Pulmonary hypertension (PHT) is a heterogeneous complex vascular disease that has intrigued vascular biologists and clinicians for decades. The need for collaborative efforts and research networks became apparent early on. An NIH-driven effort in 1981 resulted in the first registry of idiopathic pulmonary arterial hypertension (PAH), one of the most mysterious and deadly subtypes of PHT. Its findings defined the magnitude of the clinical problem and triggered an explosion of research efforts that have been steadily increasing since then. A few years earlier (1973), a WHO-sponsored international collaborative effort, first attempted to define the clinical phenotypes of PHT. Since this first WHO classification, several have followed producing a regularly updated classification system, based on standard clinical data. The hypothesis was that many PHT subtypes within each of the 5 WHO classes share a common pathogenesis (and thus may benefit from the same therapy), partly because they have similar basic lung pathology, particularly those in the WHO Class I PHT that included idiopathic PAH. The WHO classifications could not clearly define the phenotype of PHT subtypes, and the diagnosis of idiopathic PAH remained that of exclusion (ie, PHT that does not fit the diagnostic criteria of the other WHO classes). Article, see p 1136 The WHO classifications were very important in the community’s initial efforts to understand the clinical complexity of PHT, which was being diagnosed earlier and more accurately because of advances in imaging and the emergence of specialized PHT clinics. The weakness of the WHO classification-driven approach became apparent as preclinical research revealed a tremendous diversity of molecular mechanisms in animal models and human tissues (despite similarities in lung pathology) and clinical experience exposed many gray zones among PHT subtypes. Most of the clinical trials to date have been based on the WHO classification paradigm and produced drugs that …

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PVDOMICS将肺动脉高压领域带入精确医学时代

肺动脉高压（PHT）是一种异质复杂的血管疾病，数十年来一直困扰着血管生物学家和临床医生。在早期，对协作努力和研究网络的需求就很明显了。美国国家卫生研究院（NIH）于1981年做出的努力导致了特发性肺动脉高压（PAH）的首次注册，这是PHT最神秘和致命的亚型之一。它的发现确定了临床问题的严重性，并引发了自那时以来稳定增长的研究工作的爆炸式增长。几年前（1973年），世界卫生组织（WHO）发起的一项国际合作努力，首次尝试确定PHT的临床表型。自世界卫生组织首次分类以来，随后有几家根据标准临床数据产生了定期更新的分类系统。假设是，WHO的5个类别中的许多PHT亚型都具有共同的发病机制（因此可能会受益于相同的治疗方法），部分原因是它们具有相似的基本肺部病理学，尤其是WHO I类PHT中包括特发性PAH的那些。WHO的分类不能明确定义PHT亚型的表型，特发性PAH的诊断仍排除在外（即PHT不符合其他WHO分类标准的诊断）。文章，请参阅第1136页WHO的分类在社区初步了解PHT的临床复杂性方面非常重要，由于影像学的进步和专门PHT诊所的出现，对PHT的临床复杂性进行了更早，更准确的诊断。由于临床前研究表明动物模型和人体组织的分子机制差异极大（尽管肺病理学相似），并且临床经验暴露了PHT亚型中的许多灰色区域，因此WHO分类驱动方法的弱点变得显而易见。迄今为止，大多数临床试验都基于WHO分类范例，并产生了……