Pulmonary arterial hypertension in adults with congenital heart disease.
Pulmonary arterial hypertension (PAH) is a common problem in adult patients with congenital heart disease. We review available data on aetiology, clinical presentation, prognosis and management of PAH in this setting. In addition, we discuss general management strategies and emerging disease-targeting therapies.
Diller
G-P
GP
Department of Cardiology and Angiology, Adult Congenital and Valvular Heart Disease Centre (EMAH Zentrum), University Hospital Muenster, University of Muenster, Muenster, Germany. gerhard.diller@ukmuenster.de
Baumgartner
H
H
D000328:Adult; D015897:Comorbidity; D004562:Electrocardiography; D006330:Heart Defects, Congenital / Q000453:epidemiology* / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology* / Q000628:therapy; D015995:Prevalence; D011651:Pulmonary Artery / Q000473:pathology; D012307:Risk Factors; D012720:Severity of Illness Index