Simonca Larisa, Tulloh Robert
Department of Congenital Heart Disease, Bristol Royal Hospital for Children, Bristol BS2 8BJ, UK.
Children (Basel). 2017 Jul 24;4(7):60. doi: 10.3390/children4070060.
Pulmonary arterial hypertension (PAH) management has been transformed in recent times with the advent of cheap and effective diagnostic tools and therapy. Sildenafil, a phosphodiesterase-V inhibitor, has been at the centre of this treatment, and its success in treating PAH has led to its widespread uptake in adult and paediatric pulmonary hypertension (PH), as a first line treatment choice. This might apply to persistent pulmonary hypertension of the newborn (PPHN) or bronchopulmonary dysplasia, as well as to more complex diseases, such as idiopathic pulmonary hypertension. Although recent data regarding long-term mortality and the repeal of Food and Drug Administration (FDA) approval has complicated the issue, Sildenafil continues to be the major treatment option for paediatric PH for patients in a variety of contexts, and this does not seem likely to change in the foreseeable future. In this review, we provide a summary of pulmonary hypertension in infants and children and the use of Sildenafil for such diseases.
近年来,随着廉价且有效的诊断工具和治疗方法的出现,肺动脉高压(PAH)的管理发生了变革。西地那非,一种磷酸二酯酶-5抑制剂,一直是这种治疗的核心药物,它在治疗PAH方面的成功促使其在成人和儿童肺动脉高压(PH)中作为一线治疗选择被广泛应用。这可能适用于新生儿持续性肺动脉高压(PPHN)或支气管肺发育不良,以及更复杂的疾病,如特发性肺动脉高压。尽管最近有关长期死亡率的数据以及美国食品药品监督管理局(FDA)批准的撤销使问题变得复杂,但西地那非在各种情况下仍然是儿童PH患者的主要治疗选择,并且在可预见的未来似乎不太可能改变。在这篇综述中,我们总结了婴幼儿肺动脉高压以及西地那非在这些疾病中的应用。