Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China.
Medical Intensive Care Unit, Peking Union Medical College Hospital (PUMCH), Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.
BMC Pulm Med. 2022 Aug 1;22(1):295. doi: 10.1186/s12890-022-02081-0.
Connective tissue disease associated pulmonary hypertension (CTD-PH) is classified as a subgroup of WHO group 1 PH, also called pulmonary arterial hypertension (PAH). However, not all CTD-PH fit hemodynamic definition of PAH. This study investigates the diversity of hemodynamic types of CTD-PH, their differences in clinical characteristics and outcomes.
We performed a retrospective cohort study. CTD-PH patients were enrolled and divided into WHO group1 PH, WHO group 2 PH and hyperdynamic PH (mPAP > 20 mmHg, PVR < 3WU, PAWP < 15 mmHg) according to hemodynamics obtained by right heart catheterization. Patients with severe lung diseases, heart failure with reduced ejection fraction, pulmonary embolism, and hepatic cirrhosis were excluded. Baseline characteristics, autoantibodies, cardiac function, echocardiogram parameters, hemodynamics and survival rates were compared.
A total of 202 CTD-PH patients were included, 138 in WHO group 1 PH, 33 in WHO group 2 PH and 31 in hyperdynamic PH. We found hyperdynamic PH is less severe, presenting lower NT-proBNP level, better WHO function class, lower mPAP and PVR, higher cardiac output, and less cardiac remodeling. Incidence of anti-RNP was significantly lower in patients with elevated PAWP. Short-term survival was worse in WHO group 2 PH, yet 5-year survival rates didn't differ between groups.
Considering diversity in hemodynamic types, CTD-PH is more than a subgroup of PAH. Different types of CTD-PH present different clinical phenotypes and outcome. Phenotyping PH in CTD-PH patients is important.
结缔组织病相关肺动脉高压（CTD-PH）被归类为世界卫生组织（WHO）第 1 组肺动脉高压（PAH）的一个亚组，也称为肺动脉高压（PAH）。然而，并非所有的 CTD-PH 都符合 PAH 的血流动力学定义。本研究调查了 CTD-PH 的血流动力学类型的多样性，及其在临床特征和结局方面的差异。
我们进行了一项回顾性队列研究。根据右心导管检查获得的血流动力学，将 CTD-PH 患者分为 WHO 第 1 组 PH、WHO 第 2 组 PH 和高动力 PH（mPAP>20mmHg，PVR<3WU，PAWP<15mmHg）。排除严重肺部疾病、射血分数降低性心力衰竭、肺栓塞和肝硬化患者。比较了基线特征、自身抗体、心功能、超声心动图参数、血流动力学和生存率。
共纳入 202 例 CTD-PH 患者，其中 138 例为 WHO 第 1 组 PH，33 例为 WHO 第 2 组 PH，31 例为高动力 PH。我们发现高动力 PH 程度较轻，NT-proBNP 水平较低，WHO 功能分级较好，mPAP 和 PVR 较低，心输出量较高，心脏重构较少。PAWP 升高患者抗 RNP 发生率明显降低。WHO 第 2 组 PH 的短期生存率较差，但各组 5 年生存率无差异。
考虑到血流动力学类型的多样性，CTD-PH 不仅仅是 PAH 的一个亚组。不同类型的 CTD-PH 表现出不同的临床表型和结局。对 CTD-PH 患者进行 PH 表型分析很重要。
