Key result
Recent advances in PAH-targeted drugs have improved survival, though pediatric management still largely depends on adult studies and expert clinical experience rather than pediatric evidence.
Why the study?
Pediatric pulmonary hypertension differs significantly from adult disease, yet its management still mainly depends on adult evidence-based studies and clinical experience.
Pediatric pulmonary hypertension management currently relies heavily on adult data, highlighting the need for pediatric-specific evidence-based studies.
Answers are grounded in this paper and cite their sources.
Pediatric PAH management warrants caution extrapolating adult data; leaves open need for dedicated pediatric trials.
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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F. Rana Olguntürk (2020) conducted a review in Pediatric pulmonary hypertension. PAH-targeted drugs and treatment strategies was evaluated. Recent advances in PAH-targeted drugs have improved survival, though pediatric management still largely depends on adult studies and expert clinical experience rather than pediatric evidence.
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