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结节病患者的肺动脉高压:单中心经验。

Pulmonary hypertension in patients with sarcoidosis: A single-center experience.

机构信息

Department of Cardiology, Derince Training and Research Hospital; Kocaeli-Turkey.

Department of Cardiology, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital; İstanbul-Turkey.

出版信息

Anatol J Cardiol. 2021 Jan;25(1):36-41. doi: 10.14744/AnatolJCardiol.2020.88054.

Abstract

OBJECTIVE

Sarcoidosis is a systemic granulomatous disease rarely complicated by pulmonary hypertension (PH). The prevalence of PH in sarcoidosis is unclear and has differences between ethnic groups. This study aimed to investigate the prevalence and predictors of PH in a Turkish cohort.

METHODS

The study included 55 patients with biopsy-proven sarcoidosis in a single center. All patients underwent detailed transthoracic echocardiography (TTE) to assess the probability of PH as recommended. Right heart catheterization (RHC) was performed for patients with intermediate-high risk of PH. Patients with mean pulmonary artery pressure >20 mm Hg by RHC were defined as PH. Demographic and clinical characteristics, laboratory data, spirometry, 6-min walk test, and TTE were compared between low and intermediate-high risk PH groups.

RESULTS

The probability of PH was low with 47 patients. Eight patients had intermediate-high probability of PH, and two of them refused to undergo RHC. Of six intermediate-high probability patients, three had PH, and all of them had post-precapillary PH. The prevalence of PH in sarcoidosis was 5.5% (3/55). Six-minute walk distance (6 MWD) and diastolic parameters (E/A ratio, E' wave, and left atrial volume) were significantly lower, and New York Heart association class and N-terminal probrain natriuretic peptide (NT-proBNP) level were higher in intermediate-high risk PH patients compared with low-risk PH patients.

CONCLUSION

The frequency of PH in sarcoidosis was 5.5% in a Turkish cohort. NT-proBNP, 6 MWD, diastolic function parameters, and myocardial strain parameters can be useful predictors of PH in patients with sarcoidosis, besides known echocardiographic parameters.

摘要

目的

结节病是一种罕见合并肺动脉高压(PH)的系统性肉芽肿性疾病。结节病合并 PH 的患病率尚不清楚,且在不同种族间存在差异。本研究旨在探讨土耳其队列中 PH 的患病率和预测因素。

方法

本研究纳入了单中心 55 例经活检证实的结节病患者。所有患者均接受详细的经胸超声心动图(TTE)检查,以评估 PH 的可能性,推荐采用该方法。对于 PH 中高危患者行右心导管检查(RHC)。RHC 提示平均肺动脉压>20mmHg 的患者定义为 PH。比较低危和中高危 PH 组患者的人口统计学和临床特征、实验室数据、肺功能、6 分钟步行试验和 TTE。

结果

47 例患者 PH 可能性低,8 例患者 PH 中高危,其中 2 例拒绝行 RHC。在 6 例中高危 PH 患者中,有 3 例存在 PH,且均为毛细血管前 PH。结节病 PH 的患病率为 5.5%(3/55)。与低危 PH 患者相比,中高危 PH 患者的 6 分钟步行距离(6MWD)和舒张功能参数(E/A 比值、E'波和左心房容积)显著降低,纽约心功能协会(NYHA)心功能分级和 N 末端脑利钠肽前体(NT-proBNP)水平更高。

结论

在土耳其队列中,结节病合并 PH 的频率为 5.5%。除了已知的超声心动图参数外,NT-proBNP、6MWD、舒张功能参数和心肌应变参数可作为结节病患者 PH 的有用预测因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/55c2/7803809/6e962286743f/AJC-25-1-36-g01.jpg

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