Predictors of outcome in chronic thromboembolic pulmonary hypertension

Predictors of outcome in chronic thromboembolic pulmonary hypertension
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DOI:
10.1161/circulationaha.106.661041
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发表时间:
2007-04-24
期刊:
影响因子:
37.8
通讯作者:
Lang, Irene M.
Lang, Irene M.
中科院分区:
医学1区
文献类型:
--
作者:
Bonderman, Diana;Skoro-Sajer, Nika;Lang, Irene M.

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背景——慢性血栓栓塞性肺动脉高压(CTEPH)的特点是腔内血栓组织和肺动脉纤维闭塞。最近,发现相关的医疗状况,如脾切除术、用于治疗脑积水的脑室分流术、永久性中央静脉导管、炎症性肠病和骨髓炎,与 CTEPH 的发生有关。该研究的目的是确定这些新的危险因素对生存的影响。方法和结果 - 1992 年 1 月至 2006 年 12 月期间,使用我们中心定制的计算机数据库对 181 名诊断为 CTEPH 的患者进行了跟踪。使用 Cox 回归模型来检查生存率与相关医疗状况、年龄、性别、血流动力学参数、诊断时改良的纽约心脏协会功能分级、CTEPH 类型、肺动脉内膜切除术和抗心磷脂抗体/狼疮抗凝剂之间的关系。在中位观察时间22.1个月(范围0.03至152)个月内,48例(27%)出现心血管死亡或肺移植的临床终点。肺动脉内膜切除术(风险比,0.14;95% CI,0.05至0.41;P=0.0003)、相关医疗状况(风险比,3.17;95% CI,1.70至5.92;P=0.0003)和肺血管阻力(风险比,1.02;95% CI,1.00至1.04;P=0.04)是生存的预测因子。有相关医疗状况的患者术后 30 天死亡率(24% 比 9%)和术后肺动脉高压发生率(92% 比 20%)显着更高。结论 - CTEPH 易感医疗状况,如脾切除、永久性中央静脉导管和某些炎症性疾病,预测 CTEPH 的生存率较差。
Background - Chronic thromboembolic pulmonary hypertension ( CTEPH) is characterized by intraluminal thrombus organization and fibrous obliteration of pulmonary arteries. Recently, associated medical conditions such as splenectomy, ventriculoatrial shunt for the treatment of hydrocephalus, permanent central intravenous lines, inflammatory bowel disease, and osteomyelitis were found to be associated with the development of CTEPH. The study aim was to define the impact of these novel risk factors on survival.Methods and Results - Between January 1992 and December 2006, 181 patients diagnosed with CTEPH were tracked with the use of our center's customized computer database. A Cox regression model was used to examine relations between survival and associated medical conditions, age, sex, hemodynamic parameters, modified New York Heart Association functional class at diagnosis, CTEPH type, pulmonary endarterectomy, and anti-cardiolipin antibodies/lupus anticoagulant. During a median observation time of 22.1 ( range, 0.03 to 152) months, the clinical end point of cardiovascular death or lung transplantation occurred in 48 cases ( 27%). Pulmonary endarterectomy ( hazard ratio, 0.14; 95% CI, 0.05 to 0.41; P=0.0003), associated medical conditions ( hazard ratio, 3.17; 95% CI, 1.70 to 5.92; P=0.0003), and pulmonary vascular resistance ( hazard ratio, 1.02; 95% CI, 1.00 to 1.04; P=0.04) were predictors of survival. Thirty-day postoperative mortality ( 24% versus 9%) and the incidence of postoperative pulmonary hypertension ( 92% versus 20%) were substantially higher in patients with associated medical conditions.Conclusions - CTEPH-predisposing medical conditions, such as splenectomy, permanent central intravenous lines, and certain inflammatory disorders, predict poor survival in CTEPH.