Dextro-transposition of the great arteries corrective surgery
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Cafer Zorkun, M.D., Ph.D. [3]; Keri Shafer, M.D. [4]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [5]
Overview
Overview
Recent advances in surgical correction of transposition of the great arteries have reduced the mortality drastically from 95% in uncorrected patients to 5% in corrected patients[1].
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General features
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Cafer Zorkun, M.D., Ph.D. [3]; Keri Shafer, M.D. [4]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [5]
Overview
Corrective Surgery
Arterial switch operations (ASO) are now-days preferred over Atrial switch procedures (Mustard and Senning Operations) because of the following reasons-
- Arterial switch procedure are comparatively easier to perform
- ASO have similar survival benefits compared to atrial switch procedure
- Decreased risk of complications like arrhythmias and heart failure in ASO compared to atrial switch procedures
- Decreased peri-operative mortality in ASO compared to atrial switch procedures
Salient features of surgery are
- It is best to perform the ASO as early as possible in the children. Most infants undergo definitive repair within the first 2 weeks of life.
- The type of surgical procedure done depends on the type of lesion the child has i.e. simple or complex transposition of the great arteries.
- The general rules that are followed are-
- Simple D-TGA (D-TGA without any associated lesion)- Arterial switch operation (ASO).
- D-TGA plus ventricular septal defect-Arterial switch operation (ASO) and VSD closure.
- D-TGA plus large VSD plus pulmonary stenosis – Rastelli procedure
References
Acknowledgements and Initial Contributors to Page
Leida Perez, M.D.
Arterial switch or Jatene Operation
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Cafer Zorkun, M.D., Ph.D. [3]; Keri Shafer, M.D. [4]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [5]
Overview
Arterial switch or Jatene Operation
- The successful anatomical correction of TGA was first described in 1975 by Jatene et al.
- It has become the preferred procedure for most of the D-TGA (see above for the conditions)
- An arterial switch operation involves the following steps-
- The baby will be placed under general anesthesia.
- The heart and vessels are accessed via median sternotomy.
- The heart/lung machine (cardiopulmonary bypass machine) is connected.
- Since, the heart/lung machine needs its “circulation” to be filled with blood, a child will require a blood transfusion for this surgery.
- The patient is cooled for 20 minutes to 20 Celsius degree rectal temperature.
- Once the heart is stopped and emptied, the aorta and the pulmonary artery are divided.
- The site of the aortic transection is marked before the cross clamp is applied.
- The aorta and pulmonary artery are transected at a level above the valve sinuses.
- The ostium of the coronary arteries are excised along with a large segment of surrounding aortic wall and sutured into place in the neo-aorta (basal part of the pulmonary artery).
- The pulmonary trunk is moved forward into its new position anterior to the aorta.
- Finally, the switched great arteries are sutured into place.
- The heart is then allowed to fill and take over its normal function. Temporary pacemaker wires and drainage tubes are then placed and the chest is closed.
- Some arterial switch recipients may present with post-operative pulmonary stenosis, which would then be repaired with angioplasty, pulmonary stenting via heart cath or median sternotomy, and/or xenograft.
- Lecompte maneuver – During this surgery the bifurcation of the pulmonary arteries are placed anterior to the aorta. This helps in straddling the ascending aorta to the left and right pulmonary arteries. This reduces the tension that could be there due to anterio translocation of the pulmonary arterial root. This has helped to decrease the pulmonary artery stenosis that may occur as an complication of ASO.
ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[1][2]
Imaging for Dextro-Transposition of the Great Arteries After Arterial Switch Operation (DO NOT EDIT)[1][2]
| Class I |
|
“1. 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)” |
|
“2. 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 |
|
“1. Periodic MRI or CT can be considered appropriate to evaluate the anatomy and hemodynamics in more detail. (Level of Evidence: C)” |
| Class IIa |
|
“1. Coronary angiography is reasonable in all adults with d-TGA after ASO to rule out significant coronary artery obstruction. (Level of Evidence: C)” |
References
- ↑ 1.0 1.1 1.2 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). “ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease)”. Circulation. 118 (23): 2395–451. doi:10.1161/CIRCULATIONAHA.108.190811. PMID 18997168.
- ↑ 2.0 2.1 2.2 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). “ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons”. J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.
Acknowledgements and Initial Contributors to Page
Leida Perez, M.D.
Atrial switch repair
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Cafer Zorkun, M.D., Ph.D. [3]; Keri Shafer, M.D. [4]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [5]
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 [1](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[1](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:
|
” |
(ACC/AHA) Recommendation for Interventional Catheterization for Adults with Repaired Dextro-Transposition of the Great Arteries[1](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:
|
” |
For ACC/AHA Level of evidence and classes click:ACC AHA Guidelines Classification Scheme
References
- ↑ 1.0 1.1 1.2 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). “ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons”. J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.
Acknowledgements and Initial Contributors to Page
Leida Perez, M.D.
Rastelli operation
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Cafer Zorkun, M.D., Ph.D. [3]; Keri Shafer, M.D. [4]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [5]
Overview
- Done for patients with TGA, VSD, and pulmonary outflow tract obstruction.
- It depends on appropriate VSD anatomy (large and subaortic) because then it will be used as part of the left ventricular outflow tract (LVOT), involving placement of a baffle within the RV to direct blood flow from the VSD to the aorta. A conduit is inserted between the RV and the pulmonary artery, which is stitched.
- Advantage- Left Ventricle becomes the systemic ventricle
- Disadvantage- The conduit will likely need to be replaced several times during the patient’s life.
- The appropriate age for this operation is still debated, due to the higher risk with the early repair.
- The younger the patient the smaller the conduit, needing earlier reoperation.
(ACC/AHA) Recommendation for Diagnostic Catheterization for Adults With Repaired Dextro-Transposition of the Great Arteries[1](DONOT EDIT)
| “ |
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: 1. Coronary artery delineation before any intervention for right ventricular outflow tract (RVOT) obstruction. (Level of Evidence: C) 2. Assessment of residual VSD. (Level of Evidence: C) 3. Assessment of PAH, with potential for vasodilator testing. (Level of Evidence: C) 4. Assessment of subaortic obstruction across the left ventricle-to-aorta tunnel. (Level of Evidence: C) |
” |
(ACC/AHA) Recommendation for Interventional Catheterization for Adults with Repaired Dextro-Transposition of the Great Arteries[1](DONOT EDIT)
| “ |
1. For adults with d-TGA, VSD, and PS, after Rastelli-type repair, interventional catheterization can be beneficial to assist in the following:
|
” |
For ACC/AHA Level of evidence and classes click:ACC AHA Guidelines Classification Scheme
References
- ↑ 1.0 1.1 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). “ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons”. J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.
Acknowledgements and Initial Contributors to Page
Leida Perez, M.D.
ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[2][3]
ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[2][3]
Evaluation of the Operated Patient With Dextro-Transposition of the Great Arteries (DO NOT EDIT)[2][3]
| Class I |
| “1. Patients with repaired d-TGA should have annual follow-up with a cardiologist who has expertise in the management of ACHD patients. (Level of Evidence: C)” |
| Class I |
| “1. Surgeons with training and expertise in congenital heart disease (CHD) should perform operations in patients with d-TGA and the following indications: |
| a. Moderate to severe systemic (morphological tricuspid) AV valve regurgitation without significant ventricular dysfunction. (Carrel & Pfammatter, 2000) (Level of Evidence: B) |
| 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) |
| c. Superior vena cava or inferior vena cava obstruction not amenable to percutaneous treatment. (Level of Evidence: B) |
| d. Pulmonary venous pathway obstruction not amenable to percutaneous intervention. (Level of Evidence: B) |
| e. Symptomatic severe subpulmonary stenosis. (Level of Evidence: B)” |
| Class I |
| “1. It is recommended that surgery be performed in patients after the ASO with the following indications: |
| a. 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) |
| b. Coronary artery abnormality with myocardial ischemia not amenable to percutaneous intervention. (Level of Evidence: C) |
| c. Severe neoaortic valve regurgitation. (Level of Evidence: C) |
| d. 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)” |
| Class I |
| “1. Reoperation for conduit and/or valve replacement after Rastelli repair of d-TGA is recommended in patients with the following indications: |
| a. Conduit obstruction peak-to-peak gradient greater than 50 mm Hg. (Level of Evidence: C) |
| b. RV/LV pressure ratio greater than 0.7. (Level of Evidence: C) |
| c. Lesser degrees of conduit obstruction if pregnancy is being planned or greater degrees of exercise are desired. (Level of Evidence: C) |
| d. Subaortic (baffle) obstruction (mean gradient greater than 50 mm Hg). (Level of Evidence: C) |
| e. 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) |
| f. Presence of concomitant severe aortic regurgitation (AR). (Level of Evidence: C)” |
| “2. Reoperation for conduit regurgitation after Rastelli repair of d-TGA is recommended in patients with severe conduit regurgitation and the following indicators: |
| a. Symptoms or declining exercise tolerance. (Level of Evidence: C) |
| b. Severely depressed RV function. (Level of Evidence: C) |
| c. Severe RV enlargement. (Level of Evidence: C) |
| d. Development/progression of atrial or ventricular arrhythmias. (Level of Evidence: C) |
| e. More than moderate tricuspid regurgitation (TR). (Level of Evidence: C)” |
| “3. 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)” |
| “4. Surgical closure of residual VSD in adults after Rastelli repair of d-TGA is recommended with the following indicators: |
| a. Pulmonary blood flow/systemic blood flow (Qp/Qs) greater than 1.5:1. (Level of Evidence: B) |
| b. Systolic pulmonary artery pressure greater than 50 mm Hg. (Level of Evidence: B) |
| c. Increasing LV size from volume overload. (Level of Evidence: C) |
| d. Decreasing RV function from pressure overload. (Level of Evidence: B) |
| e. RVOT obstruction (peak instantaneous gradient greater than 50 mm Hg). |
| f. 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)” |
| “5. 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)” |
| “6. 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 |
| “1. 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)” |
References
References
- ↑ Hutter PA, Kreb DL, Mantel SF, Hitchcock JF, Meijboom EJ, Bennink GB (2002). “Twenty-five years’ experience with the arterial switch operation”. J Thorac Cardiovasc Surg. 124 (4): 790–7. PMID 12324738.
- ↑ 2.0 2.1 2.2 2.3 2.4 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). “ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease)”. Circulation. 118 (23): 2395–451. doi:10.1161/CIRCULATIONAHA.108.190811. PMID 18997168.
- ↑ 3.0 3.1 3.2 3.3 3.4 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). “ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons”. J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.
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