CARDIOLOGISTS IN DEVARABESANAHALLI BANGALORE
Complex congenital heart disease: Fontan repair
Anatomy and physiology
Babies with a single ventricle or equivalent defect are often treated during childhood with a
operation to connect venous return directly to the pulmonary arteries—a Fontan
repair. The prognosis is usually good: 75% of these patients survive at least 20 years.
Problems develop because of failure of the systemic ventricle, obstruction of the venous to
pulmonary connection, atrial enlargement and AV valve regurgitation. Atrial arrhythmias
become increasingly common. Sinus node dysfunction may necessitate pacing, which requires
an epicardial electrode in most cases.
Echocardiography allows assessment of ventricular function and the AV valve. Obstruction
in the Fontan connections can be examined with Doppler. MRI is increasingly useful for this
Severe AV valve regurgitation, cyanosis and ventricular dysfunction are indications for intervention,
including transplant or revision of the Fontan. Atrial arrhythmias can be treated with
Pregnancy and contraception
Pregnancy is possible in some patients with excellent Fontan function, good LV function and
minimal AV valve regurgitation. Maternal risk is high if the Fontan is failing. There may be
problems with the need to withdraw ACE inhibitors and with anticoagulation.