Analysis Group, Inc., Boston, MA, USA.
Curr Med Res Opin. 2011 Sep;27(9):1763-8. doi: 10.1185/03007995.2011.604310. Epub 2011 Jul 27.
The prevalence of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in the US is largely unknown. Prior research has estimated PAH prevalence in Europe at ∼15-52 per million.
Using a privately insured claims database (1999-2007) for the under age 65 population and a Medicare claims database for the 65+ population, and following the current clinical classification of PH, CTEPH patients were identified as having: ≥2 claims for pulmonary hypertension (PH) [ICD-9-CM: 416.0, 416.8]; ≥1 claim for pulmonary embolism (PE) ≤12 months prior or 1 month after the initial PH claim (index date). PAH patients were identified: ≥2 claims for primary PH [416.0]; no left heart disease, lung diseases, CTEPH, or miscellaneous PH diagnoses ≤12 months prior or 1 month after the index date. Both cohorts were required to have ≥1 claim for right heart catheterization ≤6 months prior to any PH claim, or ≥1 claim for echocardiogram ≤6 months prior to a specialist-diagnosed PH claim. Age- and gender-standardized prevalence rates per million individuals (PMI) were calculated using appropriate population weights.
Prevalence rates (95% CI) of CTEPH were estimated at 63 (34-91) PMI among the privately insured (<65), and 1007 (904-1111) PMI among the Medicare population (≥65). The corresponding estimates for PAH were 109 (71-146) PMI among the <65 population, and 451 (384-519) PMI for Medicare.
Identification of PAH and CTEPH patients in administrative claims data is challenging, due to lack of specific ICD-9-CM codes for the conditions and risk of misdiagnosis.
Prevalence rates of CTEPH and PAH increase with age, and are higher among women. The increased risk of PE may explain the sharp age gradient for CTEPH prevalence. The estimated US prevalence of PAH is higher than existing estimates.
美国肺动脉高压（PAH）和慢性血栓栓塞性肺动脉高压（CTEPH）的患病率尚不清楚。先前的研究估计，欧洲 PAH 的患病率约为每百万 15-52 例。
使用私人保险索赔数据库（1999-2007 年）对 65 岁以下人群进行分析，使用医疗保险索赔数据库对 65 岁以上人群进行分析，并采用当前的 PH 临床分类，将 CTEPH 患者定义为：≥2 次肺动脉高压（PH）索赔[ICD-9-CM：416.0、416.8]；在首次 PH 索赔（索引日期）前 12 个月或之后 1 个月内，至少有 1 次肺栓塞（PE）索赔。PAH 患者的定义为：≥2 次原发性 PH 索赔[416.0]；在索引日期前 12 个月或之后 1 个月内，无左心疾病、肺部疾病、CTEPH 或其他 PH 诊断。两个队列都需要在任何 PH 索赔前 6 个月内至少有 1 次右心导管检查或在专家诊断 PH 索赔前 6 个月内至少有 1 次超声心动图检查。使用适当的人口权重计算每百万个体（PMI）的年龄和性别标准化患病率。
在私人保险人群中（<65 岁），CTEPH 的患病率估计为 63（34-91）/百万，在医疗保险人群中（≥65 岁）为 1007（904-1111）/百万。<65 岁人群中 PAH 的相应估计值为 109（71-146）/百万，医疗保险人群中为 451（384-519）/百万。
由于缺乏特定的 ICD-9-CM 编码，以及误诊的风险，在行政索赔数据中识别 PAH 和 CTEPH 患者具有挑战性。
CTEPH 和 PAH 的患病率随年龄增长而增加，且女性患病率更高。PE 的风险增加可能解释了 CTEPH 患病率的急剧年龄梯度。估计的美国 PAH 患病率高于现有估计值。
