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Victor Morell, MD
Transposition of the great arteries (TGA) is a congenital heart defect resulting from abnormal development during the first eight weeks of pregnancy. The anatomic connections between the heart and the major arteries that exit its ventricles are reversed from their normal configuration; the aorta is connected to the right ventricle and the pulmonary artery is connected to the left ventricle.
Poorly oxygenated blood is pumped back out to the body by the right ventricle, through the incorrectly attached aorta, without being oxygenated by the lungs. Blood circulating through the left side of the heart is repeatedly sent to the lungs without returning to the systemic circulation.
A ventricular septal defect (VSD) often accompanies TGA, which is advantageous for neonatal survival because it provides some mixing of oxygenated and non-oxygenated blood prior to returning to the systemic circulation. TGA can also be accompanied by a left ventricular outflow tract obstruction (LVOTO), a narrowing below the pulmonary valve that blocks blood flow from the left ventricle to the pulmonary arteries. The primary symptom of TGA in newborns is cyanosis. TGA makes up 3% of congenital heart defects. Complex TGA with VSD and LVOTO comprise only 0.67% of all congenital heart defects and present a unique surgical challenge.1
Our well-established program in the Division of Pediatric Cardiothoracic Surgery within the UPMC Department of Cardiothoracic Surgery performs surgical interventions for congenital heart defects and a full range of congenital and acquired heart and lung disorders in neonates, infants, children, and adults, with outcomes that are among the best in the United States. We are experts in the surgical correction of complex TGA.
Surgical correction of complex TGA with VSD and LVOTO can be accomplished with either the Rastelli procedure or the Nikaidoh procedure, an arterial switch procedure. At UPMC Children’s Hospital of Pittsburgh, we prefer the Nikaidoh procedure and, within our program, have worked to establish the safety, efficacy, and durability of this surgery. We also teach this technique to others.
The Nikaidoh procedure results in a more acceptable anatomic repair than other approaches and preserves the right ventricular volume. The technique of aortic translocation was first described by Hisashi Nikaidoh, MD, in 1984. However, it was largely underutilized until 2004 when I presented a series of 12 patients on whom I had performed the procedure at the 50th Annual Meeting of the Southern Thoracic Surgical Association. I also presented the aortic translocation procedure at the World Congress of Pediatric Cardiology and Cardiac Surgery later the same year. In 2005, we published a paper in the Annals of Thoracic Surgery detailing our modification of Dr. Nikaidoh’s technique and its results and outcomes on the 12 patients1, becoming one of the most cited papers on Nikaidoh procedure. I have visited institutions across the U.S. and worldwide to lecture and teach the procedure to other surgeons.
The Nikaidoh procedure is especially useful in patients with an inlet or restrictive VSD, a small right ventricle, a straddling atrioventricular valve, or anomalous coronary anatomy that prevents a distal right ventriculotomy. Surgeons can also perform this procedure to treat patients with a double-outlet right ventricle with malposition of the great arteries and LVOTO. During the last decade, the Nikaidoh procedure has gained popularity among congenital cardiac surgeons for the management of patients with complex transposition. In the most recent report from The Society of Thoracic Surgery Congenital Database (June 2021)2, approximately 40% (n= 119) of the patients with TGA, VSD, and LVOTO or DORV-TGA that underwent a biventricular repair were managed with this aortic translocation procedure.
Based on my experience performing the Nikaidoh procedure on more than 45 patients with outstanding durability and excellent outcomes, it is important to have a clear understanding of the cardiac anatomy before the start of the operation, and to recognize which factors preclude this type of repair.3 The surgeon must be aware of the spatial relationship of the great vessels, the size and location of the VSD, the coronary anatomy, the size of the pulmonary annulus, the presence of abnormal atrioventricular valve attachments, and the right ventricular size. Surgical palliation with a systemic-to-pulmonary-artery shunt or the creation of an atrial septal defect often precedes surgical correction of TGA/VSD/LVOTO, and the surgeon must be knowledgeable of previous interventions. After the chest and pericardium are opened, the surgeon must again perform a careful assessment of the patient’s anatomy and any previous interventions to verify that the Nikaidoh procedure is likely to successfully ameliorate the patient’s congenital defect.
After cardiopulmonary bypass is initiated, the procedure begins with extensive mobilization of the proximal coronary arteries. Under mild-to-moderate hypothermia and after cross-clamping the aorta, the aortic root is harvested from the right ventricle. The pulmonary artery is then transected proximally, and the conal septum is divided down to the level of the VSD, thus relieving the LVOTO. The aortic root and coronary arteries are translocated en bloc posteriorly, and the LVOTO reconstruction is completed by suturing the VSD patch. The ascending aorta is divided so that the distal pulmonary artery and its branches can be brought forward (the Lecompte maneuver), and the aortic root is then re-anastomosed.
Next, we make a longitudinal incision in the anterior wall of the main pulmonary artery, which is usually hypoplastic, and suture the posterior wall to the posterior aspect of the right ventricular outflow tract (RVOT); reconstruction is then completed using an anterior pericardial patch.
The Nikaidoh procedure has become the preferred surgical technique for managing patients at UPMC Children’s Hospital of Pittsburgh whenever it is indicated. Published experiences using the Nikaidoh technique are limited to high-volume centers, where UPMC has become a national and international referral site for patients who require this complex procedure. Encouraging early and midterm results suggesting better outcomes compared with the Rastelli repair1,2,4,5 have prompted the utilization of this procedure over the years, with good long-term outcomes for the patients.
We are proud to continue our long tradition of surgical excellence and innovation to impact the lives of children with congenital heart defects positively.
To learn more about the UPMC Heart and Vascular Institute or to refer a patient, call 412-770-4949. To learn more about the Heart Institute at UPMC Children's Hospital of Pittsburgh, click here.
1. Morell VO, Jacobs JP, Quintessenza JA. Aortic translocation in the management of transposition of the great arteries with ventricular septal defect and pulmonary stenosis: results and follow-up. Ann Thorac Surg. 2005;79(6):2089-92; discussion 2092-3.
2. Seese L, Turbendian HK, Thibault D, Da Fonseca Da Silva L, Hill K, Castro-Medina M, Viegas M, Da Silva JP, Jacobs JP, Jacobs ML, Shillingford M, Morell VO, Utilization and Outcomes of the Nikaidoh, Rastelli, and REV Procedures: An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann Thorac Surg. 2021.
3. Morell VO. Nikaidoh Procedure: How I Teach It. Ann Thorac Surg. 2017;104(5):1446-9.
4. Nikaidoh H. Nikaidoh procedure: a perspective. Eur J Cardiothorac Surg. 2016;50:1001-5.
5. Yeh T, Jr., Ramaciotti C, Leonard SR, Roy L, Nikaidoh H. The aortic translocation (Nikaidoh) procedure: midterm results superior to the Rastelli procedure. J Thorac Cardiovasc Surg. 2007;133(2):461-9.