慢性血栓栓塞性肺动脉高压患者的长期预后：国际前瞻性登记研究结果。
Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry.
机构信息
From KU Leuven - University of Leuven, University Hospitals of Leuven, Belgium (M.D.); Medical University of Vienna, Austria (I.L.); Papworth Hospital, Cambridge, United Kingdom (J.P.-Z., C.T., D.J.); Clinical Department of Cardiology and Angiology of the First Faculty of Medicine and General Teaching Hospital, Prague; Czech Republic (P.J., D.A., J.L.); San Matteo Hospital, University of Pavia, Italy (A.M.D., M.M.); St Antonius Ziekenhuis, Nieuwegein, The Netherlands (R.S.); OLVG, Amsterdam, The Netherlands (P.B.); Medical Center for Postgraduate Education, ECZ-Otwock, Poland (A.T.); Aarhus University Hospital, Skejby, Denmark (S.M.); Regional Specialist Hospital and Medical University, Wroclaw, Poland (J.L.); Slovak Medical University and National Institute of Cardiovascular Diseases, Bratislava, Slovakia (I.S.); Hospital Clínic- IDIBAPS-CIBER Enfermedades Respiratorias, Universtiy of Barcelona, Spain (J.A.B.); Toronto General Hospital, Canada (M.d.P.); Medizinische Hochschule Hannover, Germany, and German Center of Lung Research (DZL), Hannover, Germany (M.M.H.); Mater Misericordiae University Hospital, Dublin, Ireland (S.G.); Universitätspital Zürich, Switzerland (R.S.); Hospital Universitario 12 Octubre-CIBER Enfermedades Respiratorias, Madrid, Spain (M.A.G.-S.); Medical University of Graz, Austria and Ludwig Boltzmann Institute for Lung Vascular Research, Austria (G.K.); University Paris Sud (Paris XI), INSERM U 999, Hôpital Le Kremlin Bicêtre, France (X.J., P.D., G.S.); and Kerckhoff Heart and Lung Center, Bad Nauheim, Germany (E.M.).
出版信息
Circulation. 2016 Mar 1;133(9):859-71. doi: 10.1161/CIRCULATIONAHA.115.016522. Epub 2016 Jan 29.
BACKGROUND
Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic obstructions of large pulmonary arteries combined with small-vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated patients who have chronic thromboembolic pulmonary hypertension.
METHODS AND RESULTS
A total of 679 patients newly diagnosed with chronic thromboembolic pulmonary hypertension were prospectively included over a 24-month period. Estimated survival at 1, 2, and 3 years was 93% (95% confidence interval [CI], 90-95), 91% (95% CI, 87-93), and 89% (95% CI, 86-92) in operated patients (n=404), and only 88% (95% CI, 83-91), 79% (95% CI, 74-83), and 70% (95% CI, 64-76) in not-operated patients (n=275). In both operated and not-operated patients, pulmonary arterial hypertension-targeted therapy did not affect survival estimates significantly. Mortality was associated with New York Heart Association functional class IV (hazard ratio [HR], 4.16; 95% CI, 1.49-11.62; P=0.0065 and HR, 4.76; 95% CI, 1.76-12.88; P=0.0021), increased right atrial pressure (HR, 1.34; 95% CI, 0.95-1.90; P=0.0992 and HR, 1.50; 95% CI, 1.20-1.88; P=0.0004), and a history of cancer (HR, 3.02; 95% CI, 1.36-6.69; P=0.0065 and HR, 2.15; 95% CI, 1.18-3.94; P=0.0129) in operated and not-operated patients, respectively. Additional correlates of mortality were bridging therapy with pulmonary arterial hypertension-targeted drugs, postoperative pulmonary hypertension, surgical complications, and additional cardiac procedures in operated patients, and comorbidities such as coronary disease, left heart failure, and chronic obstructive pulmonary disease in not-operated patients.
CONCLUSIONS
The long-term prognosis of operated patients currently is excellent and better than the outcome of not-operated patients.
背景
慢性血栓栓塞性肺动脉高压是急性肺栓塞的罕见并发症，其特征是大肺动脉的纤维血栓阻塞，伴有小血管动脉病。它可以通过肺动脉内膜切除术治愈，对于不能手术的患者，可以通过药物治疗在临床上得到改善。在 27 个中心建立了一个欧洲登记处，以评估接受和未接受慢性血栓栓塞性肺动脉高压治疗的 2 个不同人群的长期预后及其预后相关性。
方法和结果
在 24 个月的时间内，前瞻性纳入了 679 例新诊断为慢性血栓栓塞性肺动脉高压的患者。手术患者（n=404）的估计 1 年、2 年和 3 年生存率分别为 93%（95%置信区间[CI]，90-95）、91%（95%CI，87-93）和 89%（95%CI，86-92），而非手术患者（n=275）分别为 88%（95%CI，83-91）、79%（95%CI，74-83）和 70%（95%CI，64-76）。在手术和非手术患者中，肺动脉高压靶向治疗均未显著影响生存率估计值。死亡率与纽约心脏协会功能分级 IV 相关（危险比[HR]，4.16；95%CI，1.49-11.62；P=0.0065 和 HR，4.76；95%CI，1.76-12.88；P=0.0021），右心房压升高（HR，1.34；95%CI，0.95-1.90；P=0.0992 和 HR，1.50；95%CI，1.20-1.88；P=0.0004），以及癌症病史（HR，3.02；95%CI，1.36-6.69；P=0.0065 和 HR，2.15；95%CI，1.18-3.94；P=0.0129），分别在手术和非手术患者中。手术患者死亡率的其他相关因素包括肺动脉高压靶向药物桥接治疗、术后肺动脉高压、手术并发症和额外的心脏手术，而非手术患者死亡率的其他相关因素包括合并症，如冠心病、左心衰竭和慢性阻塞性肺疾病。
结论
目前手术患者的长期预后良好，优于未手术患者的预后。
Zotero 插件