Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA.
Am J Respir Crit Care Med. 2013 Aug 1;188(3):365-9. doi: 10.1164/rccm.201209-1640OC.
The causes and circumstances surrounding death are understudied in patients with pulmonary arterial hypertension (PAH).
We sought to determine the specific reasons and characteristics surrounding the death of patients with PAH.
All deaths of patients with pulmonary hypertension (PH) followed in the Cleveland Clinic Pulmonary Vascular Program were prospectively reviewed by the PH team. A total of 84 patients with PAH (age 58 ± 14 yr; 73% females) who died between June 2008 and May 2012 were included.
PH was determined to be the direct cause of death (right heart failure or sudden death) in 37 (44%) patients; PH contributed to but did not directly cause death in 37 (44%) patients; and the death was not related to PH in the remaining cases (n = 7; 8.3%). In three (3.6%) patients the final cause of death could not be adequately assessed. Most patients died in a healthcare environment and most received PH-specific therapies. In our cohort, 50% of all patients with PAH and 75.7% of those who died of right heart failure received parenteral prostanoid therapy. Less than half of patients had advanced healthcare directives.
Most patients with PAH in our cohort died of their disease; however, right ventricular failure or sudden death was the sole cause of death in less than half of patients.
肺动脉高压（PAH）患者的死亡原因和情况研究不足。
我们旨在确定 PAH 患者死亡的具体原因和特征。
克利夫兰诊所肺血管计划中所有接受肺动脉高压（PH）治疗的患者死亡均由 PH 团队前瞻性审查。共纳入 84 例 2008 年 6 月至 2012 年 5 月期间死于 PAH（年龄 58±14 岁；73%为女性）的患者。
PH 被确定为 37 例（44%）患者直接死亡原因（右心衰竭或心源性猝死）；PH 导致但未直接导致 37 例（44%）患者死亡；其余病例（n=7；8.3%）与 PH 无关。在 3 例（3.6%）患者中，最终死因无法充分评估。大多数患者在医疗环境中死亡，大多数患者接受了 PH 特异性治疗。在我们的队列中，50%的 PAH 患者和 75.7%死于右心衰竭的患者接受了肠外前列环素治疗。不到一半的患者有先进的医疗保健指令。
我们队列中的大多数 PAH 患者死于疾病，但不到一半的患者右心室衰竭或心源性猝死是唯一的死亡原因。
