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Tex Heart Inst J. 2000; 27(2): 193–195.
PMCID: PMC101054

Neonatal Arterial Switch Operation for Transposition of the Great Arteries in a Patient with Mirror Image Dextrocardia and Situs Inversus Totalis

Abstract

The neonatal arterial switch operation has become the standard therapy for D-transposition of the great arteries in the absence of left ventricular outflow tract obstruction. We describe our experience of successful arterial switch operation after balloon atrial septostomy in a 5-day-old infant girl who had atrial and visceral situs inversus totalis, mirror image dextrocardia, and D-transposition of the great arteries. To our knowledge, ours is the first report of this operation in a patient with this anatomy.

Key words: Arterial switch operation, dextrocardia, situs inversus, transposition of the great vessels

Surgical management of D-transposition of the great arteries (D-TGA) has evolved over several decades under the influence of a few key surgical pioneers. 1–3 Definitive anatomical correction, however, was not described until 1975 with the advent of Jatene's arterial switch operation (ASO). 4 Anatomical correction was intended to avoid the long-term complications of a systemic right ventricle and the complex atrial arrhythmias inherent to the Mustard and Senning operations. 5 The arterial switch operation has been well described in neonates with atrial and visceral situs solitus. 6 We describe a patient with atrial and visceral situs inversus totalis, mirror image dextrocardia, and D-TGA who underwent successful neonatal arterial switch operation following balloon atrial septostomy. To the best of our knowledge, the arterial switch operation has not been described previously in a patient with this anatomy.

Case Report

In March 1999, 3.06 kg girl was born at term to a 25-year-old mother (gravida 2, para 2) via spontaneous vaginal delivery. Apgar scores were 9 and 9 at 1 and 5 minutes. Physical examination revealed a moderately cyanosed neonate with baseline oxygen saturation of 70%. Vital signs were otherwise normal. The lungs were clear to auscultation, and respiratory effort was unlabored. Palpation of the precordium revealed a ventricular tap at the right lower sternal border and the apex beat at the 5th intercostal space in the right mid-clavicular line. Auscultation of the right chest revealed a normal 1st heart sound and a single loud 2nd heart sound. No murmurs, clicks, or gallops were noted. The liver was palpable 1 cm below the left costal margin. The pulses were normal in volume and character.

Chest radiography demonstrated dextrocardia, cardiomegaly (cardiothoracic ratio 0.75), a right aortic arch, and clear lung fields with normal pulmonary vascular markings (Fig. 1). A gastric air bubble was noted on the right side, and a liver shadow was visible on the left side. The electrocardiogram revealed a low left atrial rhythm and dextrocardia (Fig. 2). Abdominal ultrasound demonstrated the stomach and spleen on the right side and the liver on the left. The aorta and the inferior vena cava were inverted. Echocardiography demonstrated dextrocardia, atrial situs inversus, atrioventricular concordance, and ventriculo-arterial discordance (I,L,L). There was bidirectional shunting through a moderate-size (5-mm) nonrestrictive perimembranous ventricular septal defect, and there was left-to-right shunting through a patent foramen ovale. Continuous low-velocity left-to-right shunting due to a large patent ductus arteriosus was also present, and the aortic arch was on the right. The coronary artery pattern was mirror image Yacoub type A.

figure 17FF1
Fig. 1 Chest radiography demonstrates dextrocardia, cardiomegaly, and a gastric air bubble on the right side.
figure 17FF2
Fig. 2 Electrocardiogram demonstrates low left atrial rhythm and dextrocardia.

The patient was electively intubated and a prostaglandin infusion was initiated. On day of life 2, she underwent balloon atrial septostomy to improve mixing at the atrial level and to reduce left atrial hypertension (Figs. 3 and 4). Oxygen saturation improved to 85% in room air following the procedure, and the prostaglandin was discontinued without change in oxygen saturations. At this point, she was transported to Texas Children's Hospital for further management.

On day of life 5, the patient underwent arterial switch operation. Under full-flow cardiopulmonary bypass, the aorta and pulmonary artery were transected, a Lecompte maneuver was performed, and the coronary arteries were re-anastomosed to the aorta in the standard fashion. The atrial and ventricular septal defects were patched using a transatrial approach. Postoperative transesophageal echocardiography demonstrated no new obstruction in the aorta or pulmonary artery, and no residual shunting at the atrial or ventricular level. The patient returned from the operating room on dopamine (3 μg/kg/min) and minimal ventilation settings. She had an uneventful postoperative course, during which there was no disturbance in cardiac rhythm, blood pressure, or perfusion, and no ST segment change. She was extubated within 36 hours and weaned from hemodynamic support by postoperative day 2. On postoperative day 5, she was discharged to her referral hospital.

Discussion

D-transposition of the great arteries is the most common cyanotic congenital heart lesion to present in the 1st week of life and affects between 2.6% and 7.8% of infants who have congenital heart disease. 6 It is commonly associated with ventricular septal defect (50% of D-TGA cases), atrial septal defect (20%), atrioventricular valve abnormalities (10%), arch obstruction (10%), abnormal coronary artery patterns (5%), and subaortic or subpulmonary stenosis (5%). 7

Transthoracic echocardiography alone is generally sufficient to delineate the anatomy and coronary artery branching pattern, thereby avoiding the need for a diagnostic catheterization. Balloon atrial septostomy, introduced by Rashkind 8 in 1966, revolutionized management of these patients and can be performed in the catheterization laboratory or by the bedside.

The arterial switch operation has become the standard surgical repair for D-TGA in the absence of left ventricular outflow tract obstruction. The current mortality risk for neonatal ASO is less than 5%, even in the presence of a complex coronary arrangement. 9 At Texas Children's Hospital, we recommend that all patients undergo balloon atrial septostomy prior to surgery in order to achieve optimal atrial level mixing, reduce left atrial hypertension, and minimize preoperative mortality. The optimal timing of arterial switch operation is between day of life 7 and 28, before the pulmonary vascular resistance falls and while the left ventricle remains capable of sustaining a systemic workload.

Establishment of unimpeded coronary blood flow remains the most crucial intraoperative factor in achieving successful arterial switch. Adverse outcome in the early experience with arterial switch was often attributed to impeded flow. As surgeons have gained experience with coronary artery transfer and greater awareness of coronary artery patterns over the past 10 years, morbidity and mortality rates have fallen substantially. 10 The highest mortality rates have traditionally been seen in patients who have intramural coronary patterns. Recently, transferring a single coronary button that houses both coronary ostia—and unroofing the intramural coronary to enable a 2-button repair—have greatly improved outcome in this group. 9,11 Interestingly, coronary artery pattern has become less of a concern, and poor outcomes have been attributed to poor preoperative ventricular function. 9

In conclusion, the success of early neonatal arterial switch operation for D-TGA has been replicated with low mortality at several centers. 6,10,12 We have presented a report of successful neonatal arterial switch in a patient with atrial and visceral situs in-versus totalis, dextrocardia, atrioventricular concordance, and ventriculo-arterial discordance (I,L,L). To our knowledge, ours is the first report of this operation in a patient with this anatomy.

Footnotes

Address for reprints: Charles D. Fraser, Jr., MD, Chief, Division of Pediatric Cardiovascular Surgery, Texas Children's Hospital, MC 2-2280, Houston, TX 77030

References

1. Blalock A, Hanlon CR. The surgical treatment of complete transposition of the aorta and the pulmonary artery. Surg Gynecol Obstet 1950;90:1–15. [PubMed]
2. Mustard WT, Chute AL, Keith JD, Sirek A, Rowe RD, Vlad P. A surgical approach to transposition of the great vessels with extracorporeal circuit. Surgery 1954;36:39–51. [PubMed]
3. Senning A. Surgical correction of transposition of the great vessels. Surgery 1959;45:966–80. [PubMed]
4. Jatene AD, Fontes VF, Paulista PP, de Souza LC, Neger F, Galantier M, Sousa JE. Successful anatomic correction of transposition of the great vessels. A preliminary report. Arq Bras Cardiol 1975;28:461–4. [PubMed]
5. Gewillig M, Cullen S, Mertens B, Lesaffre E, Deanfield J. Risk factors for arrhythmia and death after Mustard operation for simple transposition of the great arteries. Circulation 1991;84(5 Suppl):III187–92. [PubMed]
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7. Neches WH, Park SC, Ettedgui JA. Transposition of the great arteries. In: Garson A Jr, Bricker JT, Fisher DJ, Neish SR, editors. The science and practice of pediatric cardiology. Vol. 1. Baltimore: Williams & Wilkins, 1998:1463–1500.
8. Rashkind WJ, Miller WW. Creation of an atrial septal defect without thoracotomy. A palliative approach to complete transposition of the great arteries. JAMA 1966;196: 991–2. [PubMed]
9. Planche C, Lacour-Gayet F, Serraf A. Arterial switch. Pediatr Cardiol 1998:19:297–307. [PubMed]
10. Brawn WJ, Mee RB. Early results for anatomic correction of transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect. J Thorac Cardiovasc Surg 1988;95:230–8. [PubMed]
11. Asou T, Karl TR, Pawade A, Mee RB. Arterial switch: translocation of the intramural coronary. Ann Thorac Surg 1994;57:461–65. [PubMed]
12. Planche C, Bruniaux J, Lacour-Gayet F, Kachaner J, Binet JP, Sidi D, et al. Switch operation for transposition of the great arteries in neonates. A study of 120 patients. J Thorac Cardiovasc Surg 1988;96:354–63. [PubMed]

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