title: [Pulmonary arterial hypertension in adult patients with congenital heart disease].
abstract: Pulmonary Hypertension (PH) is definited by a mean pulmonary artery pressure (PAPm) >25 mmHg at rest. The Dana Point 2008 Revised Classification System represents the most recent classification system update with respect of various etiologies of PH. About 10 % of adolescents or adults with uncorrected congenital heart disease (CHD) with left-to-right shunt and high pulmonary blood flow develop Pulmonary Arterial Hypertension (PAH) . Progressive vascular remodeling and increase in pulmonary vascular resistance (PVR) may ultimately lead to reversal of the shunt (pulmonary to systemic) causing cyanosis and determining the so-called Eisenmenger Syndrome (ES). Recent advances in the early diagnosis and medical targeted treatment of adult patients with CHD-PAH and ES can improve PAP, PVR and exercise tolerance, together with NYHA Class and survival, and may potentially reverse the vascular remodeling process in selected patients.
authors.affiliation: Dipartimento Medicina Interna e Medicina Vascolare IRCCS Policlinico San Donato (Milano), Italy. giorgio.serino@grupposandonato.it
authors.forename: G
authors.initials: G
authors.lastname: Serino
authors.forename: F
authors.initials: F
authors.lastname: Giacomazzi
mesh_terms: D000328:Adult; D006330:Heart Defects, Congenital / Q000150:complications* / Q000503:physiopathology; D006331:Heart Diseases / Q000150:complications* / Q000151:congenital* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy