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Published on: May 11, 2015
An update on the diagnosis and treatment of pediatric pulmonary hypertension
1Professor of Pediatrics and Pediatric Cardiology, PhD in medical physiology, Former Head of Pediatrics and Pediatric Cardiology in Gazi University Faculty of Medicine. Founder of Pediatric Cardiology and PAH center in Gazi University. Former President of Turkish Association of Pediatric Cardiology and Surgery, Gazi University , Ankara, Turkiye.
Insights
Pediatric pulmonary arterial hypertension (PAH) management relies on adult data. New research is shifting pediatric PAH treatment toward evidence-based strategies, improving patient outcomes.
Area of Science:
- Pediatric Cardiology
- Pulmonary Medicine
- Pharmacology
Background:
- Pulmonary hypertension (PH) is a serious condition affecting pulmonary arterioles, with distinct pediatric presentations.
- Pediatric PH often stems from idiopathic causes or congenital heart disease.
- Current pediatric PH management largely relies on adult studies and expert experience.
Purpose of the Study:
- To review recent updates in pediatric pulmonary arterial hypertension (PAH) definition, classification, and diagnosis.
- To discuss current and prospective pediatric PAH therapies, including targets, actions, side effects, and dosages.
Main Methods:
- Literature review of recent advancements in pediatric PAH.
- Analysis of therapeutic targets, actions, side effects, and dosages for pediatric PAH treatments.
Main Results:
- Advances in treatment options have enhanced quality of life and survival for PAH patients.
- Adult PAH therapies require further investigation for pediatric efficacy and safety.
- Growing research activity is expected to drive evidence-based treatment recommendations for pediatric PH.
Conclusions:
- While a cure for PAH remains elusive, current treatments improve patient outcomes.
- Pediatric PAH treatment strategies are evolving from experience-based to evidence-based.
- Future research will refine therapeutic approaches for pediatric pulmonary hypertension.
Introduction:
Pulmonary hypertension (PH) is a heterogeneous disease that mainly affects the pulmonary arterioles, leading to significant morbidity and mortality. Pulmonary hypertension in children from birth to adolescence presents important differences from that of adults. The majority of pediatric pulmonary arterial hypertension (PAH) cases are idiopathic or associated with congenital heart disease. However, the management of pediatric PAH mainly depends on the results of evidence-based adult studies and the clinical experiences of pediatric experts.
Areas Covered:
This article briefly reviews the recent updates on the definition, classification, and diagnostic evaluation of pediatric PAH and their impact on treatment strategies. The main purpose of this review is to discuss the current pediatric therapies, as well as the prospective therapies, in terms of therapeutic targets, actions, side effects, and dosages.
Expert Opinion:
Although there is no cure for PAH, recent advances in the form of new treatment options have improved the quality of life and survival rates of PAH patients. PAH-targeted drugs and treatment strategies for adult PAH have not been sufficiently studied in children. However, the growing scientific activity in that field will surely change the treatment option recommendations in pediatric PH from experience-based to evidence-based in the near future.
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