摘要
Congenitalheartdisease(CHD)-associatedpulmonaryarterialhypertension(PAH)includesaheterogeneouspatientpopulationthatcanbecharacterizedbytheunderlyingcardiacmalformation.CHD-associatedPAHhasanestimatedprevalenceof5–10%inadultpatients,withanincreasingnumberofpatientssurvivingtoadulthoodbecauseofadvancesinthesurgicalmanagementandthedevelopmentofpulmonaryarterialhypertension(PAH)-targetedpharmacotherapy.Althoughlimiteddataexist,targetedPAHpharmacotherapyhasproventobebeneficialinpatientswithCHD-associatedPAH,withobservedimprovementinfunctionalclass,increaseinexercisecapacity,andimprovementinqualityoflifeandcardiopulmonaryhemodynamics.Additionally,therehasbeenincreasinginterestinthe“treat-to-close”strategy.PAH-targetedpharmacotherapymaybeusedtooptimizecardiopulmonaryhemodynamicssoastoimprovepatients’operabilityinrepairingthecardiacdefect.Althoughtherehavebeensignificantadvancesinthemanagementofthisdiseasestateinthepast2decades,mortalityremainshigh,andongoingclinicaltrialsareneededtobetterunderstandthetreat-to-closestrategy.
出版日期
2018年10月27日（中国期刊网平台首次上网日期，不代表论文的发表时间）
