Quezada Loaiza Carlos Andrés, Velázquez Martín María Teresa, Jiménez López-Guarch Carmen, Ruiz Cano María José, Navas Tejedor Paula, Carreira Patricia Esmeralda, Flox Camacho Ángela, de Pablo Gafas Alicia, Delgado Jiménez Juan Francisco, Gómez Sánchez Miguel Ángel, Escribano Subías Pilar
Unidad Multidisciplinar de Hipertensión Pulmonar, Servicio de Neumología, Hospital Universitario 12 de Octubre, Madrid, Spain.
Unidad Multidisciplinar de Hipertensión Pulmonar, Servicio de Cardiología, Hospital Universitario 12 de Octubre, Madrid, Spain.
Rev Esp Cardiol (Engl Ed). 2017 Nov;70(11):915-923. doi: 10.1016/j.rec.2016.12.044. Epub 2017 Apr 26.
Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary vascular resistance, right ventricular dysfunction and death. Despite scientific advances, is still associated with high morbidity and mortality. The aim is to describe the clinical approach and determine the prognostic factors of patients with PAH treated in a national reference center over 30 years.
Three hundred and seventy nine consecutive patients with PAH (January 1984 to December 2014) were studied. Were divided into 3 periods of time: before 2004, 2004-2009 and 2010-2014. Prognostic factors (multivariate analysis) were analyzed for clinical deterioration.
Median age was 44 years (68.6% women), functional class III-IV: 72%. An increase was observed in more complex etiologies in the last period of time: Pulmonary venooclusive disease and portopulmonary hypertension. Upfront combination therapy significantly increased (5% before 2004 vs 27% after 2010; P < .05). Multivariate analysis showed prognostic significance in age, sex, etiology and combined clinical variables as they are independent predictors of clinical deterioration (P < .05). Survival free from death or transplantation for the 1st, 3rd and 5th year was 92.2%, 80.6% and 68.5% respectively. The median survival was 9 years (95% confidence interval, 7.532-11.959) CONCLUSIONS: The PAH is a heterogeneous and complex disease, the median survival free from death or transplantation in our series is 9 years after diagnosis. The structure of a multidisciplinary unit PAH must adapt quickly to changes that occur over time incorporating new diagnostic and therapeutic techniques.
肺动脉高压（PAH）的特征为肺血管阻力增加、右心室功能障碍及死亡。尽管有科学进展，但它仍与高发病率和死亡率相关。目的是描述临床治疗方法并确定在一家国家参考中心接受治疗30年的PAH患者的预后因素。
对379例连续的PAH患者（1984年1月至2014年12月）进行研究。将其分为3个时间段：2004年之前、2004 - 2009年和2010 - 2014年。对预后因素（多变量分析）进行临床恶化分析。
中位年龄为44岁（68.6%为女性），功能分级III - IV级：72%。在最后一个时间段观察到更复杂病因有所增加：肺静脉闭塞性疾病和门脉性肺动脉高压。初始联合治疗显著增加（2004年之前为5%，2010年之后为27%；P <.05）。多变量分析显示年龄、性别、病因及综合临床变量具有预后意义，因为它们是临床恶化的独立预测因素（P <.05）。第1、3和5年无死亡或移植的生存率分别为92.
