Dextro-transposition of great arteries ACC/AHA guidelines

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editors-In-Chief: Keri Shafer, M.D. [2]; Atif Mohammad, M.D., Priyamvada Singh, MBBS[3]

Major Recommendations

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 of the Operated Patient with Dextro-Transposition of the Great Arteries (d-TGA)

Class I

• Patients with repaired d-TGA should have annual follow-up with a cardiologist who has expertise in the management of adult congenital heart disease (ACHD) patients. (Level of Evidence: C)

Recommendations for Imaging for Dextro-Transposition of the Great Arteries after Atrial Baffle Procedure

Class I

• In patients with d-TGA repaired by atrial baffle procedure, comprehensive echocardiographic imaging should be performed in a regional ACHD center to evaluate the anatomy and hemodynamics. (Level of Evidence: B)
• Additional imaging with transesophageal echocardiography (TEE), computed tomography (CT), or magnetic resonance imaging (MRI), as appropriate, should be performed in a regional ACHD center to evaluate the great arteries and veins, as well as ventricular function, in patients with prior atrial baffle repair of d-TGA. (Level of Evidence: B)

Class IIa

• Echocardiography contrast injection with agitated saline can be useful to evaluate baffle anatomy and shunting in patients with previously repaired d-TGA after atrial baffle. (Level of Evidence: B)
• TEE can be effective for more detailed baffle evaluation for patients with d-TGA. (Level of Evidence: B)

Recommendations for Imaging for Dextro-Transposition of the Great Arteries after Arterial Switch Operation

Class I

• Comprehensive echocardiographic imaging to evaluate the anatomy and hemodynamics in patients with d-TGA and prior arterial switch operation (ASO) repair should be performed at least every 2 years at a center with expertise in ACHD. (Level of Evidence: C)
• After prior ASO repair for d-TGA, all adults should have at least 1 evaluation of coronary artery patency. Coronary angiography should be performed if this cannot be established noninvasively. (Level of Evidence: C)

Class IIa

• Periodic MRI or CT can be considered appropriate to evaluate the anatomy and hemodynamics in more detail. (Level of Evidence: C)

Recommendation for Cardiac Catheterization after Arterial Switch Operation

Class IIa

• Coronary angiography is reasonable in all adults with d-TGA after ASO to rule out significant coronary artery obstruction. (Level of Evidence: C)

Recommendation for Diagnostic Catheterization for Adults With Repaired Dextro-Transposition of the Great Arteries

Class I

• Diagnostic catheterization of the adult with d-TGA should be performed in centers with expertise in the catheterization and management of ACHD patients. (Level of Evidence: C)

Class IIa

• For adults with d-TGA after atrial baffle procedure (Mustard or Senning), diagnostic catheterization can be beneficial to assist in the following:
  • Hemodynamic assessment. (Level of Evidence: C)
  • Assessment of baffle leak. (Level of Evidence: B)
  • Assessment of superior vena cava or inferior vena cava pathway obstruction. (Level of Evidence: B)
  • Assessment of pulmonary venous pathway obstruction. (Level of Evidence: B)
  • Suspected myocardial ischemia or unexplained systemic RV dysfunction. (Level of Evidence: B)
  • Significant left ventricular (LV) outflow obstruction at any level (LV pressure greater than 50% of systemic levels, or less in the setting of right ventricular [RV] dysfunction). (Level of Evidence: B)
  • Assessment of pulmonary arterial hypertension (PAH), with potential for vasodilator testing. (Level of Evidence: C)

• For adults with d-TGA, ventricular septal defect (VSD), and pulmonary stenosis (PS), after Rastelli-type repair, diagnostic catheterization can be beneficial to assist in the following:
  • Coronary artery delineation before any intervention for right ventricular outflow tract (RVOT) obstruction. (Level of Evidence: C)
  • Assessment of residual VSD. (Level of Evidence: C)
  • Assessment of PAH, with potential for vasodilator testing. (Level of Evidence: C)
  • Assessment of subaortic obstruction across the left ventricle-to-aorta tunnel. (Level of Evidence: C)

Management Strategies

Recommendations for Interventional Catheterization for Adults with Dextro-Transposition of the Great Arteries

Class IIa

• Interventional catheterization of the adult with d-TGA can be performed in centers with expertise in the catheterization and management of ACHD patients. (Level of Evidence: C)
• For adults with d-TGA after atrial baffle procedure (Mustard or Senning), interventional catheterization can be beneficial to assist in the following:
  • Occlusion of baffle leak. (Level of Evidence: B)
  • Dilation or stenting of superior vena cava or inferior vena cava pathway obstruction. (Level of Evidence: B)
  • Dilation or stenting of pulmonary venous pathway obstruction. (Level of Evidence: B)
  • For adults with d-TGA after ASO, interventional catheterization can be beneficial to assist in dilation or stenting of supravalvular and branch pulmonary artery stenosis. (Level of Evidence: B)
  • For adults with d-TGA, VSD, and PS, after Rastelli-type repair, interventional catheterization can be beneficial to assist in the following:
    • Dilation with or without stent implantation of conduit obstruction (RV pressure greater than 50% of systemic levels, or peak-to-peak gradient greater than 30 mm Hg; these indications may be lessened in the setting of RV dysfunction). (Level of Evidence: C)
    • Device closure of residual VSD. (Level of Evidence: C)

Recommendations for Surgical Interventions

After Atrial Baffle Procedure (Mustard, Senning)

Class I

• Surgeons with training and expertise in congenital heart disease (CHD) should perform operations in patients with d-TGA and the following indications:
  • Moderate to severe systemic (morphological tricuspid) AV valve regurgitation without significant ventricular dysfunction. (Carrel & Pfammatter, 2000) (Level of Evidence: B)
  • Baffle leak with left-to-right shunt greater than 1.5:1, right to-left shunt with arterial desaturation at rest or with exercise, symptoms, and progressive ventricular enlargement that is not amenable to device intervention. (Level of Evidence: B)
  • Superior vena cava or inferior vena cava obstruction not amenable to percutaneous treatment. (Level of Evidence: B)
  • Pulmonary venous pathway obstruction not amenable to percutaneous intervention. (Level of Evidence: B)
  • Symptomatic severe subpulmonary stenosis. (Level of Evidence: B)

After Arterial Switch Operation

Class I

• It is recommended that surgery be performed in patients after the ASO with the following indications:
  • RVOT obstruction peak-to-peak gradient greater than 50 mm Hg or right ventricle/left ventricle pressure ratio greater than 0.7, not amenable or responsive to percutaneous treatment; lesser degrees of obstruction if pregnancy is planned, greater degrees of exercise are desired, or concomitant severe pulmonary regurgitation is present. (Level of Evidence: C)
  • Coronary artery abnormality with myocardial ischemia not amenable to percutaneous intervention. (Level of Evidence: C)
  • Severe neoaortic valve regurgitation. (Level of Evidence: C)
  • Severe neoaortic root dilatation (greater than 55 mm) after ASO. (Coady et al., 1999) (This recommendation is based on data for other forms of degenerative aortic root aneurysms). (Level of Evidence: C)

After Rastelli Procedure

Class I

• Reoperation for conduit and/or valve replacement after Rastelli repair of d-TGA is recommended in patients with the following indications:
  • Conduit obstruction peak-to-peak gradient greater than 50 mm Hg. (Level of Evidence: C)
  • RV/LV pressure ratio greater than 0.7. (Level of Evidence: C)
  • Lesser degrees of conduit obstruction if pregnancy is being planned or greater degrees of exercise are desired. (Level of Evidence: C)
  • Subaortic (baffle) obstruction (mean gradient greater than 50 mm Hg). (Level of Evidence: C)
  • Lesser degrees of subaortic (baffle) obstruction if LV hypertrophy is present, pregnancy is being planned, or greater degrees of exercise are desired. (Level of Evidence: C)
  • Presence of concomitant severe aortic regurgitation (AR). (Level of Evidence: C)
• Reoperation for conduit regurgitation after Rastelli repair of d-TGA is recommended in patients with severe conduit regurgitation and the following indicators:
  • Symptoms or declining exercise tolerance. (Level of Evidence: C)
  • Severely depressed RV function. (Level of Evidence: C)
  • Severe RV enlargement. (Level of Evidence: C)
  • Development/progression of atrial or ventricular arrhythmias. (Level of Evidence: C)
  • More than moderate tricuspid regurgitation (TR). (Level of Evidence: C)
• Collaboration between surgeons and interventional cardiologists, which may include preoperative stenting, intraoperative stenting, or intraoperative patch angioplasty with or without conduit replacements, is recommended to determine the most feasible treatment for pulmonary artery stenosis. (Level of Evidence: C)
• Surgical closure of residual VSD in adults after Rastelli repair of d-TGA is recommended with the following indicators:
  • Pulmonary blood flow/systemic blood flow (Qp/Qs) greater than 1.5:1. (Level of Evidence: B)
  • Systolic pulmonary artery pressure greater than 50 mm Hg. (Level of Evidence: B)
  • Increasing LV size from volume overload. (Level of Evidence: C)
  • Decreasing RV function from pressure overload. (Level of Evidence: C)
  • RVOT obstruction (peak instantaneous gradient greater than 50 mm Hg). (Level of Evidence: B)
  • Pulmonary artery pressure less than two thirds of systemic pressure, or PVR less than two thirds of systemic vascular resistance, with a net left-to-right shunt of 1.5:1, or a decrease in pulmonary artery pressure with pulmonary vasodilators (oxygen, nitric oxide, or prostaglandins). (Level of Evidence: B)
  • Surgery is recommended after Rastelli repair of d-TGA in adults with branch pulmonary artery stenosis not amenable to percutaneous treatment. (Level of Evidence: C)
  • In the presence of a residual intracardiac shunt or significant systemic venous obstruction, permanent pacing, if indicated, should be performed with epicardial leads. (Carrel & Pfammatter, 2000) (Level of Evidence: B)

Class IIa

• A concomitant Maze procedure can be effective for the treatment of intermittent or chronic atrial tachyarrhythmias in adults with d-TGA requiring reoperation for any reason. (Level of Evidence: C)

Recommendations for Electrophysiology Testing/Pacing Issues in Dextro-Transposition of the Great Arteries

Class I

• Clinicians should be mindful of the risk of sudden arrhythmic death among adults after atrial baffle repair of d-TGA. These events usually relate to ventricular tachycardia (VT) but may be caused in some cases by rapidly conducted intra-atrial reentrant tachycardia (IART) or progressive atrioventricular (AV) block. (Level of Evidence: B)
• Consultation with an electrophysiologist who is experienced with CHD is recommended to assist with treatment decisions. (Level of Evidence: B)
• Pacemaker implantation is recommended for patients with d-TGA with either symptomatic sinus bradycardia or sick sinus syndrome. (Level of Evidence: B)

Class IIa

• Routine surveillance with history, electrocardiogram (ECG), assessment of RV function, and periodic Holter monitoring can be beneficial as part of routine follow-up. (Level of Evidence: B)

Key Issues to Evaluate and Follow-Up

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 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 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

• Before women with d-TGA contemplate pregnancy, a comprehensive clinical, functional, and echocardiographic evaluation should be performed at a center with expertise in ACHD. (Level of Evidence: C)

External links

• Cardiothoracic Surgery Network (CTSNet)
• ped/2548 at eMedicine
• Heart Center Encyclopedia, Cincinnati Children's Hospital Medical Center
• HeartCentreOnline
• Med-Lib
• PediHeart
• Seattle Children's Hospital Heart Center
• Variety Children's Heart Centre

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