RT Journal Article
SR Electronic
T1 Managing adult Fontan patients: where do we stand?
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 438
OP 450
DO 10.1183/16000617.0091-2016
VO 25
IS 142
A1 Paul Clift
A1 David Celermajer
YR 2016
UL http://err.ersjournals.com/content/25/142/438.abstract
AB 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.Innovative solutions are required to meet the challenges of the Fontan circulation faced in adult life http://ow.ly/XTSm305oH8b