Department of Pediatric, King Fahd Armed Forces Hospital, Jeddah, Saudi Arabia.
Department of Pediatric, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
Ann Thorac Med. 2014 Jul;9(Suppl 1):S113-20. doi: 10.4103/1817-1737.134053.
Pulmonary hypertension (PH) is relatively uncommon in children. Pulmonary arterial hypertension (PAH) in pediatric comprises a wide spectrum of diseases, from a transient neonatal condition to a progressive disease associated with morbidity and mortality. Most common PAH in pediatric are idiopathic (IPAH) or PAH associated with congenital heart disease (PAH-CHD), while other associated conditions, such as connective tissue disease (CTD), are less common in pediatrics. Despite better understanding of PH and the availability of new medications during recent decades; the diagnosis, investigation and choice of therapy remain a challenge in children, as evidence-based recommendations depend mainly on adult studies. In this review, we provide a detailed discussion about the distinctive features of PAH in pediatric, mainly emphacysing on classification and diagnostic algorithm.
肺动脉高压(PH)在儿童中相对少见。儿科肺动脉高压(PAH)包括广泛的疾病谱,从短暂的新生儿期疾病到与发病率和死亡率相关的进行性疾病。儿科最常见的 PAH 是特发性(IPAH)或与先天性心脏病(PAH-CHD)相关的 PAH,而其他相关疾病,如结缔组织疾病(CTD),在儿科中则较少见。尽管近年来对 PH 的认识有所提高,并且出现了新的药物,但儿童的诊断、检查和治疗选择仍然是一个挑战,因为基于证据的建议主要依赖于成人研究。在这篇综述中,我们详细讨论了儿科 PAH 的独特特征,主要强调了分类和诊断算法。