Causes and Circumstances of Death in Pulmonary Arterial Hypertension
Causes and Circumstances of Death in Pulmonary Arterial Hypertension
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DOI：
10.1164/rccm.201209-1640oc
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发表时间：
2013-08-01
影响因子：
24.7
通讯作者：
Dweik, Raed A.
中科院分区：
文献类型：
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作者：
Tonelli, Adriano R.;Arelli, Vineesha;Dweik, Raed A.
基本原理：肺动脉高压（PAH）患者死亡的原因和情况尚未得到充分研究。Objectives：我们试图确定PAH患者死亡的具体原因和特征。Methods：PH团队前瞻性回顾了克利夫兰临床肺血管计划中肺动脉高压（PH）患者的所有死亡。共84例PAH患者研究对象为2008年6月至2012年5月期间死亡的100名儿童（年龄58 ± 14岁; 73%为女性）。（右心衰竭或猝死）37例（44%）; PH促成但未直接导致死亡37例（44%）;其余7例（8.3%）死亡与PH无关。3例（3.6%）患者的最终死亡原因无法充分评估。大多数患者在医疗环境中死亡，大多数接受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腺素类治疗。不到一半的患者有先进的healthcare directives.Conclusions：在我们的队列中，大多数PAH患者死于他们的疾病，但是，右心室衰竭或猝死是唯一的死亡原因，在不到一半的患者。
Rationale: The causes and circumstances surrounding death are understudied in patients with pulmonary arterial hypertension (PAH).Objectives: We sought to determine the specific reasons and characteristics surrounding the death of patients with PAH.Methods: 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.Measurements and Main Results: 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.Conclusions: 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.