Management of patients with pulmonary arterial hypertension due to congenital heart disease: recent advances and future directions

• Published in: Expert review of cardiovascular therapy Vol. 13; no. 12; p. 1377
• Main Authors: Blok, Ilja M, van Riel, Annelieke C M J, Mulder, Barbara J M, Bouma, Berto J
• Format: Journal Article
• Language: English
• Published: England 02.12.2015
• Subjects: Adult; Disease Management; Early Medical Intervention - methods; Echocardiography - methods; Heart Defects, Congenital - complications; Heart Defects, Congenital - physiopathology; Heart Defects, Congenital - surgery; Humans; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - physiopathology; Hypertension, Pulmonary - therapy; Hypoglycemic Agents - therapeutic use; Prognosis; closed defect; predictors; management; therapy; congenital heart disease; pulmonary arterial hypertension; surgery
• DOI: 10.1586/14779072.2015.1101341

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.