Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany (K.M.O., M.M.H.); Department of Pneumology, University Hospitals of Leuven, Leuven, Belgium (M.D.); University of Giessen and Marburg Lung Center (UGMLC), German Center for Lung Research (DZL), Hannover, Germany (H.A.G., H.T.); Department of Rheumatology and Clinical Immunology, Charité University Hospital, and Epidemiology unit, German Rheumatism Research Centre, Berlin, Germany (D.H.); Clinic of Internal Medicine, University Hospital, Zurich, Switzerland (R.S.); University Hospital Heidelberg, Heidelberg, Germany (E.G.); Medical Clinic I, Clinic Loewenstein, Loewenstein, Germany (G.S.); Clinic III for Internal Medicine (Cardiology), and Center for Molecular Medicine Cologne (CMMC), University of Cologne, Germany (S.R.); Internal Medicine I, University Hospital Carl Gustav Carus of Technical University Dresden, Dresden, Germany (M. Halank); Department of Internal Medicine, Respiratory Medicine and Cardiology, Mission Medical Hospital, Würzburg, Germany (M. Held); Department of Internal Medicine II, Division of Pneumology, University Medical Center Regensburg, Regensburg, Germany (T.J.L.); Department of Internal Medicine V, University of Munich, Munich, Germany (J.B.); University Medical Center Hamburg-Eppendorf, Center of Oncology, Department of Respiratory Medicine, Hamburg, Germany (H. Klose); LungenClinic Grosshansdorf, Germany (M.C.); Clinic of Internal Medicine, Department of Respiratory Medicine, Ernst Moritz Arndt University of Greifswald, Greifswald, Germany (R.E.); Department of Cardiology, DRK Kliniken Berlin Köpenick, Berlin, Germany (C.F.O.); Department of Cardiovascular and Respiratory Diseases, Sapienza, University of Rome; Rome, Italy (C.D.V.); Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy (L.S.); Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands (A.V.-N.); Dep
Circulation. 2014 Jan 7;129(1):57-65. doi: 10.1161/CIRCULATIONAHA.113.004526. Epub 2013 Sep 30.
For almost 30 years, anticoagulation has been recommended for patients with idiopathic pulmonary arterial hypertension (IPAH). Supporting evidence, however, is limited, and it is unclear whether this recommendation is still justified in the modern management era and whether it should be extended to patients with other forms of pulmonary arterial hypertension (PAH).
We analyzed data from Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA), an ongoing European pulmonary hypertension registry. Survival rates of patients with IPAH and other forms of PAH were compared by the use of anticoagulation. The sample consisted of 1283 consecutively enrolled patients with newly diagnosed PAH. Anticoagulation was used in 66% of 800 patients with IPAH and in 43% of 483 patients with other forms of PAH. In patients with IPAH, there was a significantly better 3-year survival (P=0.006) in patients on anticoagulation compared with patients who never received anticoagulation, albeit the patients in the anticoagulation group had more severe disease at baseline. The survival difference at 3 years remained statistically significant (P=0.017) in a matched-pair analysis of n=336 IPAH patients. The beneficial effect of anticoagulation on survival of IPAH patients was confirmed by Cox multivariable regression analysis (hazard ratio, 0.79; 95% confidence interval, 0.66-0.94). In contrast, the use of anticoagulants was not associated with a survival benefit in patients with other forms of PAH.
The present data suggest that the use of anticoagulation is associated with a survival benefit in patients with IPAH, supporting current treatment recommendations. The evidence remains inconclusive for other forms of PAH.
http://www.clinicaltrials.gov. Unique identifier: NCT01347216.
近 30 年来，一直建议对特发性肺动脉高压（IPAH）患者进行抗凝治疗。然而，支持这一建议的证据有限，并且不清楚在现代管理时代，这一建议是否仍然合理，以及是否应该扩展到其他形式的肺动脉高压（PAH）患者。
我们分析了正在进行的欧洲肺动脉高压注册研究——比较性、前瞻性新发起的肺动脉高压治疗注册研究（COMPERA）中的数据。使用抗凝治疗比较 IPAH 和其他形式 PAH 患者的生存率。该样本包括 1283 例新诊断为 PAH 的连续入组患者。在 800 例 IPAH 患者中，有 66%使用了抗凝治疗，在 483 例其他形式 PAH 患者中，有 43%使用了抗凝治疗。在 IPAH 患者中，与从未接受抗凝治疗的患者相比，接受抗凝治疗的患者 3 年生存率显著提高（P=0.006），尽管抗凝组患者的基线疾病更严重。在 n=336 例 IPAH 患者的匹配对分析中，3 年时的生存差异仍具有统计学意义（P=0.017）。Cox 多变量回归分析证实抗凝治疗对 IPAH 患者生存的有益影响（风险比，0.79；95%置信区间，0.66-0.94）。相比之下，抗凝治疗与其他形式 PAH 患者的生存获益无关。
目前的数据表明，抗凝治疗与 IPAH 患者的生存获益相关，支持当前的治疗建议。对于其他形式的 PAH，证据仍然不确定。
