Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry.
Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry.
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DOI：
10.1161/jaha.121.024969
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发表时间：
2022-05-03
影响因子：
5.4
通讯作者：
PHAR Investigators *
中科院分区：
文献类型：
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作者：
Chang KY;Duval S;Badesch DB;Bull TM;Chakinala MM;De Marco T;Frantz RP;Hemnes A;Mathai SC;Rosenzweig EB;Ryan JJ;Thenappan T;PHAR Investigators *
目前美国的肺动脉高压(PAH)死亡率数据是基于2010年前登记的患者。我们试图使用PHAR(肺高压协会注册)来确定现代PAH的死亡率。我们确定了在2015年9月至2020年9月期间在PHAR登记的所有成年PAH患者(N=935)。我们使用Kaplan-Meier生存分析和Cox比例风险模型来评估1、2和3年的死亡率。通过3个有效的风险评分，根据疾病严重程度对患者进行分层。在未接受治疗的患者中，我们比较了基于初始治疗策略的存活率。中位年龄为56岁(44-68岁)，其中76%为女性。在935例患者中，483例(52%)在确诊后6个月内发生了≤。中位随访时间为489天(281-812天)，121例死亡(12.9%)。1、2、3年死亡率分别为8%(95%可信区间，6%~10%)、16%(95%可信区间，13%~19%)和21%(95%可信区间，17%~25%)。按低、中、高危PAH分级，1、2、3年死亡率分别为1%、4%~6%、7%~11%；7%~8%、11%~16%、18%~20%；高危12%~19%、22%~38%、28%~55%。在未接受治疗的患者中，最初的联合治疗与较好的一年生存率相关(调整后的风险比，0.43[95%可信区间，0.19-0.95]；P=0.037)。中高危PAH患者的死亡率在PHAR中仍然高得令人无法接受，这表明早期诊断、积极使用现有治疗方法的重要性，以及需要更好的治疗方法。
Current mortality data for pulmonary arterial hypertension (PAH) in the United States are based on registries that enrolled patients prior to 2010. We sought to determine mortality in PAH in the modern era using the PHAR (Pulmonary Hypertension Association Registry). We identified all adult patients with PAH enrolled in the PHAR between September 2015 and September 2020 (N=935). We used Kaplan‐Meier survival analysis and Cox proportional hazards models to assess mortality at 1, 2, and 3 years. Patients were stratified based on disease severity by 3 validated risk scores. In treatment‐naïve patients, we compared survival based on initial treatment strategy. The median age was 56 years (44–68 years), and 76% were women. Of the 935 patients, 483 (52%) were ≤6 months from PAH diagnosis. There were 121 deaths (12.9%) during a median follow‐up time of 489 days (281–812 days). The 1‐, 2‐, and 3‐year mortality was 8% (95% CI, 6%–10%), 16% (95% CI, 13%–19%), and 21% (95% CI, 17%–25%), respectively. When stratified into low‐, intermediate‐, and high‐risk PAH, the mortality at 1, 2, and 3 years was 1%, 4% to 6%, and 7% to 11% for low risk; 7% to 8%, 11% to 16%, and 18% to 20% for intermediate risk; and 12% to 19%, 22% to 38%, and 28% to 55% for high risk, respectively. In treatment‐naïve patients, initial combination therapy was associated with better 1‐year survival (adjusted hazard ratio, 0.43 [95% CI, 0.19–0.95]; P=0.037). Mortality in the intermediate‐ and high‐risk patients with PAH remains unacceptably high in the PHAR, suggesting the importance for early diagnosis, aggressive use of available therapies, and the need for better therapeutics.