Pulmonary hypertension in Portugal: first data from a nationwide registry.
Pulmonary hypertension in Portugal: first data from a nationwide registry.
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DOI：
10.1155/2013/489574
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发表时间：
2013
影响因子：
--
通讯作者：
Reis A
中科院分区：
文献类型：
--
作者：
Baptista R;Meireles J;Agapito A;Castro G;da Silva AM;Shiang T;Gonçalves F;Robalo-Martins S;Nunes-Diogo A;Reis A
介绍。肺动脉高压（PAH）是一种罕见的疾病，必须在专门的中心管理；因此，获得流行病学国家数据至关重要。方法。我们与来自葡萄牙的五个中心联合开展了一项前瞻性、观察性和多中心注册研究，纳入了PAH或慢性血栓栓塞性肺动脉高压（CTEPH）的成人事件患者。结果。在本研究纳入的79例患者中，46例（58.2%）被分类为PAH， 33例（41.8%）被分类为CTEPH。PAH患者平均年龄43.4±16.4岁。特发性多环芳烃是最常见的病因（37%）。发病时，PAH患者右心房压（RAP）升高（7.7±5.9 mmHg），平均肺血管阻力（11.4±6.5 Wood units），心脏指数低（2.7±1.1 L·min−1·m−2）；无患者使用选择性肺血管扩张剂；然而，在随访中，大多数患者接受单一（50%）、双重（28%）或三重（9%）联合血管扩张剂治疗。一年生存率为93.5%，与CTEPH患者（93.9%）相似，CTEPH患者年龄较大（60.0±12.5岁），RAP较高（11.0±5.2 mmHg, P = 0.015）。结论。我们首次描述了葡萄牙PAH和CTEPH患者的诊断、管理和预后的全国性数据。临床表现和结果与其他国家登记的报告具有可比性。
Introduction. Pulmonary arterial hypertension (PAH) is a rare disease that must be managed in specialized centers; therefore, the availability of epidemiological national data is critical. Methods. We conducted a prospective, observational, and multicenter registry with a joint collaboration from five centers from Portugal and included adult incident patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH). Results. Of the 79 patients enrolled in this study, 46 (58.2%) were classified as PAH and 33 patients (41.8%) as CTEPH. PAH patients had a mean age of 43.4 ± 16.4 years. Idiopathic PAH was the most common etiology (37%). At presentation, PAH patients had elevated right atrial pressure (RAP) (7.7 ± 5.9 mmHg) and mean pulmonary vascular resistance (11.4 ± 6.5 Wood units), with a low cardiac index (2.7 ± 1.1 L·min−1 ·m−2); no patient was under selective pulmonary vasodilators; however, at follow-up, most patients were on single (50%), double (28%), or triple (9%) combination vasodilator therapy. One-year survival was 93.5%, similar to CTEPH patients (93.9%), that were older (60.0 ± 12.5 years) and had higher RAP (11.0 ± 5.2 mmHg, P = 0.015). Conclusions. We describe for the first time nationwide data on the diagnosis, management, and prognosis of PAH and CTEPH patients in Portugal. Clinical presentation and outcomes are comparable with those reported on other national registries.
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影响因子：
39.3
作者：
Galiè N;Manes A;Negro L;Palazzini M;Bacchi-Reggiani ML;Branzi A
通讯作者：
Branzi A
影响因子：
9.6
作者：
Jing, Zhi-Cheng;Xu, Xi-Qi;Yang, Yue-Jin
通讯作者：
Yang, Yue-Jin
影响因子：
168.9
作者：
Galie, N.;Rubin, L. J.;Simonneau, G.
通讯作者：
Simonneau, G.
影响因子：
158.5
作者：
Abenhaim, L;Moride, Y;Begaud, B
通讯作者：
Begaud, B
影响因子：
24.3
作者：
Peacock, A. J.;Murphy, N. F.;Stewart, S.
通讯作者：
Stewart, S.