Authors: Amelia Sheppard, MD, Peggy Vogt, MD, and Stephanie Grant, MD – Emory University/Children’s Healthcare of Atlanta
A one-week-old female with postnatal diagnosis d-Transposition of the Great Arteries (d-TGA) with severe left ventricular outflow tract obstruction (LVOTO) and a remote restrictive ventricular septal defect (VSD) successfully underwent a balloon atrial septostomy. Given the patient's anatomic diagnosis, what is the MOST appropriate future surgical plan?
EXPLANATION
Transposition of the great arteries (TGA) is one of the most common cyanotic congenital heart disease states that presents in neonates. However d-TGA with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO), also called TGA with VSD and pulmonic stenosis (PS), occurs in less than 1% of all congenital heart defects.1,2 d-TGA-VSD-LVOTO cannot be repaired with the classic arterial switch operation because it would convert severe pulmonic stenosis to severe neo-aortic stenosis.3 Some patients with minor LVOTO may undergo an arterial switch operation with LVOT resection, but those with fixed and severe obstruction are typically repaired by the Rastelli, Réparation a l’Etage Ventriculaire (REV), or Nikaidoh procedures.2
The Rastelli operation was introduced in 1969.2 It involves creation of an intracardiac baffle from the VSD to the aortic valve, connecting the left ventricle to the aortic valve and systemic circulation.2 The Rastelli is performed through a right ventriculotomy.2 The pulmonary artery is divided, and an extracardiac valved conduit is placed to connect the right ventricle to the pulmonary artery.2 The Rastelli establishes the mitral valve and left ventricle as part of the systemic circulation.2 Early complications of the Rastelli include recurrent LVOTO due to accumulation of fibrotic tissue, and failure of the VSD to enlarge with the patient's somatic growth.4 Additionally, the intracardiac baffle can reduce right ventricular volume, which when combined with the RV anterior wall defect from placement of the valved conduit, can contribute to RV dysfunction.5 Late complications of the Rastelli include conduit replacement due to patient somatic growth or conduit calcification, LVOTO, arrhythmias, and poor long-term survival.4,5
The REV procedure involves similar left ventricular baffling to the aorta as well as conal septal resection and a Lecompte maneuver to translocate the pulmonary artery to the right ventricle instead of a homograft conduit.2
In 1984, Nikaidoh introduced the aortic root translocation and biventricular outflow tract reconstruction technique.3 The right ventricular outflow tract (RVOT) is reconstructed with a conduit or transannular patch.1,3 If the great vessels are oriented in an anteroposterior position and there is a small pulmonary annulus, then the aortic root and coronary arteries are translocated en bloc, avoiding coronary reanastomosis.1,3 Care must be taken to avoid tension or kinking of the coronary arteries, which is one of the greatest risks of the procedure.5
The major advantages of the Nikaidoh are an anatomically aligned LVOT, positioning the aortic valve with the high pressure systemic circulation, and orthotopic position of the RV-PA conduit.1 Drawbacks of the Nikaidoh are its greater technical challenge, longer bypass and aortic cross-clamp times and longer postoperative ventilator requirements.5 Midterm follow-up among various patient series shows few to none experience LVOTO or aortic insufficiency.1,3,5 Overall the operation allows better mid-term survival and fewer reoperations.1
In the above question, d-TGA with a remote, restrictive VSD and severe LVOTO is best surgically repaired with the Nikaidoh procedure.1 An arterial switch operation would address the ventriculo-arterial discordance but not the LVOTO. VSD closure alone in the above scenario would not address VA discordance or LVOTO, and the remote location of the VSD would require an extensive intraventricular baffle, making this patient a poor candidate for a Rastelli.
REFERENCES
1. Agarwal V, Vaidyanathan S. Aortic root translocation: the Bex-Nikaidoh procedure. Indian J Thorac Cardiovasc Surg. 2021;37(Suppl 1):36-41. doi:10.1007/s12055-020-00956-7
2. Qureshi AM, Justino H and Heinle JS. Transposition of the Great Arteries. In: Shaddy RE, Penny DJ, Feltes TF, et al, eds. Moss & Adams’ Heart Disease in Infants, Children, and Adolescents. 10th ed. Wolters Kluwer Health; 2022: 2303-2351
3. Nikaidoh H. Aortic translocation and biventricular outflow tract reconstruction. A new surgical repair for transposition of the great arteries associated with ventricular septal defect and pulmonary stenosis. J Thorac Cardiovasc Surg. 1984;88(3):365-372.
4. Kreutzer C, De Vive J, Oppido G, et al. Twenty-five-year experience with Rastelli repair for transposition of the great arteries. J Thorac Cardiovasc Surg. 2000;120(2):211-223. doi:10.1067/mtc.2000.108163
5. Hu SS, Liu ZG, Li SJ, et al. Strategy for biventricular outflow tract reconstruction: Rastelli, REV, or Nikaidoh procedure?. J Thorac Cardiovasc Surg. 2008;135(2):331-338. doi:10.1016/j.jtcvs.2007.09.060
5. Hu SS, Liu ZG, Li SJ, et al. Strategy for biventricular outflow tract reconstruction: Rastelli, REV, or Nikaidoh procedure?. J Thorac Cardiovasc Surg. 2008;135(2):331-338. doi:10.1016/j.jtcvs.2007.09.060