Use of pulmonary arterial hypertension-approved therapy in the treatment of non-group 1 pulmonary hypertension at US referral centers
Use of pulmonary arterial hypertension-approved therapy in the treatment of non-group 1 pulmonary hypertension at US referral centers
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DOI：
10.1086/681264
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发表时间：
2015-06-01
影响因子：
2.6
通讯作者：
Robbins, Ivan M.
中科院分区：
文献类型：
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作者：
Trammell, Aaron W.;Pugh, Meredith E.;Robbins, Ivan M.
肺动脉高压（PH）是左心疾病和肺实质疾病的常见并发症，预示着死亡率的增加。越来越多的药物被批准用于治疗世界卫生组织（WHO） 1组肺动脉高压（PAH）。然而，它们在其他病因的PH中没有得到很好的研究（WHO组2-5）。我们试图评估PAH转诊中心在这组不同患者中使用的治疗方法。我们开展了一项半定量在线调查，旨在评估美国肺血管疾病中心使用pah批准的治疗方法治疗非1组ph的情况。50个中心中有30个完成了调查。几乎所有中心（93%）都报道了非1组PH患者使用PAH治疗，包括77%的2组PH和80%的3组PH。经肺梯度升高或肺血管阻力升高以及右心室（RV）功能障碍的存在通常被认为是支持左心继发PH患者使用PAH治疗的原因。对于PH合并肺实质疾病的患者，肺功能损害程度和右心室功能障碍是影响PAH治疗使用的最重要因素。总之，肺血管疾病治疗中心对WHO 2-5组PH患者使用pah批准的治疗方法，主要依靠血流动力学和RV功能评估来确定治疗候选人。由于临床实践已经超出了这些病因的证据范围，因此需要设计临床试验来测试多环芳烃治疗左心肺疾病引起的PH的疗效。
Pulmonary hypertension (PH) is a frequent complication of left heart disease and parenchymal lung disease, and it portends increased mortality. A growing number of medications are approved for the treatment of World Health Organization (WHO) group 1 pulmonary arterial hypertension (PAH). However, they are not well studied in PH of other etiologies (WHO groups 2-5). We sought to assess treatment approaches used by PAH referral centers in this diverse group of patients. We developed a semiquantitative online survey designed to evaluate the use of PAH-approved therapy by pulmonary vascular disease centers in the United States for management of non-group 1 PH. Thirty of 50 centers completed the survey. Almost all centers (93%) reported using PAH therapy for patients with non-group 1 PH, including 77% with group 2 PH and 80% with group 3 PH. Elevated transpulmonary gradient or pulmonary vascular resistance and the presence of right ventricular (RV) dysfunction were commonly cited as supporting use of PAH therapy in patients with PH secondary to left heart disease. For patients with PH and concomitant parenchymal lung disease, degree of pulmonary function impairment and RV dysfunction were most important in influencing use of PAH therapy. In conclusion, pulmonary vascular disease treatment centers use PAH-approved therapy for patients with WHO group 2-5 PH, mostly relying on hemodynamics and assessment of RV function to identify candidates for therapy. Clinical trials designed to test the efficacy of PAH therapy in PH due to left heart and lung disease are needed, as clinical practice has extended beyond the evidence for these etiologies of PH.