Introduction - Professor Richard Kirk 2023

Professor Richard Kirk
MA FRCP FRCPCH
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    Introduction
    Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (mPAP) > 20 mmHg in children > 3 months age, at sea level. The etiology includes cardiac, pulmonary, connective tissue, pulmonary veno-occlusive  diseases. Pulmonary arterial hypertension (PAH) is thought to result from an imbalance in the amount and ratio of vasoactive substances including prostacyclin, nitric  oxide, and endothelin-1. In many instances the triggers for PAH are unknown but there is a subgroup with identified genetic abnormalities which may be inherited.

    These webpages have relied heavily on the 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary  hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT, Co-chairs Georg Hansmann & Martin Koestenberger. Another excellent resource is a special edition of Cardiovascular Diagnosis and Therapy 2021 mainly devoted to all aspects of PAH.
    Further Reading
    Last Updated: April 2023
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