Ask the Expert: Modalities Used to Help Prolong Survival in Patients With Advanced PAH

Advances in Pulmonary Hypertension

¹ 

关键词: Cardiology;    Pulmonology;    Psychosocial Issues;    Lifestyle Issues;    Social Work;    WHO Group 1- Pulmonary Arterial Hypertension;    Surgical Interventions;    PPHN;    Exercise;    HIV Infection;    Systolic dysfunction;    nitric oxide;    Inotropes;    Cardiac Index;    Fick;    Thermodilution;    Transplantation;    ECMO;    endothelial dysfunction;    dyspnea;    Functional Class;    chest pain;    Drug development;    clinical trial endpoints;    Classification;   

DOI  :  

学科分类：医学（综合）

来源: Pulmonary Hypertension Association

【 摘 要 】

Pulmonary arterial hypertension (PAH) is a relentless, progressive disease of the small and medium-sized pulmonary arteries that often culminates in development of right ventricular (RV) failure. Earlier detection and advances in pharmacotherapy have greatly enhanced quality of life and reduced clinical worsening, but a cure remains elusive, and many patients require lung transplantation. Even with close monitoring, the clinical course can be unpredictable and patients can present with pulmonary hypertensive crisis, characterized by profound RV failure leading to marked reductions in cardiac output with subsequent end organ hypoperfusion and systemic hypotension. Limited published data are available to guide decision making in this regard, and treatment focuses on optimization of volume status via diuretics and ultrafiltration, judicious use of inotropes, and the use of inhaled or intravenous pulmonary arterial vasodilators.

【 授权许可】

All Rights reserved