小结
定义
病史和体格检查
关键诊断因素
- 20 - 50 岁
- 女性
- 呼吸困难
- 第二心音中的肺音增强（P2）
- 三尖瓣反流杂音
- 家族史
其他诊断因素
- 疲劳
- 外周水肿
- 发绀
- 兴奋剂使用
- 晕厥
- 胸痛
- 近晕厥
- 肺区舒张早期、高音调杂音
- 颈静脉怒张
危险因素
- 家族史
- 女性
- 2 型骨形态生成蛋白受体（BMPR2）突变
- 食欲抑制剂
- 药物及毒素
诊断性检查
首要检查
- 胸部 X 线检查
- 心电图 (ECG)
- 经胸多普勒超声心动图
- 右心导管检查
- 抗核抗体 (ANA)
- 肺功能检查
- 动脉血气 （ABG）
- 夜间血氧测定
- 通气与血流灌注比值 (V/Q) 显像
- 6 分钟步行试验
- B 型利尿钠肽 (BNP) 或 N 末端脑利钠肽激素原 (NT-proBNP)
- 全血细胞计数 (FBC）
- 肝功能检查 (LFT)
- 甲状腺功能检测
- HIV 血清学
需考虑的检查
- 高分辨率胸部 CT 扫描 ± CT 肺血管造影
- 心脏磁共振成像
- 使用吸入一氧化氮、吸入伊洛前列素或静脉用依前列醇进行血管扩张试验
治疗流程
急性血管反应试验为阳性反应，无钙通道阻滞剂禁忌证
急性血管反应试验阴性或存在钙离子通道阻滞剂禁忌证：无心肺合并症
撰稿人
作者
Muhammad Sameed, MD
Pulmonary & Critical Care Fellow
Respiratory Institute
Cleveland Clinic
Cleveland
OH
利益声明
MS declares that he has no competing interests.
Gustavo A. Heresi, MD, MS
Director, Pulmonary Vascular and CTEPH Program
Department of Pulmonary and Critical Care Medicine
Respiratory Institute
Cleveland Clinic
Cleveland
OH
利益声明
GAH has received speaking and advisory board fees from Bayer Healthcare, the manufacturer of riociguat.
鸣谢
Dr Muhammad Sameed and Dr Gustavo A. Heresi would like to gratefully acknowledge Dr Raed A. Dweik, a previous contributor to this topic.
利益声明
RAD declares that he has no competing interests.
同行评议者
Michael J. Krowka, MD
Division of Pulmonary and Critical Care Medicine
Mayo Clinic College of Medicine
Rochester
MN
利益声明
MJK declares that he has no competing interests.
Charlie Elliot, MB ChB, MRCP
Consultant Physician in Respiratory and General Internal Medicine
Sheffield Pulmonary Vascular Disease Unit
Royal Hallamshire Hospital
Sheffield
UK
利益声明
CE has received reimbursement for attending several conferences as well as lecture fees from Actelion Pharmaceuticals, GSK, and Bayer.
Maria Giovanna Triveri, MD, PhD
Assistant Professor of Cardiology
Medical Director of the Pulmonary Hypertension Program
Icahn School of Medicine at Mount Sinai Hospital
New York
NY
利益声明
MGT has participated on Advisory Boards of Bayer and Actelion and received financial compensation for attendance.
鉴别诊断
- 与左侧心脏疾病（肺静脉高压）有关的肺动脉高压（PAH）
- 与肺呼吸性疾病和/或缺氧有关的 PAH
- 慢性血栓和/或栓塞性疾病所致的 PAH
更多 鉴别诊断指南
- 2022 年ESC/ERS 肺动脉高血压诊断和治疗指南
- ACR 适宜性标准：疑似肺动脉高压
更多 指南
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