Nelson Textbook of Pediatrics 22nd Edition | Idiopathic/familial pulmonary arterial hypertension. BMPR2 mutation, Eisenmenger syndrome, PPHN. Diagnosis: echocardiography, cardiac catheterization. Treatment: CCBs, prostacyclin (epoprostenol), endothelin antagonists (bosentan), PDE-5 inhibitors (sildenafil), iNO, lung transplantation.
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