Key result
This review outlines the updated 2018 Nice Pulmonary Hypertension Classification, discusses the genetic basis and clinical presentation of pediatric pulmonary arterial hypertension, and highlights the challenges in managing pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis.
The 6th WSPH updated the definition of PH to mPAP > 20 mmHg, and genetic profiling in pediatric PAH is highlighted as crucial since mutations like BMPR2 are associated with significantly poorer survival requiring earlier aggressive therapy.
May warrant genetic profiling in pediatric PAH; leaves open effective PVOD/PCH management strategies.
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Ayako Nagai (2019) conducted a review in Pediatric Pulmonary Hypertension. This review outlines the updated 2018 Nice Pulmonary Hypertension Classification, discusses the genetic basis and clinical presentation of pediatric pulmonary arterial hypertension, and highlights the challenges in managing pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis.
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