Mouratoglou Sophia Anastasia, Arvanitaki Alexandra, Papadopoulos George, Souza Rogerio, Giannakoulas George
3rd Department of Cardiology, Hippokrateio General Hospital of Thessaloniki, Aristotle University of Thessaloniki, Greece.
2nd Department of Cardiology, Hippokrateio General Hospital of Thessaloniki, Aristotle University of Thessaloniki, Greece.
Int J Cardiol Congenit Heart Dis. 2025 May 27;21:100594. doi: 10.1016/j.ijcchd.2025.100594. eCollection 2025 Sep.
Pulmonary arterial hypertension (PAH) is a progressive and life-threatening condition characterized by elevated pulmonary vascular resistance, ultimately leading to right heart failure and death. Advances in the understanding of PAH pathophysiology have driven the development of targeted therapies that improve functional capacity, quality of life and survival. The current treatment paradigm emphasizes early and aggressive therapy, with combination therapy now considered standard of care, offering superior outcomes compared to monotherapy. Herewith, we examine the underlying pathophysiological mechanisms of PAH and explore the modes of action and clinical efficacy of major therapeutic classes, providing a comprehensive overview of contemporary treatment strategies.
肺动脉高压（PAH）是一种进行性且危及生命的疾病，其特征为肺血管阻力升高，最终导致右心衰竭和死亡。对PAH病理生理学认识的进展推动了靶向治疗的发展，这些治疗可改善功能能力、生活质量和生存率。当前的治疗模式强调早期积极治疗，联合治疗现被视为标准治疗方案，与单一疗法相比疗效更佳。在此，我们研究PAH的潜在病理生理机制，探讨主要治疗类别药物的作用方式和临床疗效，全面概述当代治疗策略。
