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 Guidelines - Recommendation for Surgical Options for Patients With Single Ventricle (DO NOT EDIT)

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Class I 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:

• 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 Guidelines - Recommendation for Evaluation and Follow-Up After Fontan Procedure (DO NOT EDIT)

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Class I 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)

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

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