title: Managing adult Fontan patients: where do we stand?
abstract: The Fontan operation is performed as a palliative procedure to improve survival in infants born with a functionally univentricular circulation. The success of the operation is demonstrated by a growing adult Fontan population that exists with this unique physiology. Late follow-up has demonstrated expected and unexpected sequelae, and has shown multisystem effects of this circulation. This review discusses the challenges of managing the late complications in terms of understanding this unique physiology and the innovative therapeutic interventions that are being investigated. The challenge remains to maintain quality of life for adult survivors, as well as extending life expectancy.
authors.affiliation: Dept of Cardiology, University Hospital Birmingham, Birmingham, UK Paul.Clift@uhb.nhs.uk.
authors.forename: Paul
authors.initials: P
authors.lastname: Clift
authors.affiliation: Dept of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: David
authors.initials: D
authors.lastname: Celermajer
mesh_terms: D000328:Adult; D000367:Age Factors; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D062186:Arterial Pressure / Q000187:drug effects; D018729:Fontan Procedure / Q000009:adverse effects* / Q000401:mortality; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000208:ethnology / Q000401:mortality / Q000503:physiopathology; D010166:Palliative Care; D011237:Predictive Value of Tests; D011651:Pulmonary Artery / Q000187:drug effects / Q000503:physiopathology; D012307:Risk Factors; D017741:Survivors; D016896:Treatment Outcome; D066253:Vascular Remodeling / Q000187:drug effects
chemical_list: D000959:Antihypertensive Agents