From the Department of Anesthesiology, Critical Care and Pain Medicine, Division of Cardiac Anesthesia, Boston Children's Hospital, Boston, Massachusetts.
Department of Cardiology, Division of Invasive Cardiology, Boston Children's Hospital, Boston, Massachusetts.
Anesth Analg. 2019 Jul;129(1):27-40. doi: 10.1213/ANE.0000000000003924.
Pulmonary vein stenosis (PVS) is a rare disorder that leads to progressive narrowing of the extrapulmonary veins. PVS has been reported in both children and adults and in its worse iteration leads to pulmonary hypertension, right ventricular failure, and death. Multiple etiologies of PVS have been described in children and adults. This review will focus on intraluminal PVS in children. Intraluminal PVS has an estimated incidence ranging from 0.0017% to 0.03%. It is associated with conditions such as prematurity, bronchopulmonary dysplasia, necrotizing enterocolitis, Smith-Lemli-Opitz syndrome, and Down syndrome. Cardiac catheterization and pulmonary vein angiography are the gold standard for diagnosis and anatomic delineation. Other imaging modalities including magnetic resonance imaging, chest tomography, and transesophageal echocardiography are increasingly being used. Mortality of PVS in children is approximately 50%. Predictors of mortality include involvement of ≥3 pulmonary veins, bilateral pulmonary vein involvement, onset of PVS in infancy, elevated pulmonary artery pressure or systolic pulmonary artery-to-aortic pressure ratio, right ventricular dysfunction, restenosis after surgery, distal/upstream disease, and disease progression to previously uninvolved pulmonary veins. Treatment includes catheter-based pulmonary vein dilations with or without stenting, surgical interventions, medical therapy, and in some instances, lung transplantation. Cardiac catheterization for PVS involves a comprehensive hemodynamic and anatomic assessment of the pulmonary veins as well as therapeutic transcatheter interventions. Several surgical strategies have been used. Sutureless repair is currently most commonly used, but patch venoplasty, endarterectomy, ostial resection, and reimplantation are used in select circumstances as well. Medical therapies such as imatinib mesylate and bevacizumab are increasingly being used in an effort to suppress the myofibroblastic proliferation seen in PVS patients. Lung transplantation has been used as an alternative treatment strategy for end-stage, refractory PVS. Nonetheless, despite the different innovative approaches used, morbidity and mortality remain high. At present, the preferred treatment strategy is frequent reassessment of disease progression to guide use of catheter-based and surgical interventions in conjunction with medical therapy.
肺静脉狭窄（PVS）是一种罕见的疾病，可导致肺外静脉进行性狭窄。PVS 可发生于儿童和成人，严重时可导致肺动脉高压、右心衰竭和死亡。儿童和成人的 PVS 有多种病因。本综述将重点介绍儿童的腔内 PVS。腔内 PVS 的估计发病率为 0.0017%至 0.03%。它与早产儿、支气管肺发育不良、坏死性小肠结肠炎、Smith-Lemli-Opitz 综合征和唐氏综合征等疾病有关。心导管检查和肺静脉血管造影是诊断和解剖描绘的金标准。其他成像方式，包括磁共振成像、胸部计算机断层扫描和经食管超声心动图，也越来越多地被应用。儿童 PVS 的死亡率约为 50%。死亡率的预测因素包括：≥3 条肺静脉受累、双侧肺静脉受累、婴儿期发生 PVS、肺动脉压或收缩期肺动脉与主动脉压比值升高、右心功能障碍、手术后再狭窄、远端/上游病变以及疾病进展至以前未受累的肺静脉。治疗包括经导管肺静脉扩张术伴或不伴支架置入、手术干预、药物治疗，在某些情况下还包括肺移植。心导管检查用于 PVS 时，需要全面评估肺静脉的血流动力学和解剖情况，并进行治疗性经导管干预。目前已经采用了几种手术策略。无缝线修复术目前最常用，但在某些情况下也会使用补片静脉成形术、内膜切除术、口部切除术和再植入术。伊马替尼和贝伐单抗等药物治疗也越来越多地被用于抑制 PVS 患者的肌成纤维细胞增殖。肺移植已被用作晚期、难治性 PVS 的替代治疗策略。尽管采用了不同的创新方法，但发病率和死亡率仍然很高。目前，首选的治疗策略是频繁评估疾病进展，以指导使用经导管和手术干预联合药物治疗。
