Tricuspid atresia surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Keri Shafer, M.D. [2] Priyamvada Singh, MBBS [[3]]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [[4]]

Surgery

ACC/AHA 2008 Guidelines - Recommendation for Surgical Options for Patients With Single Ventricle - Tricuspid atresia (DO NOT EDIT)

Class I

"1.Surgeons with training and expertise in congenital heart disease (CHD) should perform operations for single-ventricle anatomy or physiology.(Level of Evidence: C) "

Surgical Interventions

There are a number of interventional methods to address a tricuspid atresia. These include:

• PGE1 to maintain patent ductus arteriosus
• Modified Blalock-Taussig shunt to maintain pulmonary blood flow by placing a Gortex conduit between the subclavian artery and the pulmonary artery.
• Cavopulmonary anastomosis (hemi-Fontan or bidirectional Glenn) to provide stable pulmonary flow
• Fontan procedure to redirect inferior vena cava and hepatic vein flow into the pulmonary circulation

ACC/AHA 2008 Guidelines - Recommendation for Evaluation and Follow-Up After Fontan Procedure - Tricuspid atresia (DO NOT EDIT)

Class I

"1.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) "

ACC/AHA 2008 Guidelines - Recommendations for Surgery for Adults with Prior Fontan Repair - Tricuspid atresia (DO NOT EDIT)

Class I

"1.Surgeons with training and expertise in CHD should perform operations on patients with prior Fontan repair for single-ventricle physiology.(Level of Evidence: C) "

"2.Reoperation after Fontan is indicated for the following:(Level of Evidence: C) "

"a.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) "

"b.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) "

"c.Moderate to severe systemic atrioventricular (AV) valve regurgitation.(Level of Evidence: C) "

"d.Significant (greater than 30-mm Hg peak-to-peak) subaortic obstruction.(Level of Evidence: C) "

"e.Fontan pathway obstruction.(Level of Evidence: C) "

"f.Development of venous collateral channels or pulmonary arteriovenous malformation not amenable to transcatheter management.(Level of Evidence: C) "

"g.Pulmonary venous obstruction.(Level of Evidence: C) "

"h.Rhythm abnormalities, such as complete AV block or sick sinus syndrome, that require epicardial pacemaker insertion.(Level of Evidence: C) "

"i.Creation or closure of a fenestration not amenable to transcatheter intervention.(Level of Evidence: C) "

Class IIa

"1.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) "

Class IIb

"1.Heart transplantation may be beneficial for severe SV dysfunction or protein-losing enteropathy (PLE).(Level of Evidence: C) "