title: The exceptional and far-flung manifestations of heart failure in Eisenmenger syndrome.
abstract: Dramatic advances in the diagnosis and treatment of congenital heart disease (CHD), the most common inborn defect, has resulted in a growing population of adults with CHD. Eisenmenger syndrome (ES) represents the extreme form of pulmonary arterial hypertension associated with CHD, characterized by markedly increased pulmonary vascular resistance with consequently reversed or bidirectional shunting. While ES is a direct consequence of a heart defect, it is a fundamentally multisystem syndrome with wide-ranging clinical manifestations. The introduction of targeted pulmonary hypertension therapies aimed has subtly shifted clinical focus from preventing iatrogenic and other adverse events toward cautious therapeutic activism.
authors.affiliation: Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.
authors.forename: Alexander R
authors.initials: AR
authors.lastname: Opotowsky
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Landzberg
authors.forename: Maurice
authors.initials: M
authors.lastname: Beghetti
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000379:methods; D002965:Classification; D019468:Disease Management; D004541:Eisenmenger Complex / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000601:surgery; D005060:Europe; D017079:Exercise Tolerance / Q000187:drug effects; D065627:Familial Primary Pulmonary Hypertension; D006333:Heart Failure / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D007501:Iron / Q000627:therapeutic use; D009102:Multiple Organ Failure / Q000209:etiology / Q000503:physiopathology / Q000517:prevention & control; D010102:Oxygen Inhalation Therapy / Q000379:methods; D017410:Practice Guidelines as Topic; D015995:Prevalence; D011379:Prognosis; D011652:Pulmonary Circulation / Q000187:drug effects; D014131:Trace Elements / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D014131:Trace Elements; D007501:Iron
keywords: Congenital heart disease; Eisenmenger syndrome; Pulmonary hypertension; Systemic-to-pulmonary arterial shunts