Dear Colleagues,

In the past couple of decades, the management of pulmonary hypertension (PH) has significantly evolved, especially in the field of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Briefly, early diagnosis, PH-specific therapies, and initial combination therapy for PAH has improved prognosis, as well as multimodal imaging, invasive intervention such as pulmonary endarterectomy and balloon pulmonary angioplasty, and PH-specific therapies for CTEPH. However, there are still significant issues to be clarified. Although right ventricular function is considered to be an important prognostic predictor of PH, its adaptation for increased afterload varies among patients and underlying diseases. The treatment algorithm for patients with idiopathic, heritable, drug-associated, and connective tissue-disease-associated PAH in the latest ESC/ERS PH guidelines differentiates initial combination therapy and monotherapy according to the presence of cardiopulmonary comorbidities. PH-specific therapies for patients with cardiopulmonary comorbidities, called “atypical PAH”, are sometimes challenging because of increased adverse effects and limited efficacy. Finally, the management of PH associated with lung diseases and/or hypoxia (Group 3) is also challenging, including which patients we need to treat with PH-specific therapies and which drugs we need to select. In this Special Issue, we would mainly like to highlight those unsolved issues in the management of PH.

Dr. Shigefumi Fukui
Guest Editor

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• pulmonary arterial hypertension
• chronic thromboembolic pulmonary hypertension
• multimodal imaging
• pulmonary endarterectomy
• balloon pulmonary angioplasty