左心疾病：系统性硬化症早期肺动脉高压的常见病因，与NT-proBNP水平升高或明显的心脏纤维化无关，但与MR-proANP和MR-proADM水平升高相关：对法裔加拿大人队列的回顾性分析

Left heart disease: a frequent cause of early pulmonary hypertension in systemic sclerosis, unrelated to elevated NT-proBNP levels or overt cardiac fibrosis but associated with increased levels of MR-proANP and MR-proADM: retrospective analysis of a French Canadian cohort.

作者信息

Miller L, Chartrand S, Koenig M, Goulet J-R, Rich É, Chin A S, Chartrand-Lefebvre C, Abrahamowicz M, Senécal J-L, Grodzicky T

机构信息

Division of Rheumatology, Hospital Centre of the University of Montreal (CHUM) , Montreal, Québec , Canada.

出版信息

Scand J Rheumatol. 2014;43(4):314-23. doi: 10.3109/03009742.2013.854407. Epub 2014 Feb 6.

DOI:10.3109/03009742.2013.854407

PMID:25089008

Abstract

OBJECTIVES

Pulmonary hypertension (PH) causes mortality in systemic sclerosis (SSc). Pulmonary arterial hypertension (PAH) and left heart disease (LHD) are frequent causes of PH. Therefore, we studied PAH and LHD in early PH.

METHOD

A total of 432 French Canadian SSc patients were studied retrospectively. All underwent screening for PH. We analysed clinical, serological, and radiographic data from 26 patients with early PH diagnosed by right heart catheterization (RHC). SSc patients with (n = 21) and without PH (n = 19) were prospectively re-evaluated by cardiac magnetic resonance imaging (MRI) and serial measurements of N-terminal pro-brain natriuretic peptide (NT-proBNP) and the haemodynamic biomarkers mid-regional pro-atrial natriuritic peptide (MR-proANP) and mid-regional pro-adrenomedullin (MR-proADM).

RESULTS

The most frequent cause of early PH was LHD (58%). PAH was seen in 34% of patients. No association was found between the type of PH and autoantibodies. Early LHD-PH, but not early PAH, was associated with lower NT-proBNP (p = 0.024), but MR-proANP and MR-proADM levels were higher in early LHD-PH than in patients without PH (p = 0.014 and p = 0.012, respectively). Only one patient had abnormal cardiac MRI explaining LHD-PH.

CONCLUSIONS

Early PH in SSc, like late PH, is heterogeneous and RHC is essential for determining its underlying cause. The most frequent cause of early PH was LHD. Levels of MR-proANP and MR-proADM, but not NT-proBNP, were increased in early LHD-PH, and may be more reliable than NT-proBNP as a biomarker of early PH in this subgroup of patients. Cardiac MRI did not explain LHD-PH. This study is the first to identify a high frequency of LHD in early PH correlating with normal NT-proBNP levels but increased MR-proANP and MR-proADM levels in SSc patients.

摘要

目的

肺动脉高压（PH）可导致系统性硬化症（SSc）患者死亡。肺动脉高压（PAH）和左心疾病（LHD）是PH的常见病因。因此，我们对早期PH中的PAH和LHD进行了研究。

方法

对432例法裔加拿大SSc患者进行回顾性研究。所有患者均接受了PH筛查。我们分析了26例经右心导管检查（RHC）确诊为早期PH患者的临床、血清学和影像学数据。对有PH（n = 21）和无PH（n = 19）的SSc患者进行了心脏磁共振成像（MRI）以及N末端脑钠肽前体（NT-proBNP）、血流动力学生物标志物中段心房利钠肽原（MR-proANP）和中段肾上腺髓质素（MR-proADM）的系列测量。

结果

早期PH最常见的病因是LHD（58%）。34%的患者存在PAH。未发现PH类型与自身抗体之间存在关联。早期LHD-PH而非早期PAH与较低的NT-proBNP相关（p = 0.024），但早期LHD-PH患者的MR-proANP和MR-proADM水平高于无PH患者（分别为p = 0.014和p = 0.012）。仅有1例患者心脏MRI异常可解释LHD-PH。

结论

SSc中的早期PH与晚期PH一样具有异质性，RHC对于确定其潜在病因至关重要。早期PH最常见的病因是LHD。早期LHD-PH患者的MR-proANP和MR-proADM水平升高，而NT-proBNP水平未升高，在该亚组患者中，作为早期PH的生物标志物，MR-proANP和MR-proADM可能比NT-proBNP更可靠。心脏MRI无法解释LHD-PH。本研究首次发现早期PH中LHD的高发生率与正常NT-proBNP水平相关，但SSc患者的MR-proANP和MR-proADM水平升高。