L-transposition of the great arteries ACC/AHA guidelines


 * Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [mailto:psingh@perfuse.org]; ; Keri Shafer, M.D. [mailto:kshafer@bidmc.harvard.edu]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [mailto:kfeeney@perfuse.org]

Overview
Recommendations 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.

Recommendations for Evaluation and Follow-Up of Patients with Congenitally Corrected Transposition of the Great Arteries (CCTGA)

Class I


 * All patients with CCTGA should have a regular follow-up with a cardiologist who has expertise in adult congenital heart disease (ACHD). (Level of Evidence: C)
 * Echocardiography-Doppler study and/or magnetic resonance imaging (MRI) should be performed yearly or at least every other year by staff trained in imaging complex congenital heart disease (CHD). (Level of Evidence: C)
 * The following diagnostic evaluations are recommended for patients with CCTGA:
 * Electrocardiogram (ECG). (Level of Evidence: C)
 * Chest x-ray. (Level of Evidence: C)
 * Echocardiography-Doppler study. (Level of Evidence: C)
 * MRI. (Level of Evidence: C)
 * Exercise testing. (Level of Evidence: C)

Interventional Therapy

Recommendations for Catheter Interventions

Class IIa


 * For patients with unrepaired CCTGA, cardiac catheterization can be effective to assess the following:
 * Hemodynamic status in the setting of arrhythmia. (Level of Evidence: C)
 * Unexplained systemic ventricle (SV) dysfunction, to define the degree of systemic AV valve regurgitation, degree of intracardiac shunting, and coronary artery anatomy. (Level of Evidence: C)
 * Unexplained volume retention or cyanosis, especially when noninvasive assessment of pulmonary outflow obstruction is limited. (Level of Evidence: C)

Recommendations for Surgical Intervention

Class I


 * Surgeons with training and expertise in CHD should perform operations for patients with CCTGA for the following indications:
 * Unrepaired CCTGA and severe atrioventricular (AV) valve regurgitation. (Level of Evidence: B)
 * Anatomic repair with atrial and arterial level switch/Rastelli repair in cases in which the left ventricle is functioning at systemic pressures. (Level of Evidence: B)
 * Simple ventricular septal defect (VSD) closure when the VSD is not favorable for left ventricular (LV)-to-aorta baffling or is restrictive. (Level of Evidence: B)
 * LV–to–pulmonary artery conduit in rare cases with LV dysfunction and severe LV outflow obstruction. (Level of Evidence: B)
 * Evidence of moderate or progressive systemic AV valve regurgitation. (Level of Evidence: B)
 * Conduit obstruction with systemic or nearly systemic right ventricular (RV) pressures and/or RV dysfunction after anatomic repair. (Level of Evidence: B)
 * Conduit obstruction and systemic or suprasystemic LV pressures in a patient with nonanatomic correction. (Level of Evidence: B)
 * Moderate or severe aortic regurgitation (AR)/neo-AR and onset of ventricular dysfunction or progressive ventricular dilatation. (Level of Evidence: B)

Recommendations for Postoperative Care

Class I


 * Patients with prior repair of CCTGA should have regular follow-up with a cardiologist with expertise in ACHD. (Level of Evidence: C)
 * Echocardiography-Doppler study and/or MRI should be performed yearly or at least every other year by staff trained in imaging complex CHD. (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 with the following indications:
 * Prosthetic cardiac valve. (Level of Evidence: B)
 * Previous 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 by 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)

Recommendation for Reproduction

Class I


 * All women with CCTGA (whether repaired or not) should seek counseling from a cardiologist with expertise in ACHD before proceeding with a pregnancy. (Level of Evidence: C)