Santos Mário, Gomes Ana, Cruz Célia, Rocha Joana, Ricardo Miguel, Gonçalves Fabienne, Carvalho Luísa, Vicente Margarida, Melo Alzira, Reis Abílio
Unidade de Doença Vascular Pulmonar, Hospital Santo António, Centro Hospitalar do Porto, Porto, Portugal; Serviço de Cardiologia, Hospital Santo António, Centro Hospitalar do Porto, Porto, Portugal; Unidade de Investigação Cardiovascular, Faculdade de Medicina da Universidade do Porto, Porto, Portugal.
Serviço de Medicina Interna, Hospital Santo António, Centro Hospitalar do Porto, Porto, Portugal.
Rev Port Cardiol (Engl Ed). 2018 Sep;37(9):749-757. doi: 10.1016/j.repc.2018.02.009. Epub 2018 Aug 23.
This study aims to assess the long-term survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients followed in a Portuguese pulmonary hypertension (PH) referral center.
We studied PAH and CTEPH patients diagnosed between January 2005 and December 2016. Cumulative survival was estimated using the Kaplan-Meier method. Survival trends were compared over two periods (2005-2010 vs. 2011-2016).
Of the 142 studied PH patients (age 54±18 years; 31% male), 47 had CTEPH and 95 had group 1 PH. Most patients with CTEPH and idiopathic/heritable PAH (I/HPAH) were in NYHA III-IV at diagnosis (64% and 57%, respectively). At the time of death, 31% of patients with connective tissue disease (CTD)-associated PAH (CTD-PAH) and all I/HPAH patients were on double or triple combination therapy. No patient underwent lung transplantation. Pulmonary endarterectomy or angioplasty were performed in 36% of CTEPH patients. Age at diagnosis tended to increase over time in CTD-PAH (53±15 vs. 63±15 years; p=0.13) and I/HPAH (39±15 vs. 51±19 years; p=0.10). The five-year survival estimates for I/HPAH, CTD-PAH and CTEPH patients were 80%, 52%, and 81%, respectively. Over time, CTD-PAH and CTEPH showed better five-year survival (33 vs. 67% and 77 vs. 84%), but I/HPAH did not (84 vs. 75%).
Our data indicate a trend toward improved survival over time of CTD-PAH and CTEPH patients treated at a Portuguese referral PH center. Earlier diagnosis, increasing use of parenteral prostanoids, and surgical treatment may further improve PH prognosis.
本研究旨在评估在葡萄牙一家肺动脉高压（PH）转诊中心随访的肺动脉高压（PAH）和慢性血栓栓塞性肺动脉高压（CTEPH）患者的长期生存率。
我们研究了2005年1月至2016年12月期间诊断的PAH和CTEPH患者。采用Kaplan-Meier方法估计累积生存率。比较了两个时期（2005 - 2010年与2011 - 2016年）的生存趋势。
在142例接受研究的PH患者中（年龄54±18岁；31%为男性），47例患有CTEPH，95例患有1组PH。大多数CTEPH和特发性/遗传性PAH（I/HPAH）患者在诊断时处于纽约心脏协会（NYHA）III - IV级（分别为64%和57%）。在死亡时，31%的结缔组织病（CTD）相关PAH（CTD - PAH）患者和所有I/HPAH患者正在接受双联或三联联合治疗。没有患者接受肺移植。36%的CTEPH患者接受了肺动脉内膜切除术或血管成形术。CTD - PAH（53±15岁对63±15岁；p = 0.13）和I/HPAH（39±15岁对51±19岁；p = 0.10）的诊断年龄随时间有增加趋势。I/HPAH、CTD - PAH和CTEPH患者的五年生存率估计分别为80%、52%和81%。随着时间推移，CTD - PAH和CTEPH显示出更好的五年生存率（33%对67%和77%对84%），但I/HPAH没有（84%对75%）。
我们的数据表明，在葡萄牙一家转诊PH中心接受治疗的CTD - PAH和CTEPH患者的生存率随时间有改善趋势。早期诊断、增加肠外前列腺素的使用以及手术治疗可能进一步改善PH的预后。
