Tricuspid atresia ACC/AHA guidelines

Associate Editor-In-Chief: Keri Shafer, M.D. [mailto:kshafer@bidmc.harvard.edu] Priyamvada Singh, MBBS mailto:psingh@perfuse.org

Assistant Editor-In-Chief: Kristin Feeney, B.S. mailto:kfeeney@perfuse.org

Major Recommendations (DONOT EDIT)

The American College of Cardiology/American Heart Association (ACC/AHA) classification of the recommendations for patient evaluation and treatment (classes I-III) and the levels of evidence (A-C) are defined at the end of the "Major Recommendations" field.

Clinical Features and Evaluation of the Unoperated or Palliated Patient

Recommendations for Catheterization Before Fontan Procedure

Class I


 * In the evaluation of hemodynamics to assess the potential for definitive palliation of unoperated or shunt-palliated adults with univentricular hearts, catheterization is indicated to:
 * Assess the nature of pulmonary artery obstruction, with potential to restore maximal continuous, effective, unimpeded systemic venous flow to the maximal number of pulmonary artery segments. (Level of Evidence: C)
 * Assess and eliminate systemic-to-pulmonary vein collaterals. (Level of Evidence: C
 * Assess and eliminate systemic-to-pulmonary artery connections. (Level of Evidence: C)
 * For adults with systemic-to-pulmonary shunts, the potential for perioperative transcatheter shunt exclusion should be examined. (Level of Evidence: C)

Recommendation for Surgical Options for Patients With Single Ventricle

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)

Recommendation for Evaluation and Follow-Up After Fontan Procedure

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)

Recommendation for Imaging

Class I


 * All patients with prior Fontan type of repair should have periodic echocardiographic and/or magnetic resonance examinations performed by staff with expertise in ACHD. (Level of Evidence: C)

Recommendation for Diagnostic and Interventional Catheterization after Fontan Procedure

Class I


 * Catheterization of adults with a Fontan type of repair of single-ventricle physiology should be performed in regional centers with expertise in ACHD. (Level of Evidence: C)

Recommendations for Management Strategies for the Patient with Prior Fontan Repair

Class I


 * Management of patients with prior Fontan repair should be coordinated with a regional ACHD center. Local cardiologists, internists, and family care physicians should develop ongoing relationships with such a center with continuous availability of specialists. (Level of Evidence: C)
 * At least yearly follow-up is recommended for patients after Fontan repair. (Level of Evidence: C)
 * Arrhythmia management is frequently an issue, and consultation with an electrophysiologist is recommended as a vital part of care. (Level of Evidence: C)
 * New-onset atrial tachyarrhythmia should prompt a comprehensive noninvasive imaging evaluation to identify associated atrial/baffle thrombus, anatomic abnormalities of the Fontan pathway, or ventricular dysfunction. (Level of Evidence: C)

Recommendations for Medical Therapy

Class I


 * Warfarin should be given for patients who have a documented atrial shunt, atrial thrombus, atrial arrhythmias, or a thromboembolic event. (Level of Evidence: C)

Class IIa


 * It is reasonable to treat systemic ventricle (SV) dysfunction with angiotensin-converting enzyme (ACE) inhibitors and diuretics. (Level of Evidence: C)

Recommendations for Surgery for Adults with Prior Fontan Repair

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)

Key Issues to Evaluate and Follow-Up

Recommendations for Electrophysiology Testing/Pacing Issues in Single-Ventricle Physiology and after Fontan Procedure

Class I


 * Arrhythmia management is frequently an issue in patients after the Fontan procedure, and consultation with an electrophysiologist with expertise in CHD is recommended as a vital part of care. (Level of Evidence: C)
 * New-onset atrial tachyarrhythmias should prompt a comprehensive noninvasive imaging evaluation to identify associated atrial/baffle thrombus, anatomic abnormalities of the Fontan pathway, or ventricular dysfunction. (Level of Evidence: C)
 * Electrophysiological studies in adults with Fontan physiology should be performed at centers with expertise in the management of such patients. (Level of Evidence: C)
 * Clinicians must be mindful of the high risk for symptomatic intra-atrial reentrant tachycardia (IART) in adult patients who have undergone the Fontan operation. This arrhythmia can cause serious hemodynamic compromise and contribute to atrial thrombus formation. Treatment is often difficult, and consultation with an electrophysiologist who is experienced with CHD is recommended whenever recurrent IART is detected. (Level of Evidence: C)

Recommendations for Endocarditis Prophylaxis

Class IIa


 * Antibiotic prophylaxis before dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa is reasonable in those patients with the following indications:
 * Prosthetic cardiac valve. (Level of Evidence: B)
 * Previous infective endocarditis (IE). (Level of Evidence: B)
 * Unrepaired and palliated cyanotic CHD, including surgically constructed palliative shunts and conduits. (Level of Evidence: B)
 * Completely repaired CHD with prosthetic materials, whether placed by surgery or catheter intervention, during the first 6 months after the procedure. (Level of Evidence: B)
 * Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device that inhibit endothelialization. (Level of Evidence: B)
 * It is reasonable to consider antibiotic prophylaxis against IE before vaginal delivery at the time of membrane rupture in select patients with the highest risk of adverse outcomes. This includes patients with the following indications:
 * Prosthetic cardiac valve or prosthetic material used for cardiac valve repair. (Level of Evidence: C)
 * Unrepaired and palliated cyanotic CHD, including surgically constructed palliative shunts and conduits. (Level of Evidence: C)

Class III


 * Prophylaxis against IE is not recommended for nondental procedures (such as esophagogastroduodenoscopy or colonoscopy) in the absence of active infection. (Level of Evidence: C)

Recommendations for Reproduction

Class


 * All women with a Fontan operation should have a comprehensive evaluation by a physician with expertise in ACHD before proceeding with a pregnancy. (Level of Evidence: C)

Class III


 * Pregnancy should not be planned without consultation and evaluation at a comprehensive ACHD center with experience and expertise in maternal and prenatal management of complex CHD. (Level of Evidence: C)

Additional Reading

 * Moss and Adams' Heart Disease in Infants, Children, and Adolescents Hugh D. Allen, Arthur J. Moss, David J. Driscoll, Forrest H. Adams, Timothy F. Feltes, Robert E. Shaddy, 2007 ISBN 0781786843