Those with congenital heart defects may need to have the Fontan procedure done to repair heart defects that become apparent at birth. These complex congential heart defects that can require the Fontan procedure include tricuspid atresia, hydoplastic left heart syndrome, single ventricle and pulmonary atresia. The Fontan procedure provides blood flow when the right ventricular pumping chamber is unable to before its job.
There are ten requirements or characteristics that as used to foresee a good outcome for the Fontan procedure. The ten requirements of the Fontan procedure is that the patient is above age four, has normal right atrial size, normal heart rhythm, low lung blood vessel resistance, low pulmonary artery pressure, no atriovascular valve leak, no distortion of lung arteries or shunts from prior surgery, normal venous drainage, normal ventricular function, and adequate pulmonary artery size.
The Fontan procedure is not just one procedure. There are three different procedures that could be performed. The original Fontan procedure is the artiopulmonary connection. The other Fontan procedures are the lateral tunnel or intracardiac total cavopulmonary connection and the extracardiac total cavopulmonary connection. Each procedure performed is used during specific conditions for the patient using the procedure that will work best for them.
There are problems that can arise after the Fontan procedure is done. There can be disturbances with heart rhythm, retention of fluid, or protein losing enteropathy. Since the Fontan procedure was first performed there have been some changes to the procedure. These changes improved the long term results of the Fontan procedure.