Managing adult Fontan patients: where do we stand?

被引:31
作者
Clift, Paul [1 ]
Celermajer, David [2 ]
机构
[1] Univ Hosp Birmingham, Dept Cardiol, Birmingham, W Midlands, England
[2] Royal Prince Alfred Hosp, Dept Cardiol, Sydney, NSW, Australia
关键词
PROTEIN-LOSING ENTEROPATHY; TOTAL CAVOPULMONARY CONNECTION; QUALITY-OF-LIFE; CONGENITAL HEART-DISEASE; INDUCED PULMONARY-HYPERTENSION; HIGH-DOSE SPIRONOLACTONE; TERM FOLLOW-UP; SINGLE VENTRICLE; EXERCISE CAPACITY; EXTRACARDIAC CONDUIT;
D O I
10.1183/16000617.0091-2016
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
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.
引用
收藏
页码:438 / 450
页数:13
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