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芬兰肺动脉高压(PAH)或慢性血栓栓塞性肺动脉高压(CTEPH)患者的治疗途径。

Treatment pathways in Finnish patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH).

作者信息

Pentikäinen Markku, Simonen Piia, Leskelä Pauliina, Harju Terttu, Jääskeläinen Pertti, Asseburg Christian, Oksanen Minna, Soini Erkki, Wennerström Christina, Puhakka Airi, Kahlos Katriina

机构信息

Helsinki University Hospital University of Helsinki Helsinki Finland.

Tampere University Hospital Tampere Finland.

出版信息

Pulm Circ. 2025 Feb 4;15(1):e12440. doi: 10.1002/pul2.12440. eCollection 2025 Jan.

Abstract

Treatment patterns of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in Finland are unknown. Guidelines now recommend early escalation of treatment for PAH. We evaluated how well Finnish practice follows guidelines, and how treatment initiations and outcomes are related. The pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension patients in Finland cohort includes all PAH and CTEPH patients diagnosed between 2008 and 2020 in all Finnish university hospitals. Drug therapy was analysed in patients with medical/procedural history available, and changes in the 4-tier comparative, prospective registry of newly initiated therapies for pulmonary hypertension (COMPERA) 2.0 risk score were evaluated. PAH patients ( = 268) were initially treated with monotherapy (52%) or double therapy (24%). After year 2015, double therapy use increased to 39%. PAH treatment at 1 year after diagnosis included phosphodiesterase 5 inhibitors (71%), endothelin-receptor antagonist (48%), prostacyclin analogue (7%), calcium channel blocker (12%) and selexipag (1%). 35% achieved low risk at 1 year, increasing to 44% for patients diagnosed after 2015. Those remaining at intermediate-high (IH) or high risk (H) (28%) were not treated less aggressively than others but were older, had more comorbidities, and often history of smoking. CTEPH patients ( = 189) were treated with pulmonary endarterectomy (PEA) (27%), balloon pulmonary angioplasty (BPA) (11%) and medical therapy only (41%) within 1 year from diagnosis. 45% achieved low risk at 1 year. We present additional results on treatment of IH and H patients, patient characteristics preceding death, and treatment persistence. We found less treatment of PAH patients with double or triple therapies and of CTEPH patients with PEA and BPA than expected but with good results. Patients not reaching low or intermediate COMPERA 2.0 were old and had comorbidities.

摘要

芬兰肺动脉高压(PAH)和慢性血栓栓塞性肺动脉高压(CTEPH)的治疗模式尚不清楚。目前指南推荐对PAH进行早期强化治疗。我们评估了芬兰的实际治疗情况与指南的符合程度,以及治疗起始与治疗结果之间的关系。芬兰队列中的肺动脉高压和慢性血栓栓塞性肺动脉高压患者包括2008年至2020年期间在芬兰所有大学医院确诊的所有PAH和CTEPH患者。对有可用医疗/手术史的患者的药物治疗进行了分析,并评估了肺动脉高压新起始治疗的4级比较性前瞻性登记(COMPERA)2.0风险评分的变化。PAH患者(n = 268)最初接受单药治疗(52%)或双联治疗(24%)。2015年后,双联治疗的使用增加到39%。诊断后1年的PAH治疗包括磷酸二酯酶5抑制剂(71%)、内皮素受体拮抗剂(48%)、前列环素类似物(7%)、钙通道阻滞剂(12%)和司来帕格(1%)。35%的患者在1年时达到低风险,2015年后确诊的患者这一比例增至44%。那些仍处于中高(IH)或高风险(H)(28%)的患者治疗并不比其他患者积极程度低,但年龄更大,合并症更多,且常有吸烟史。CTEPH患者(n = 189)在诊断后1年内接受了肺动脉内膜剥脱术(PEA)(27%)、球囊肺动脉成形术(BPA)(11%)和仅药物治疗(41%)。45%的患者在1年时达到低风险。我们还展示了关于IH和H患者治疗、死亡前患者特征以及治疗持续性的其他结果。我们发现,PAH患者接受双联或三联治疗以及CTEPH患者接受PEA和BPA治疗的情况比预期少,但效果良好。未达到COMPERA 2.0低或中风险的患者年龄较大且有合并症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2081/11791409/bd2b556e8e1f/PUL2-15-e12440-g004.jpg

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