Callahan Ryan, Gauvreau Kimberlee, Marshall Audrey C, Sena Laureen M, Baird Christopher W, Ireland Christina M, McEnaney Kerry, Bjornlund Elsa C, Mendonca Juliana T, Jenkins Kathy J
Department of Cardiology, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115, USA.
Department of Radiology, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115, USA.
Children (Basel). 2021 Mar 10;8(3):210. doi: 10.3390/children8030210.
The purpose of this study was to determine what patient and pulmonary vein characteristics at the diagnosis of intraluminal pulmonary vein stenosis (PVS) are predictive of individual vein outcomes. A retrospective, single-center, cohort sub-analysis of individual pulmonary veins of patients enrolled in the clinical trial NCT00891527 using imatinib mesylate +/- bevacizumab as adjunct therapy for the treatment of multi-vessel pediatric PVS between March 2009 and December 2014 was performed. The 72-week outcomes of the individual veins are reported. Among the 48 enrolled patients, 46 patients and 182 pulmonary veins were included in the study. Multivariable analysis demonstrated that patients with veins without distal disease at baseline (odds ratio, OR 3.69, 95% confidence interval, CI [1.52, 8.94], = 0.004), location other than left upper vein (OR 2.58, 95% CI [1.07, 6.19], = 0.034), or veins in patients ≥ 1 y/o (OR 5.59, 95% CI [1.81, 17.3], = 0.003) were at higher odds of having minimal disease at the end of the study. Veins in patients who received a higher percentage of eligible drug doses required fewer reinterventions (IRR 0.76, 95% CI [0.68, 0.85], < 0.001). The success of a multi-modal treatment approach to aggressive PVS depends on the vein location, disease severity, and drug dose intensity.
本研究的目的是确定腔内肺静脉狭窄（PVS）诊断时的哪些患者和肺静脉特征可预测个体静脉的预后。对参加临床试验NCT00891527的患者的个体肺静脉进行了一项回顾性、单中心队列亚分析，该试验在2009年3月至2014年12月期间使用甲磺酸伊马替尼+/-贝伐单抗作为辅助治疗多支小儿PVS。报告了个体静脉的72周预后。在48名入组患者中，46名患者和182条肺静脉纳入研究。多变量分析表明，基线时静脉无远端疾病的患者（比值比，OR 3.69，95%置信区间，CI [1.52, 8.94]，P = 0.004）、位于左上静脉以外位置的静脉（OR 2.58，95% CI [1.07, 6.19]，P = 0.034）或年龄≥1岁患者的静脉（OR 5.59，95% CI [1.81, 17.3]，P = 0.003）在研究结束时疾病轻微的几率更高。接受更高比例合格药物剂量的患者的静脉需要更少的再次干预（IRR 0.76，95% CI [0.68, 0.85]，P < 0.001）。积极治疗PVS的多模式治疗方法的成功取决于静脉位置、疾病严重程度和药物剂量强度。
