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Advances in targeted therapy for pulmonary arterial hypertension in children

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A CORRESPONDENCE to this article was published on 27 February 2023

Abstract

Pulmonary arterial hypertension (PAH) is a rare and devastating disease of the pulmonary vasculature with a high morbidity and mortality rate in infants and children. Currently, treatment approaches are mostly based on adult guidelines and pediatrician clinical experience, focusing on specific pulmonary antihypertensive therapy and conventional supportive care. The advent of targeted drugs has led to significant advances in the treatment of PAH in children, including endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and prostacyclins, which have been studied and proven to improve hemodynamics and functional class in children PAH. A new targeted drug, riociguat, is assessing its safety and efficacy in clinical trials. However, more randomized controlled studies are needed to evaluate the combination of drugs, treatment strategies, and clinical endpoints of targeted therapy in children PAH. In this review, we summarize the research advances of PAH-targeted therapy in children over the last decade in order to provide a theoretical basis for future studies.

Conclusion: Pulmonary arterial hypertension (PAH) is a rare and devastating pulmonary vascular disease that is associated with a variety of diseases of any age in childhood onset.

What is Known:

• Therapeutic strategies for targeted drugs for PAH in children are based almost exclusively on data from adult studies and clinical experience of pediatric specialists.

• Due to the complex etiology of PAH in children and the relative lack of clinical trial data, the selection of appropriate targeted drug therapy remains difficult.

What is New:

• We redefine the definition of pulmonary arterial hypertension in children and summarize the progress of targeted therapy of pulmonary arterial hypertension in children in the past ten years.

• The dosage and adverse reactions were summarized, and the mechanism of action was drawn according to the available targeted drugs. It can provide theoretical support for the development of guidelines and treatment strategies for the diagnosis and treatment of pulmonary arterial hypertension in children.

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Funding

This work was supported by the Research Projects of Gansu Provincial Hospital (20GSSY5-2, 21GSSYB-8), the Gansu Provincial Youth Science and Technology Fund (20JR10RA415), the Natural Science Foundation of Gansu Province (21JR11RA186), and the Guiding Plan for Scientific and Technological Development of Lanzhou (2019-ZD-102).

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Authors and Affiliations

  1. Department of Scientific Research, Gansu Provincial Hospital, 204 West Donggang R.D., Lanzhou, 730000, China

    Li Li, Xinyu Zhu, Xiaojie Chen, Jieyun Gao & Min Zhang

  2. Department of Pharmacy, Gansu Provincial Hospital, 204 West Donggang R.D., Lanzhou, 730000, China

    Chunchun Ding

  3. First Clinical College of Medicine, Lanzhou University, 1st West Donggang R.D., Lanzhou, 730000, China

    Shixun Ma

  4. General Surgery, Gansu Provincial Hospital, 204 West Donggang R.D., Lanzhou, 730000, China

    Shixun Ma

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Contributions

Shixun Ma and Min Zhang: study design, methodological advice, drafting, and revising the manuscript. Li Li: study design, collecting data, drafting, and revising the manuscript. Xinyu Zhu and Xiaojie Chen: collecting data, data entry, data cleaning and drafting the manuscript. Jieyun Gao and Chunchun Ding: interpreting of data and drafting and revising the manuscript. The author(s) read and approved the final manuscript.

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Communicated by Peter de Winter

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Li, L., Zhu, X., Chen, X. et al. Advances in targeted therapy for pulmonary arterial hypertension in children. Eur J Pediatr 182, 2067–2076 (2023). https://doi.org/10.1007/s00431-022-04750-y

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