Progress in the diagnosis and management of pulmonary hypertension in children.
Progress in the diagnosis and management of pulmonary hypertension in children.
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DOI：
10.1097/mop.0000000000000135
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发表时间：
2014-10
影响因子：
3.6
通讯作者：
Ivy D
中科院分区：
文献类型：
--
作者：
Nicolarsen J;Ivy D
肺动脉高压（PH）是一种复杂的疾病，不仅仅是肺动脉血压升高和右心室功能障碍。其多种病因和不断扩大的诊断工具和治疗方法使其成为一种异质性疾病，具有广泛的临床后遗症。仍然有许多未解答的问题挑战着我们对这种疾病的理解。儿科患者PH的研究与以往一样强大，在最新的世界卫生组织分类系统中创建并纳入了儿科特定疾病特征，提高了对支气管肺发育不良等儿科疾病PH病理生理学的理解，并日益扩大诊断工具和管理可能性。虽然在儿童中使用PH疗法以前经常依赖于专家意见和涉及成人的研究的推论，但针对儿科的研究正在得到更广泛的支持和追求，甚至最近也引起了争论，这至少刺激了进一步的合作和讨论。本综述将重点介绍PH分类系统的变化，简要探讨支气管肺发育不良中的PH，并提供该领域专家使用的诊断和管理工具的更新。
Pulmonary hypertension (PH) is a complex disease that extends beyond merely elevated pulmonary blood pressures and right ventricular dysfunction. Its multiple etiologies and ever-expanding diagnostic tools and therapeutic approaches make it a heterogeneous disease with widely variable clinical sequelae. There are still many unanswered questions that challenge our understanding of this disease. The study of PH in the pediatric patient is as robust as ever, with the creation and inclusion of pediatric-specific disease characteristics in the most recent World Health Organization classification system, improved understanding of the pathophysiology of PH in pediatric diseases like bronchopulmonary dysplasia, and increasingly expanding diagnostic tools and management possibilities. While the use of PH therapies in children previously often relied on expert opinion and inferences from studies involving adults, pediatric-targeted research is becoming more widely supported and pursued, and even has come under recent debate, which at the very least stimulates further collaboration and discussion. This review will highlight the changes in the PH classification system, briefly explore PH in bronchopulmonary dysplasia, and provide updates on the diagnostic and management tools used by experts in the field.