[Current issues in etiology, diagnostics and management in pulmonary hypertension].

ABSTRACT

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.