. 2021 Jul;162(1):212-219. doi: 10.1016/j.jtcvs.2020.08.070. Epub 2020 Aug 28.
Patients with Williams and elastin arteriopathy syndromes often have similar cardiac phenotypes characterized by supravalvar aortic stenosis (SVAS), peripheral pulmonary artery stenosis (PPAS), and coronary artery ostial stenosis (CAOS). SVAS and/or PPAS result in a marked increase in myocardial oxygen demand, whereas CAOS may limit myocardial oxygen supply. This combination predisposes to myocardial ischemic events and sudden cardiac arrest. The purpose of this study was to review our experience with the surgical repair of CAOS in patients with Williams and elastin arteriopathy syndromes.
This was a retrospective review of 16 patients with Williams (n = 11) or elastin arteriopathy (n = 5) who underwent surgical repair of CAOS as a concomitant procedure with SVAS and/or PPAS repair. Eleven patients had moderate or severe SVAS, and 10 had PPAS. The median age at surgery was 9 months (range, 3-108 months).
Seven patients had repair of the left main and right coronary ostia, 6 the left main, and 3 the right coronary ostium. Median duration of aortic crossclamp was 56 minutes and cardiopulmonary bypass time was 454 minutes. The median SVAS gradient decreased from 70 to 12 mm Hg and pulmonary artery pressure decreased from 120 to 30 mm Hg. There was 1 operative mortality (6%). The remaining 15 patients were discharged from the hospital and are alive at a median of 17 months' follow-up.
The data demonstrate that patients with Williams and elastin arteriopathy syndromes presenting with SVAS and/or PPAS plus CAOS can undergo successful repair of all hemodynamic issues simultaneously.
患有威廉姆斯和弹性蛋白血管病综合征的患者通常具有相似的心脏表型，其特征为主动脉瓣上狭窄（SVAS）、肺动脉外周狭窄（PPAS）和冠状动脉口狭窄（CAOS）。SVAS 和/或 PPAS 导致心肌耗氧量显著增加，而 CAOS 可能限制心肌供氧。这种组合易导致心肌缺血事件和心源性猝死。本研究的目的是回顾我们在伴有威廉姆斯和弹性蛋白血管病综合征的患者中对 CAOS 的手术修复的经验。
这是一项回顾性研究，共纳入 16 例接受 SVAS 和/或 PPAS 修复术的同时伴有 CAOS 手术修复的威廉姆斯（n=11）或弹性蛋白血管病（n=5）患者。11 例患者存在中度或重度 SVAS，10 例患者存在 PPAS。手术时的中位年龄为 9 个月（范围，3-108 个月）。
7 例患者修复了左主干和右冠状动脉口，6 例修复了左主干，3 例修复了右冠状动脉口。主动脉阻断的中位时间为 56 分钟，体外循环时间为 454 分钟。SVAS 梯度的中位数从 70 毫米汞柱降低至 12 毫米汞柱，肺动脉压从 120 毫米汞柱降低至 30 毫米汞柱。有 1 例患者死亡（6%）。其余 15 例患者出院，在中位 17 个月的随访中存活。
数据表明，伴有 SVAS 和/或 PPAS 加 CAOS 的威廉姆斯和弹性蛋白血管病综合征患者可同时成功修复所有血流动力学问题。
