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Prostacyclins and pulmonary arterial hypertension in children

Y Wu¹, H-M Liu, L Gu

¹Mianyang Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Mianyang, Sichuan, China. liuhm@scu.edu.cn.

European Review for Medical and Pharmacological Sciences

|January 20, 2022

Insights

Prostacyclins are crucial for treating pediatric pulmonary hypertension, with specific analogs like epoprostenol, iloprost, treprostinil, and beraprost showing varied efficacy and potential benefits in children.

Area of Science:

Pediatric Cardiology
Pulmonology
Pharmacology

Background:

Pediatric pulmonary hypertension (PPH) has low incidence but high mortality and limited treatment options.
Prostacyclin is a primary treatment for PPH, necessitating a review of its analogs' effectiveness.
Current treatments for PPH are often ineffective, highlighting the need for comprehensive therapeutic strategies.

Purpose of the Study:

To comprehensively review the advantages and disadvantages of prostacyclin and its analogs in treating pediatric pulmonary hypertension.
To synthesize current evidence on the efficacy and safety of various prostacyclin agents in children.
To provide an overview of therapeutic options for PPH.

Main Methods:

A systematic literature search was conducted in PubMed using keywords related to prostacyclins and pediatric pulmonary arterial hypertension.
The search covered studies published from December 1983 to May 2021.
A total of 238 articles were retrieved, screened for relevance, and included if published in English.

Main Results:

Epoprostenol demonstrated effectiveness in severe pediatric pulmonary hypertension.
Iloprost showed efficacy in severe persistent PPH in newborns and is suitable for inhaled use in vasoreactivity testing.
Treprostinil, a long-acting analog, exhibited the highest antiproliferative activity.
Beraprost showed potential in premature infants, but evidence is limited to a single case.

Conclusions:

Specific prostacyclin analogs offer distinct therapeutic benefits for pediatric pulmonary hypertension.
Epoprostenol and iloprost are established treatments for severe PPH, while treprostinil presents a long-acting option.
Further clinical research is required to confirm the efficacy of beraprost in premature infants and other pediatric populations.

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Abstract

Objective:

Despite its low incidence, pulmonary hypertension in children places a substantial burden on families and society because survival can be shorter than 10 months and treatment options are limited and ineffective. Drugs to treat pulmonary hypertension include endothelin antagonists, phosphodiesterase type 5 inhibitors and prostacyclin, which is the most widely used to treat pediatric pulmonary hypertension. The main aim of this study was to provide a comprehensive overview of the advantages and disadvantages of prostacyclin and its analogs for treating pulmonary hypertension in children.

Materials And Methods:

To retrieve a thorough collection of studies, we performed a search in PubMed using the following combination of keywords: (Prostacyclins) or (Epoprostenol) or (Iloprost) or (Treprostinil) or (Beraprost), (children) and (pulmonary arterial hypertension). The time limits used for the search were December 1983 to May 2021.

Results:

The search retrieved a total of 238 articles. Titles and abstracts of articles were screened for relevance, and all relevant articles published in English were included.

Conclusions:

Epoprostenol can be effective against severe pulmonary hypertension. Iloprost can treat severe persistent pulmonary hypertension in newborns and inhaled iloprost can be used in pulmonary vasoreactivity testing. Treprostinil is a long-acting prostacyclin analog, and it shows the highest antiproliferative activity among prostacyclins. Beraprost may be effective in premature infants, but available evidence comes from only one patient, so more clinical testing is needed.

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