Transposition of the Great Arteries Treatment & Management

Updated: Apr 11, 2017
  • Author: John R Charpie, MD, PhD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Treatment

Medical Care

Initial treatment of transposition of the great arteries consists of maintaining ductal patency with continuous intravenous (IV) prostaglandin E1 (PgE1) infusion to promote pulmonary blood flow, increase left atrial pressure, and promote left-to-right intercirculatory mixing at the atrial level. This is particularly important in patients with severe left ventricular outflow tract stenosis or atresia. Administration of PgE1 within the first 48 hours after birth is crucial to reduce early mortality in newborns with transposition of the great arteries, especially in the simple form. [10]

Cardiac catheterization and balloon atrial septostomy is indicated in severely hypoxemic patients with an inadequate atrial level communication and insufficient mixing (preductal saturations significantly lower than postductal saturations). Balloon atrial septostomy is used to increase the atrial level shunt and to improve mixing.

For the ill neonate, metabolic acidosis should be corrected with fluid replacement and bicarbonate administration.

Mechanical ventilation may be necessary if pulmonary edema develops in concert with severe hypoxemia.

Ultimately, the patient requires surgical repair or palliation early in life.

Consultations

Consult with a pediatric cardiologist and a pediatric cardiothoracic surgeon.

Transfer

Transfer is required for specialized diagnostic, therapeutic, and surgical interventions.

Diet and activity

Patients with transposition of the great arteries and a large ventricular septal defect who have not undergone repair may require increased caloric density during infancy (120-130 kcal/kg/d), particularly if they have significant congestive heart failure and poor weight gain.

Following definitive repair, most patients do not need a special diet.

Specific activity restrictions are dependent on the patient's residual hemodynamic abnormalities. Following the arterial switch procedure, exercise stress testing is necessary in older patients who are interested in participating in competitive sports.

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Surgical Care

Surgical approach depends on the age of the patient at presentation, the presence of associated congenital cardiac lesions, and the experience of the cardiothoracic surgeon with a given surgical technique. Most full-term neonates with uncomplicated transposition of the great arteries can undergo an arterial switch procedure in one operation, with minimal mortality.

Transposition of the great arteries with intact ventricular septum

The ideal operation is an arterial switch procedure. It represents an anatomic repair and establishes ventriculoarterial concordance. This procedure should be performed when the infant is younger than 4 weeks, as the left ventricle may not be able to handle systemic pressure postoperatively if left too long in the low-pressure, low-resistance pulmonary circuit. Rarely, however, depending on the particular coronary artery anatomy (eg, intramural coronary artery), coronary artery translocation may not be feasible, and an arterial switch is not recommended. In this subgroup, an atrial level switch (Senning or Mustard procedure) has lower surgical and short-term morbidity and mortality.

Transposition of the great arteries with ventricular septal defect

The preferred operation is an arterial switch procedure with ventricular septal defect closure (see the videos below). If the ventricular septal defect is large and nonrestrictive and coronary artery anatomy makes an arterial switch operation inadvisable, a Rastelli-type intracardiac repair may be feasible. With the Rastelli-type procedure, waiting until the infant is older and larger may be preferred because of the need for a right ventricle–pulmonary artery conduit in the Rastelli operation. If the infant has excessive congestive heart failure (with growth failure), it may be advisable to either proceed with reparative surgery or, if not feasible, band the main pulmonary artery during the newborn period to restrict pulmonary blood flow.

This video shows the repair of a newborn with transposition of the great arteries and ventricular septal defect (VSD) by means of arterial switch and VSD closure. Procedure performed by Giles Peek MD, FRCS, CTh, FFICM, The Children’s Hospital at Montefiore, Bronx, NY. Video courtesy of Montefiore.
Switch ventricular septal defect (VSD hypoplastic right arch). Procedure performed by Giles Peek MD, FRCS, CTh, FFICM, The Children’s Hospital at Montefiore, Bronx, NY. Video courtesy of Montefiore.

Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction

An arterial switch operation may not be feasible due to pulmonary valve (left ventricular outflow tract) stenosis or atresia. If the ventricular septal defect is nonrestrictive and remote from the aorta, a Rastelli intracardiac repair could be possible. Because the Rastelli procedure necessitates a conduit from the right ventricle to the pulmonary artery, delaying repair until the infant is older and larger may be preferable. In this case, placing an aortopulmonary shunt during the newborn period may be necessary to establish adequate pulmonary blood flow while waiting.

Transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease

These patients might not be appropriate surgical candidates because of the progressive increase in pulmonary vascular resistance. This is a small subgroup of patients whose conditions are not often diagnosed until after a palliative or reparative procedure is performed.

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Long-Term Monitoring

Evidence from the Boston Circulatory Arrest Trial suggests that neurodevelopmental outcomes for children with dextro-transposition of the great arteries (d-TGA) who undergo arterial switch operation (and other complex neonatal operations) may not be normal and may require further investigation and follow-up. [11]

Many patients do not require any specific medications. Possible discharge medications might include digoxin, furosemide, or both.

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