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Continuing Education in Anaesthesia, Critical Care & Pain 2008 8(1):26-30; doi:10.1093/bjaceaccp/mkm047
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© The Board of Management and Trustees of the British Journal of Anaesthesia [2008]. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

The Fontan circulation

Sandeep Nayak, MBBS MD FRCA
Specialist Registrar in Anaesthesia
Royal Liverpool Children's NHS Trust
Eaton Road
Liverpool L12 2AP
UK

P.D. Booker, MBBS MD FRCA
Consultant Paediatric Anaesthetist
Royal Liverpool Children's NHS TrustEaton Road
Liverpool L12 2AP
UK
Tel: +44 151 252 5223 Fax: +44 151 252 5460 E-mail: peterdb@liv.ac.uk

Key Words: Increasing numbers of patients with a Fontan circulation are reaching adulthood and requiring anaesthesia for non-cardiac surgery • Understanding the physiology of the Fontan circulation is essential for the successful anaesthetic management of these patients. Anaesthesia should be administered only in a centre where the relevant cardiological and intensive care unit expertise is available. • The older Fontan patient is at particular risk of thromboembolism, arrhythmias and progressive ventricular dysfunction. • Heart function in the pregnant patient with a Fontan circulation deteriorates throughout pregnancy. Elective delivery under epidural anaesthesia is usually the technique of choice. • Laparoscopic and day care surgery is usually well tolerated in the young Fontan patient.

The first 150 words of the full text of this article appear below.

In a normal biventricular heart, the systemic and pulmonary circulations are in series and each circulation is supported by a ventricle. In patients born with a single ventricular chamber, the two circulations are in parallel and patients only survive because the systemic and pulmonary venous bloods mix. In 1971, Francis Fontan and Eugene Baudet first described a procedure that diverted all systemic venous blood into the pulmonary arteries, without the interposition of a ventricle, as a surgical palliation for tricuspid atresia. The introduction of this eponymous ‘Fontan operation’ 36 yr ago revolutionized the treatment of complex congenital heart defects and remains the treatment of choice for patients born with one functional ventricle. A large number of children continue to benefit from the Fontan operation. However, despite many refinements of the surgical procedure in the past 20 yr, a relatively high proportion of patients demonstrate a gradual decline in functional capacity . . . [Full Text of this Article]


   Indications for a Fontan circulation
 

   Surgical approach
 
Stage 1: systemic-pulmonary shunt

Stage 2: superior cavopulmonary connection (Glenn type operation)

Stage 3: completion of the Fontan circulation


   Complications of a Fontan circulation
 
Diminished exercise tolerance and ventricular dysfunction

Arrhythmias

Shunts

Protein losing enteropathy

Developmental deficit

Thromboembolism


   Anaesthetic management
 
Preoperative assessment

Monitoring

Induction and maintenance

Mechanical ventilation

Postoperative care


   Pregnancy and delivery
 

   Laparoscopic surgery
 

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