Department of Cardiology, Austin Health, Melbourne, Victoria, Australia.
Department of Respiratory and Sleep Medicine, Austin Health, Melbourne, Victoria, Australia.
Intern Med J. 2023 Aug;53(8):1415-1422. doi: 10.1111/imj.15887. Epub 2022 Aug 3.
Pulmonary hypertension (PH) is an important complication of interstitial lung disease (ILD), as its development confers a poor prognosis. There are no specific recommendations for methods of assessment for PH in ILD populations.
To determine current assessment practices for PH in an Australian ILD centre.
In the Austin Health ILD database, 162 consecutive patients with idiopathic pulmonary fibrosis or connective tissue disease-associated ILD were identified and retrospectively evaluated for methods of PH assessment with transthoracic echocardiography (TTE), serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and right heart catheterisation (RHC) in relation to patient demographic and physiological parameters.
The median follow-up was 30 (14.4-56.4) months. At baseline, vital capacity was 80.0 ± 18.4% predicted, and diffusing capacity for carbon monoxide was 59.6 ± 15.2% predicted. Evaluation for PH was performed in 147 (90.7%) patients, among whom 105 (64.8%) had TTE performed at least once. At the initial TTE, 33.7% patients had high probability of PH, defined as RVSP >40 mmHg + RAp and/or right ventricular dysfunction. At the time of the most recent TTE, these criteria were met in 45 (52.3%) patients. Elevated serum NT-proBNP levels during the first year were observed in 47 (38.8%) patients. Only 14 (8.6%) patients had RHC.
Our institutional PH assessment practice in ILD demonstrates a substantial prevalence of probable PH at baseline. As new therapies emerge for the treatment of PH in ILD, well-defined screening practices are important in this population for early identification and optimal management.
肺动脉高压（PH）是间质性肺疾病（ILD）的重要并发症，因为其发展预示着预后不良。ILD 人群中没有针对 PH 评估方法的具体建议。
确定澳大利亚ILD 中心 PH 的当前评估实践。
在奥斯汀健康ILD 数据库中，确定了 162 例特发性肺纤维化或结缔组织病相关ILD 连续患者，并回顾性评估了经胸超声心动图（TTE）、血清 N 末端脑利钠肽前体（NT-proBNP）和右心导管检查（RHC）评估 PH 的方法与患者的人口统计学和生理参数的关系。
中位随访时间为 30（14.4-56.4）个月。基线时，肺活量为预测值的 80.0±18.4%，一氧化碳弥散量为预测值的 59.6±15.2%。147 例（90.7%）患者进行了 PH 评估，其中 105 例（64.8%）至少进行了一次 TTE 检查。在初始 TTE 中，33.7%的患者有高概率 PH，定义为 RVSP>40mmHg+RAP 和/或右心室功能障碍。在最近一次 TTE 时，这些标准在 45 例（52.3%）患者中得到满足。在第一年观察到 47 例（38.8%）患者血清 NT-proBNP 水平升高。仅 14 例（8.6%）患者进行了 RHC。
ILD 患者的本机构 PH 评估实践表明，基线时存在相当大的可能 PH 患病率。随着治疗 ILD 中 PH 的新疗法的出现，在该人群中定义明确的筛查实践对于早期识别和最佳管理非常重要。
