Diagnosis and treatment of pediatric pulmonary arterial hypertension |
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Authors: | Nesrine Farhat Frederic Lador Maurice Beghetti |
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Affiliation: | 1. Pediatric Cardiology Unit, University Children’s Hospital of Geneva, Geneva, Switzerland;2. Pulmonary Hypertension Program, University Hospital of Geneva, Geneva, Switzerland;3. Service de Pneumologie, University Hospital of Geneva, Geneva, Switzerland;4. Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique, University of Geneva and Lausane Switzerland, Geneva, Switzerland |
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Abstract: | Introduction: Pediatric pulmonary arterial hypertension (PAH) remains a rare and severe disease with a poor prognosis. PAH may be idiopathic, heritable or associated with systemic conditions in particular associated with congenital heart disease. Areas covered: A thorough and extensive diagnostic approach is required for a correct diagnosis. The outcome has improved over the last decade with a better diagnostic approach and with the initiation of new targeted therapies. However, there is still significant progress to achieve as there is still no cure for this devastating disease. Expert opinion: Adapted clinical studies to define the best therapeutic approach are needed. Even if the treatment approach is still mainly derived from adult data and expert consensus, several studies and registries are currently underway and should deliver important information in the next future. This review aims to give an overview of the current diagnosis and treatment strategies of PAH. |
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Keywords: | Paediatric pulmonary arterial hypertension treatment diagnosis |
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