Referral of Patients With Pulmonary Hypertension Diagnoses to Tertiary Pulmonary Hypertension Centers The Multicenter RePHerral Study
Referral of Patients With Pulmonary Hypertension Diagnoses to Tertiary Pulmonary Hypertension Centers The Multicenter RePHerral Study
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DOI：
10.1001/jamainternmed.2013.319
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发表时间：
2013-05-27
影响因子：
39
通讯作者：
Gomberg-Maitland, Mardi
中科院分区：
文献类型：
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作者：
Deano, Roderick C.;Glassner-Kolmin, Cherylanne;Gomberg-Maitland, Mardi
重要性：肺动脉高压（PH）是一种致命的疾病。虽然肺动脉高压（PAH）的预后有所改善，靶向治疗，结果是依赖于早期检测和准确diagnosis.Objective：为了确定PH诊断的准确性，在患者转诊到PH centers和频率的PAH特异性药物使用，尽管一个不确定或不正确的diagnosis.Design：多中心，描述性，横断面研究。在2010年和2011年的10个月期间，收集了新转诊患者的数据，并输入到一个安全的互联网数据库中。设置：三个大型三级PH中心。参与者：邀请140名新转诊到PH中心的连续患者参加，所有患者都同意这样做。结果：在平均年龄为56岁的140名患者中，95名（68%）由心脏病专家或肺病专家转诊，86名（61%）患有世界卫生组织功能III级或IV级疾病。五十六例转诊前诊断（40%）为PAH，42例（30%）未知，22例（16%）继发于肺部疾病或缺氧的PH。在转诊前接受明确诊断的98例患者中，32例（33%）被误诊。59例患者在三级中心首次接受了心脏右侧和/或左侧导管插入术。在38名仅接受右侧导管插入术的患者中，14名（37%）在接受手术后得到不同的诊断;在21名接受双侧心脏导管插入术的患者中，11名（52%）在接受手术后得到不同的诊断。42例患者（30%）已开始接受PAH特异性药物治疗前转介，与24个处方（57%）违反出版guidelines. Conclusion和相关性：患者转诊到PH中心的诊断和治疗往往是指晚（功能III类或IV类疾病），接受误诊，并不适当的处方药物。需要对教育工作进行重新评估，以提高诊断为PH的患者的认识、护理和结局。
Importance: Pulmonary hypertension (PH) is a fatal disease. Although the prognosis of pulmonary arterial hypertension (PAH) has improved with targeted therapies, the outcome is dependent on early detection and an accurate diagnosis.Objective: To determine the accuracy of PH diagnoses in patients referred to PH centers and the frequency of PAH-specific medication use despite an uncertain or incorrect diagnosis.Design: Multicenter, descriptive, cross-sectional study. During a 10-month period in 2010 and 2011, data on newly referred patients were collected and entered into a secure Internet database.Setting: Three large tertiary PH centers.Participants: One hundred forty consecutive patients newly referred to PH centers were invited to participate, and all consented to do so.Results: Of 140 patients referred with a mean age of 56 years, 95 (68%) were referred by cardiologists or pulmonologists and 86 (61%) had disease classified as World Health Organization functional class III or IV. Fifty-six of the prereferral diagnoses (40%) were PAH, 42 (30%) unknown, and 22 (16%) PH secondary to lung disease or hypoxia. Of the 98 patients who received a definitive diagnosis before referral, 32 (33%) received a misdiagnosis. Fifty-nine patients underwent catheterization of the right and/or left side of the heart for the first time at the tertiary center. Of the 38 patients who underwent catheterization of the right side alone, 14 (37%) received a different diagnosis after undergoing the procedure; of the 21 patients who underwent catheterization of both sides of the heart, 11 (52%) received a different diagnosis after undergoing the procedures. Forty-two patients (30%) had started receiving PAH-specific medications before referral, with 24 of the prescriptions (57%) contrary to published guidelines.Conclusions and Relevance: Patients referred to PH centers for diagnosis and treatment are often referred late (with functional class III or IV disease), receive misdiagnoses, and are inappropriately prescribed medications. A reevaluation of educational efforts is required to improve awareness and the care and outcome of patients diagnosed as having PH.