2023 Taiwan Society of Cardiology (TSOC) and Taiwan College of Rheumatology (TCR) Joint Consensus on Connective Tissue Disease-Associated Pulmonary Arterial Hypertension

Wei Chun Huang, Song Chou Hsieh, Yen Wen Wu, Tsu Yi Hsieh, Yih Jer Wu, Ko Jen Li, Min Ji Charng, Wei Sheng Chen, Shih Hsien Sung, Yen Po Tsao, Wan Jing Ho, Chien Chih Lai, Chin Chang Cheng, Hung Cheng Tsai, Chih Hsin Hsu, Cheng Hsun Lu, Yu Wei Chiu, Chieh Yu Shen, Chun Hsien Wu, Feng Cheng LiuYen Hung Lin, Fu Chiang Yeh, Wei Shin Liu, Hui Ting Lee, Shu Hao Wu, Chi Ching Chang, Chun Yuan Chu, Charles Jia Yin Hou, Chang Youh Tsai

Research output: Contribution to journalArticlepeer-review

3 Citations (Scopus)

Abstract

Background: Pulmonary arterial hypertension (PAH), defined as the presence of a mean pulmonary artery pressure > 20 mmHg, pulmonary artery wedge pressure ≤ 15 mmHg, and pulmonary vascular resistance (PVR) > 2 Wood units based on expert consensus, is characterized by a progressive and sustained increase in PVR, which may lead to right heart failure and death. PAH is a well-known complication of connective tissue diseases (CTDs), such as systemic sclerosis, systemic lupus erythematosus, Sjogren’s syndrome, and other autoimmune conditions. In the past few years, tremendous progress in the understanding of PAH pathogenesis has been made, with various novel diagnostic and screening methods for the early detection of PAH proposed worldwide. Objectives: This study aimed to obtain a comprehensive understanding and provide recommendations for the management of CTD-PAH in Taiwan, focusing on its clinical importance, prognosis, risk stratification, diagnostic and screening algorithm, and pharmacological treatment. Methods: The members of the Taiwan Society of Cardiology (TSOC) and Taiwan College of Rheumatology (TCR) reviewed the related literature thoroughly and integrated clinical trial evidence and real-world clinical experience for the development of this consensus. Conclusions: Early detection by regularly screening at-risk patients with incorporations of relevant autoantibodies and biomarkers may lead to better outcomes of CTD-PAH. This consensus proposed specific screening flowcharts for different types of CTDs, the risk assessment tools applicable to the clinical scenario in Taiwan, and a recommendation of medications in the management of CTD-PAH.

Original languageEnglish
Pages (from-to)213-241
Number of pages29
JournalActa Cardiologica Sinica
Volume39
Issue number2
DOIs
Publication statusPublished - 2023 Mar 1

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

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