Tricuspid atresia surgery

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Tricuspid atresia Microchapters


Patient Information




Differentiating Tricuspid Atresia from other Disorders

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis


History and Symptoms

Physical Examination

Laboratory Findings


Chest X Ray




Cardiac Catheterization

Other Imaging Findings

Other Diagnostic Studies


Medical Therapy


Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

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Case #1

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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]


Surgical Interventions

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

TA Fontan procedure.jpg

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[1][2]

Surgical Options for Patients With Single Ventricle (DO NOT EDIT)[1][2]

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)"

Evaluation and Follow-Up After Fontan Procedure (DO NOT EDIT)[1][2]

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)"

Surgery for Adults With Prior Fontan Repair (DO NOT EDIT)[1][2]

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)"