Dextro-transposition of the great arteries atrial switch repair


 * 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
Mustard and Senning Operations
 * It was first described by Senning in 1959.
 * In 1964, Mustard published his experience with the atrial switch.
 * This operation became popular due to an increase in survival of over 90%.
 * Both of these procedures "correct" the physiologic abnormality of the TGA by forming a baffle within the atria in order to switch the flow of blood at inflow level. As a consequence the heart and lungs will be in series.
 * The Mustard Operation consist of an atrial septectomy and placement of a baffle that directs caval blood to the mitral valve, allowing the pulmonary veins to drain into the tricuspid valve. The baffle is created from pericardium or synthetic material.
 * The Senning operation, utilized right atrial wall and atrial septal tissue (without the use of extrinsic materials), to create the baffle or wall of the caval tunnel in order to achieve the same goal as in Mustard.
 * The early mortality rate for both procedures is low, between 1 and 10%
 * The long-term outcome is affected by late complications such as atrial arrhythmia (with the highest incidence of more than 50% within 10 years), and a late right ventricular (systemic ventricular) dysfunction (approximately 10%).
 * The Senning repair is becoming more promising than Mustard due to the better long term outcomes in terms of venous obstruction and atrial haemodynamics. However, the procedure of choice for treatment of patients with d-TGA is the Arterial Switch or Jatene Operation.

==(ACC/AHA) recommendations for Imaging for Dextro-Transposition of the Great Arteries after atrial baffle procedure (DONOT EDIT)==

Class I

1. 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) 2. 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

1. 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) 2. TEE can be effective for more detailed baffle evaluation for patients with d-TGA. (Level of Evidence: B)

==(ACC/AHA) Recommendation for Diagnostic Catheterization for Adults With Repaired Dextro-Transposition of the Great Arteries (DONOT EDIT)==

Class IIa

1. For adults with d-TGA after atrial baffle procedure (Mustard or Senning), diagnostic catheterization can be beneficial to assist in the following:
 * 1. Hemodynamic assessment. (Level of Evidence: C)
 * 2. Assessment of baffle leak. (Level of Evidence: B)
 * 3. Assessment of superior vena cava or inferior vena cava pathway obstruction. (Level of Evidence: B)
 * 4. Assessment of pulmonary venous pathway obstruction. (Level of Evidence: B)
 * 5. Suspected myocardial ischemia or unexplained systemic RV dysfunction. (Level of Evidence: B)
 * 6. 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)
 * 7. Assessment of pulmonary arterial hypertension (PAH), with potential for vasodilator testing. (Level of Evidence: C)

==(ACC/AHA) Recommendation for Interventional Catheterization for Adults with Repaired Dextro-Transposition of the Great Arteries (DONOT EDIT)==

Class IIa

1. 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)

2. For adults with d-TGA after atrial baffle procedure (Mustard or Senning), interventional catheterization can be beneficial to assist in the following:
 * 1. Occlusion of baffle leak. (Level of Evidence: B)
 * 2. Dilation or stenting of superior vena cava or inferior vena cava pathway obstruction. (Level of Evidence: B)
 * 3. Dilation or stenting of pulmonary venous pathway obstruction. (Level of Evidence: B)

For ACC/AHA Level of evidence and classes click:ACC AHA Guidelines Classification Scheme

Acknowledgements and Initial Contributors to Page
Leida Perez, M.D.