Management outcomes of primary pulmonary vein stenosis
Management outcomes of primary pulmonary vein stenosis
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DOI：
10.1016/j.jtcvs.2019.08.105
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发表时间：
2020-03-01
影响因子：
6
通讯作者：
Alsoufi, Bahaaldin
中科院分区：
文献类型：
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作者：
Rosenblum, Joshua M.;Altin, Husnu Firat;Alsoufi, Bahaaldin
目的：原发性肺静脉狭窄（PPVS）越来越多地在没有肺静脉干预史的儿童中被诊断出来，并且治疗具有挑战性。我们描述了在我们中心接受手术修复PPVS的患者的特征，并检查了与治疗失败相关的因素。方法：回顾性分析2002-2016年所有接受手术治疗的PPVS患者。既往接受过涉及肺静脉或心房开关的心脏手术的患者被排除在外。进行回归分析以检查特征、PPVS特征，包括严重程度评分和与治疗失败相关的手术细节。结果：34名儿童接受了PPVS的初始手术干预。中位年龄为8.9个月（四分位数范围为5.9-18.4个月）。大多数患者（n = 31, 91%）有单侧肺静脉受累，中位PPVS严重程度评分为3.5（四分位数范围为3-5）。在竞争风险分析中，手术修复后1年，9%的患者死亡，14%的患者接受了再干预，77%的患者在没有再干预的情况下存活；5年后，这一数字分别为9%、30%和61%。与死亡率相关的因素包括双侧疾病和PPVS严重程度评分bb60。双侧疾病和PPVS严重程度评分bbb50与再干预风险相关。结论：PPVS需要多学科的管理策略。尽管早期修复令人满意，但患者仍然有复发和随后死亡的风险，特别是那些原发病灶广泛的患者。令人失望的结果强调了多机构合作的必要性，以更好地了解这种复杂的疾病，建立管理和随访方案，并探索研究性治疗模式，以改变这种罕见且具有挑战性的疾病的不利结果。
Objectives: Primary pulmonary vein stenosis (PPVS) is increasingly diagnosed in children with no prior pulmonary vein intervention history, and management is challenging. We describe characteristics of patients who underwent surgical repair of PPVS at our center, and examine factors associated with treatment failures.Methods: A retrospective review of all patients who underwent surgical intervention for PPVS (2002-2016) was completed. Patients who had undergone prior cardiac surgery involving the pulmonary veins or atrial switch were excluded. Regression analyses were performed to examine characteristics, PPVS features, including severity score, and surgical details associated with treatment failures.Results: Thirty-four children underwent initial surgical intervention for PPVS. Median age was 8.9 months (interquartile range, 5.9-18.4 months). Most patients (n = 31; 91%) had unilateral pulmonary vein involvement and the median PPVS severity score was 3.5 (interquartile range, 3-5). On competing risk analysis, 1 year following surgical repair, 9% of patients had died, 14% had undergone reintervention, and 77% were alive without reintervention; at 5 years the numbers were 9%, 30%, and 61%, respectively. Factors associated with mortality included bilateral disease and PPVS severity score >6. Bilateral disease and PPVS severity score >5 were associated with reintervention risk.Conclusions: Multidisciplinary management strategy is required for PPVS. Despite satisfactory early repair, patients continue to be at risk for recurrence and subsequent mortality, especially those with extensive primary involvement. The disappointing results underscore the need for multi-institutional collaborations to better understand this complex disease, establish management and follow-up protocols, and explore investigational treatment modalities that could modify the unfavorable outcome of this uncommon and challenging disease.