Jasińska-Stroschein Magdalena, Sztuka Katarzyna, Orszulak-Michalak Daria
Zakład Biofarmacji, Katedra Biofarmacji, Uniwersytet Medyczny, Łodź, Polska.
Wiad Lek. 2017;70(2 pt 2):386-394.
Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple clinical conditions characterizing with by an abnormal increase in mean pulmonary arterial pressure. It is a rare, debilitating disease with a poor prognosis. Despite significant progress in diagnosis and management, including disease-targeted therapies as well as development of specialized centres, PH remains a chronic disease without a cure. If untreated, it leads to right heart failure and premature death, and a multifactorial pathomechanism impacts negatively on further prognosis. Insufficient social awareness or non-specific initial symptoms accompany to delayed diagnosis and specialist treatment. In the following pages, we will review the currently classification, etiology as well as and diagnostic algorithms in PH. We discuss approved treatments, especially specific dug therapy for pulmonary arterial hypertension, and recently approved strategies for its refund. We also summarize the general measures for patients and their caregivers, as well as the role of support groups, and specialized centers in Poland.
肺动脉高压（PH）是一种病理生理紊乱，可能涉及多种临床情况，其特征是平均肺动脉压异常升高。它是一种罕见的、使人衰弱的疾病，预后较差。尽管在诊断和管理方面取得了重大进展，包括针对疾病的治疗以及专业中心的发展，但PH仍然是一种无法治愈的慢性疾病。如果不进行治疗，它会导致右心衰竭和过早死亡，并且多因素发病机制会对进一步的预后产生负面影响。社会意识不足或初始症状不具特异性会导致诊断和专科治疗延迟。在接下来的几页中，我们将回顾目前PH的分类、病因以及诊断算法。我们将讨论已获批的治疗方法，特别是针对肺动脉高压的特异性药物治疗，以及最近获批的报销策略。我们还总结了针对患者及其护理人员的一般措施，以及波兰支持小组和专业中心的作用。
