2023年台湾心脏病学会(TSOC)和台湾风湿病学院(TCR)联合共识：结缔组织病相关性肺动脉高压

Acta Cardiol Sin 2023;39:213-241Consensusdoi:10.6515/AC5.202303_39(2).20230117A2023 Taiwan Society of Cardiology (TSOC)andTaiwan College of Rheumatology (TCR)JointConsensus on Connective Tissue Disease-AssociatedPulmonary Arterial HypertensionWei-Chun Huang,3 Song-Chou Hsieh,Yen-Wen Wu,25.5.Tsu-Yi Hsieh,Yh-Jer Wu,0 Ko-Jen Li,1Min-Ji Chamg,We-Sheng ChenShih-Hsien Sung,Yen-Po Tsao Wan-Jing HoChien-ChihChin-Chang Cheng,Hung-Cheng Tsai,25 Chih-Hsin Hsu,2 Cheng-Hsun Lu,2 Yu-Wei Chiu,25 Chieh-Yu ShenChun-Hsien Wu,Feng-Cheng Liu Yen-Hung Lin,Fu-Chiang Yeh,Wei-Shin Liu,Hui-Ting Lee,Shu-Hao Wu,0 Chi-Ching Chang,3535 Chun-Yuan Chu,3 Charles Jia-Yin Hou.40 and Chang-Youh TsaBackground:Pulmonary arterial hypertension (PAH),defined as the presence of a mean pulmonary artery pressure>20 mmHg,pulmonary artery wedge pressure s 15 mmHg,and pulmonary vascular resistance (PVR)>2 Wood unitsbased on expert consensus,is characterizedby aprogressiveand sustained increase in PVR,which may lead to rightheart failure and death.PAH is a well-known complication of connective tissue diseases(CTDs),such as systemicsclerosis,systemic lupus erythematosus,Sjogren's syndrome,and other autoimmune conditions.In the past fewyears,tremendous progress in the understanding of PAH pathogenesis has been made,with various novel diagnosticand screening methods for the early detection of PAH proposed worldwide.●Received:December 21,2022 Accepted:January17,2023Department of Critical Care Medicine,Kaohsiung Veterans General Hospital,Kaohsiung:'School of Medicine,National Yang-Ming Chiao-Tung University,Taipei;Department of Physical Therapy,Fooyin University,Kaohsiung:Division of Allergy,Immunology,and Rheumatology.Department of Internal Medicine,National Taiwan University Hospital,Taipei;Division of Cardiology,Cardiovascular Medical Center,FarEastern Memorial Hospital,New Taipei City:Graduate Institute of Medicine,Yuan Ze University,Taoyuan;'Attending Physician of Division ofAllergy-Immunology-Rheumatology,Department of Inter nal Medicine;Director of Division of Clinical Training,Department of MedicalEducation,Taichung Veterans General Hospital;Program of Business,College of Business,Feng Chia University,Taichung;CardiovascularCenter,Department of Internal Medicine,MacKay Memorial Hospital,Taipei;Department of Medicine,MacKay Medical College,New TaipeiCity;Department of Internal Medicine,National Taiwan University Hospital:National Taiwan University,College of Medicne;Division ofCardiology:Division of Allergy,Immunology and Rheumatology;Department of Medicine,Taipei Veterans General Hospital;Institute ofEmergency and Critical Care Medicine;Institutes of Clinical Medicine,National Yang Ming Chiao Tung University,Taipei;Department ofCardiology,Chang Gung Memorial Hospital;College of Medicine,Chang Gung University,Taoyuan;"Department of Internal MedicinePingtung Veteran General Hospital,Pingtung:2Department of Internal Medicine,National Cheng Kung University Hospital,College ofMedicine,National