Surgeons with training and expertise in congenital heart disease (CHD) should perform operations for single-ventricle anatomy or physiology. (Level of Evidence: C)
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Surgical Interventions
There are a number of interventional methods to address a tricuspid atresia. These include:
Lifelong follow-up is recommended for patients after a Fontan type of operation; this should include a yearly evaluation by a cardiologist with expertise in the care of adult congenital heart disease (ACHD) patients. (Level of Evidence: C)
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ACC/AHA Guidelines - Recommendations for Surgery for Adults with Prior Fontan Repair (DO NOT EDIT)
Surgeons with training and expertise in CHD should perform operations on patients with prior Fontan repair for single-ventricle physiology. (Level of Evidence: C)
Reoperation after Fontan is indicated for the following:
Unintended residual atrial septal defect (ASD) that results in right-to-left shunt with symptoms and/or cyanosis not amenable to transcatheter closure. (Level of Evidence: C)
Hemodynamically significant residual systemic artery-to-pulmonary artery shunt, residual surgical shunt, or residual ventricle-to-pulmonary artery connection not amenable to transcatheter closure. (Level of Evidence: C)
Moderate to severe systemic atrioventricular (AV) valve regurgitation. (Level of Evidence: C)
Significant (greater than 30-mm Hg peak-to-peak) subaortic obstruction. (Level of Evidence: C)
Fontan pathway obstruction. (Level of Evidence: C)
Development of venous collateral channels or pulmonary arteriovenous malformation not amenable to transcatheter management. (Level of Evidence: C)
Pulmonary venous obstruction. (Level of Evidence: C)
Rhythm abnormalities, such as complete AV block or sick sinus syndrome, that require epicardial pacemaker insertion. (Level of Evidence: C)
Creation or closure of a fenestration not amenable to transcatheter intervention. (Level of Evidence: C)
Reoperation for Fontan conversion (i.e., revision of an atriopulmonary connection to an intracardiac lateral tunnel, intra-atrial conduit, or extracardiac conduit) can be useful for recurrent atrial fibrillation or flutter without hemodynamically significant anatomic abnormalities. A concomitant Maze procedure should also be performed. (Level of Evidence: C)
