Feins Eric N, Ireland Christina, Gauvreau Kimberlee, Chávez Mariana, Callahan Ryan, Jenkins Kathy J, Baird Christopher W
Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Mass.
Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, Mass.
J Thorac Cardiovasc Surg. 2022 Jun;163(6):2198-2207.e3. doi: 10.1016/j.jtcvs.2021.10.022. Epub 2021 Oct 23.
The study objective was to evaluate outcomes of pulmonary vein stenosis repair in a large single-center cohort.
Clinical data from a pulmonary vein stenosis registry were retrospectively reviewed identifying patients who underwent pulmonary vein stenosis repair. The primary/index operation was defined as the patient's first pulmonary vein stenosis operation during the study period.
Between January 2007 and August 2019, 174 patients underwent pulmonary vein stenosis repair. Bilateral pulmonary vein stenosis occurred in 111 patients (64%); 71 patients (41%) had 4-vessel disease. Fifty-nine patients (34%) had primary pulmonary vein stenosis. Median age was 9 months (interquartile range, 5-27) and weight was 6.5 kg (4.7-10.2). Surgical techniques evolved and included ostial resection, unroofing, reimplantation, sutureless, modified sutureless, and a newer anatomically focused approach of pulmonary vein stenosis resection with lateralization or patch enlargement of the pulmonary vein-left atrium connection. Twenty-three patients (13%) required reoperation. Cumulative 2-year incidence of postoperative transcatheter intervention (balloon dilation ± stenting) was 64%. One-, 2-, and 5-year survivals were 71.2%, 66.8%, and 60.6%, respectively. There was no association between surgery type and reoperation rate (hazard ratio, 2.38, P = .25) or transcatheter intervention (hazard ratio, 0.97, P = .95). The anatomically focused repair was associated with decreased mortality on univariate (hazard ratio, 0.38, P = .042) and multivariable analyses (hazard ratio, 0.19, P = .014). Antiproliferative chemotherapy was also associated with decreased mortality (hazard ratio, 0.47, P = .026).
This large single-center surgical pulmonary vein stenosis experience demonstrates encouraging midterm results. A new anatomically focused repair strategy aims to alleviate pulmonary vein angulation to minimize turbulence and shows promising early outcomes. Continued follow-up is required to understand longer-term outcomes for this surgical approach.
本研究旨在评估在一个大型单中心队列中肺静脉狭窄修复的结果。
对肺静脉狭窄登记处的临床数据进行回顾性分析，确定接受肺静脉狭窄修复的患者。初次/索引手术定义为患者在研究期间的首次肺静脉狭窄手术。
2007年1月至2019年8月期间，174例患者接受了肺静脉狭窄修复。111例患者（64%）发生双侧肺静脉狭窄；71例患者（41%）有四支血管病变。59例患者（34%）有原发性肺静脉狭窄。中位年龄为9个月（四分位间距，5 - 27），体重为6.5千克（4.7 - 10.2）。手术技术不断发展，包括开口切除、去顶、再植入、无缝合、改良无缝合，以及一种更新的以解剖为重点的肺静脉狭窄切除方法，即肺静脉 - 左心房连接侧方化或补片扩大。23例患者（13%）需要再次手术。术后经导管介入治疗（球囊扩张±支架置入）的累积2年发生率为64%。1年、2年和5年生存率分别为71.2%、66.8%和60.6%。手术类型与再次手术率（风险比，2.38，P = 0.25）或经导管介入治疗（风险比，0.97，P = 0.95）之间无关联。在单因素分析（风险比，0.38，P = 0.042）和多因素分析（风险比，0.19，P = 0.014）中，以解剖为重点的修复与死亡率降低相关。抗增殖化疗也与死亡率降低相关（风险比，0.47，P = 0.026）。
这一大型单中心手术治疗肺静脉狭窄的经验显示出令人鼓舞的中期结果。一种新的以解剖为重点的修复策略旨在减轻肺静脉成角，以尽量减少湍流，并显示出有希望的早期结果。需要持续随访以了解这种手术方法的长期结果。
