title: Management of patients with pulmonary arterial hypertension due to congenital heart disease: recent advances and future directions.
abstract: Pulmonary arterial hypertension is a serious complication of adult congenital heart disease associated with systemic-to-pulmonary shunts. Although early shunt closure restricts development of pulmonary arterial hypertension, patients remain at risk even after repair. The development of pulmonary arterial hypertension is associated with a markedly increased morbidity and mortality. It is important to identify patients with a poor prognosis using disease specific markers. Echocardiography and biomarkers arise as practical tools to determine the risk of mortality. Although pulmonary arterial hypertension cannot be cured, four classes of disease-targeting therapies are currently available and several promising therapies are being studied. There is a shift in drug studies towards more clinically relevant endpoints such as time to clinical worsening and morbidity and mortality events.
authors.affiliation: a Department of Cardiology , Academic Medical Centre , Amsterdam , The Netherlands.|b ICIN - Netherlands Heart Institute , Utrecht , The Netherlands.
authors.forename: Ilja M
authors.initials: IM
authors.lastname: Blok
authors.affiliation: a Department of Cardiology , Academic Medical Centre , Amsterdam , The Netherlands.|b ICIN - Netherlands Heart Institute , Utrecht , The Netherlands.
authors.forename: Annelieke C M J
authors.initials: AC
authors.lastname: van Riel
authors.affiliation: a Department of Cardiology , Academic Medical Centre , Amsterdam , The Netherlands.|b ICIN - Netherlands Heart Institute , Utrecht , The Netherlands.
authors.forename: Barbara J M
authors.initials: BJ
authors.lastname: Mulder
authors.affiliation: a Department of Cardiology , Academic Medical Centre , Amsterdam , The Netherlands.
authors.forename: Berto J
authors.initials: BJ
authors.lastname: Bouma
mesh_terms: D000328:Adult; D019468:Disease Management; D061345:Early Medical Intervention / Q000379:methods; D004452:Echocardiography / Q000379:methods; D006330:Heart Defects, Congenital / Q000150:complications / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D007004:Hypoglycemic Agents / Q000627:therapeutic use*; D011379:Prognosis
chemical_list: D007004:Hypoglycemic Agents
keywords: closed defect; congenital heart disease; management; predictors; pulmonary arterial hypertension; surgery; therapy