UNASSIGNED: @entity215 ( @entity215 ) with @entity1146 ( @entity1146 ) and multiple aortopulmonary collaterals (MAPCAs) is a rare and severe form of @entity299 with poor prognosis. Aortopulmonary collaterals expose pulmonary arterioles to systemic pressure resulting in @entity120 ( @entity120 ). To date, reports regarding the role of @entity120 medications in this population are sparse. The objective of this study was to assess the effect of @entity120 medications in @entity1 with @entity215 , @entity1146 and MAPCAs or similar anatomy, with emphasis on symptoms, echocardiography and invasive hemodynamics. A retrospective review was performed for @entity1 at a single tertiary care pediatric center. Twelve of 66 @entity1 were treated with @entity120 medications (18  %), and eight of these @entity1 had adequate follow-up for further analysis. Median age at last follow-up was 6  years (range 1.4-21  years). Median length of therapy with @entity120 medication was 4  years (range 0.3-17  years). @entity120 medications included @entity7855 , @entity12584 , @entity15940 , inhaled @entity13992 and @entity4770 infusion. @entity120 therapy was associated with improvement in symptoms in all @entity1 and improvement in @entity120 by hemodynamic measures in the majority of @entity1 . All @entity1 underwent at least one cardiac intervention by catheterization or surgery while taking @entity120 medication. Two @entity1 died from non- @entity120 -related causes. The remaining six @entity1 are alive and remain on @entity120 medication. This review indicates that @entity120 medications are well tolerated by this @entity1 group and provide symptomatic improvement. Further studies are required to determine whether @entity120 medications provide long-term survival benefit for @entity1 with complex @entity299 .

Use of Pulmonary Hypertension Medications in @entity1 with @entity215 with XXXX and Multiple Aortopulmonary Collaterals.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity12584 :: ('MESH:C086232', 'Chemical') :: ['bosentan'] @entity215 :: ('MESH:D013771', 'Disease') :: ['TOF', 'Tetralogy of Fallot'] @entity120 :: ('MESH:D006976', 'Disease') :: ['pulmonary hypertension', 'PH'] @entity7855 :: ('MESH:C101426', 'Chemical') :: ['sildenafil'] @entity4770 :: ('MESH:D011464', 'Chemical') :: ['prostacyclin'] @entity13992 :: ('MESH:C427248', 'Chemical') :: ['treprostinil'] @entity299 :: ('MESH:D006331', 'Disease') :: ['congenital heart disease'] @entity1146 :: ('MESH:D018633', 'Disease') :: ['pulmonary atresia', 'PA'] @entity15940 :: ('MESH:C467894', 'Chemical') :: ['ambrisentan']
@entity1146 :: (MESH:D018633,Disease) :: ['Pulmonary Atresia']
