eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Transposition of the Great Arteries: Treatment & Medication
Updated: Jun 11, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Initial treatment consists of maintaining ductal patency with continuous intravenous (IV) prostaglandin E1 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. Prostaglandin therapy may or may not benefit the patient with simple transposition of the great arteries (TGA) and an intact ventricular septum without left ventricular outflow tract obstruction.
- Cardiac catheterization, depending on the degree of restriction at the atrial septum and the timing of operative repair, is indicated for a balloon atrial septostomy in severely hypoxemic patients with an inadequate atrial level communication and insufficient mixing. The 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.
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.
- The ideal operation is an arterial switch procedure.
- Transposition of the great arteries with ventricular septal defect
- The preferred operation is an arterial switch procedure with ventricular septal defect closure.
- 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/ligate the main pulmonary artery and place an aortopulmonary shunt during the newborn period to restrict pulmonary blood flow.
- 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 (left ventricular outflow tract) stenosis or atresia.
- If the ventricular septal defect is nonrestrictive and not too 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.
Consultations
- Pediatric cardiologist
- Pediatric cardiothoracic surgeon
Diet
- 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.
Activity
- No specific activity requirements are necessary.
Medication
Transposition of the great arteries (TGA) has no specific or recommended drug therapies. Newborn infants with transposition of the great arteries (particularly those with severe left ventricular outflow tract obstruction) may derive some initial benefit from alprostadil (ie, prostaglandin E1) therapy. Patients with transposition of the great arteries and ventricular septal defect who have not undergone surgical repair, and some patients following complete repair, might potentially benefit from digoxin and diuretic therapy to improve systemic ventricular function and avoid fluid retention. All patients require antibiotic prophylaxis prior to dental and indicated surgical procedures in order to reduce the risk of subacute bacterial endocarditis. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.
Inotropic agents
These drugs increase the contractility of cardiac muscle in a dose-dependent manner (ie, positive inotropic effect).
Digoxin (Lanoxin)
Frequently used cardiac glycoside that inhibits the sarcolemmal sodium-potassium adenosine triphosphatase, which leads to an increase in intracellular calcium concentration and increased myocardial contractility.
Adult
0.125-0.5 mg PO qd
Pediatric
Preterm infant: 5-7.5 mcg/kg/d PO divided bid
Term infant: 6-10 mcg/kg/d PO divided bid
1 month to 2 years: 10-15 mcg/kg/d PO divided bid
2-5 years: 7.5-10 mcg/kg/d PO divided bid
5-10 years: 5-10 mcg/kg/d PO divided bid
>10 years: 2.5-5 mcg/kg PO qd
IV calcium may produce arrhythmias in digitalized patients; medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, PO amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil
Medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, PO colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid
Documented hypersensitivity, atrioventricular block, idiopathic hypertrophic subaortic stenosis, constrictive pericarditis, hypokalemia, renal failure
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Monitor serum potassium levels and use cautiously with hypokalemia; monitor serum digoxin level due to narrow therapeutic index; reduce dose in renal dysfunction; CNS effects, such as drowsiness, and GI effects, such as nausea and vomiting, are some of the more common adverse drug reactions; digoxin can cause cardiac arrhythmias; patients are predisposed to digoxin toxicity with hypokalemia, hypomagnesemia, hypercalcemia, and hypermagnesemia; digoxin should be administered at the same time of day in relation to meals
Loop diuretics
These drugs inhibit electrolyte reabsorption in the thick ascending limb of the loop of Henle, thus promoting diuresis.
Furosemide (Lasix)
This is a commonly used loop diuretic with moderate diuretic potency. Increases excretion of water by interfering with chloride-binding co-transport system which in turn inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule.
Adult
20-80 mg/d PO/IV/IM divided q6-12h
Pediatric
1 mg/kg/dose PO/IV qd; may increase up to tid
Nephrotoxicity of cephalosporins is increased by furosemide; metformin decreases furosemide concentrations; furosemide interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide; hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when taken concurrently with this medication; increased plasma lithium levels and toxicity are possible when taken concurrently with this medication
Documented hypersensitivity; hypokalemia; renal failure
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Monitor serum potassium levels closely; may produce intravascular dehydration, severe hypokalemia, and significant hypochloremic metabolic alkalosis; may cause hyperuricemia; may produce deafness due to ototoxicity; dose should be titrated to effect; administer PO dose with food or milk to decrease stomach upset
Prostaglandins
Temporary maintenance of patency of ductus arteriosus in neonates with ductal-dependent congenital heart disease.
Alprostadil (Prostin VR)
Identical to the naturally occurring prostaglandin E1 (PGE1) and possesses various pharmacologic effects, including vasodilation and inhibition of platelet aggregation. Temporary maintenance of patency of ductus arteriosus in neonates with ductal-dependent congenital heart disease. Relaxes smooth muscle of the ductus arteriosus. Beneficial in infants with congenital defects that restrict pulmonary or systemic blood flow and who in order to get adequate oxygenation and lower body perfusion, depend on a patent ductus arteriosus.
Adult
Not indicated
Pediatric
Neonates and infants: 0.01-0.1 mcg/kg/min IV continuous infusion depending on the therapeutic response; with ductal patency, rate may be reduced to lowest effective dosage
Limited data exist; caution with concurrent use of antiplatelet drugs or anticoagulants
Documented hypersensitivity; hyaline membrane disease or respiratory distress syndrome; persistent fetal circulation
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Apnea occurs in 10-12% of neonates with congenital heart defects; use cautiously in neonates with bleeding tendencies (inhibits platelet aggregation); may cause systemic hypotension, flushing, bradycardia, rhythm disturbances, fever, or seizurelike activity; long-term infusions associated with cortical proliferation of long bones and gastric outlet obstruction
More on Transposition of the Great Arteries |
| Overview: Transposition of the Great Arteries |
| Differential Diagnoses & Workup: Transposition of the Great Arteries |
 Treatment & Medication: Transposition of the Great Arteries |
| Follow-up: Transposition of the Great Arteries |
| Multimedia: Transposition of the Great Arteries |
| References |
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References
Rao PS. Diagnosis and management of cyanotic congenital heart disease: part I. Indian J Pediatr. Jan 2009;76(1):57-70. [Medline].
Wypij D, Newburger JW, Rappaport LA, et al. The effect of duration of deep hypothermic circulatory arrest in infant heart surgery on late neurodevelopment: the Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg. Nov 2003;126(5):1397-403. [Medline].
[Guideline] Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. J Am Dent Assoc. Jun 2007;138(6):739-45, 747-60. [Medline]. [Full Text].
Aseervatham R, Pohlner P. A clinical comparison of arterial and atrial repairs for transposition of the great arteries: early and midterm survival and functional results. Aust N Z J Surg. Mar 1998;68(3):206-8. [Medline].
Horer J, Schreiber C, Dworak E, et al. Long-term results after the Rastelli repair for transposition of the great arteries. Ann Thorac Surg. Jun 2007;83(6):2169-75. [Medline].
Kampmann C, Kuroczynski W, Trubel H, et al. Late results after PTCA for coronary stenosis after the arterial switch procedure for transposition of the great arteries. Ann Thorac Surg. Nov 2005;80(5):1641-6. [Medline].
Kirjavainen M, Happonen JM, Louhimo I. Late results of Senning operation. J Thorac Cardiovasc Surg. Mar 1999;117(3):488-95. [Medline].
Neches WH, Park SC, Ettedgui, JA. Transposition of the great arteries. In: The Science and Practice of Pediatric Cardiology. Vol 1. 1998:1463-1503.
Paul MH, Wernovsky G. Transposition of the great arteries. In: Moss and Adams Heart Disease in Infants, Children, and Adolescents. Vol 2. 1995:1154-1224.
Pedra SR, Pedra CA, Abizaid AA, et al. Intracoronary ultrasound assessment late after the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol. Jun 21 2005;45(12):2061-8. [Medline].
Planche C, Lacour-Gayet F, Serraf A. Arterial switch. Pediatr Cardiol. Jul-Aug 1998;19(4):297-307. [Medline].
Puley G, Siu S, Connelly M, et al. Arrhythmia and survival in patients >18 years of age after the mustard procedure for complete transposition of the great arteries. Am J Cardiol. Apr 1 1999;83(7):1080-4. [Medline].
Soongswang J, Adatia I, Newman C, et al. Mortality in potential arterial switch candidates with transposition of the great arteries. J Am Coll Cardiol. Sep 1998;32(3):753-7. [Medline].
Takeuchi D, Nakanishi T, Tomimatsu H, Nakazawa M. Evaluation of Right Ventricular Performance Long After the Atrial Switch Operation for Transposition of the Great Arteries Using the Doppler Tei Index. Pediatr Cardiol. Aug 17 2005;[Medline].
Wren C, Birrell G, Hawthorne G. Cardiovascular malformations in infants of diabetic mothers. Heart. Oct 2003;89(10):1217-20. [Medline].
Further Reading
Keywords
transposition of the great arteries, TGA, complete transposition of the great arteries, d-TGA, simple transposition, ventriculoarterial discordance, heart lesion in neonate, cyanotic congenital heart lesion, intact ventricular septum, ventricular septal defect, left ventricular outflow tract obstruction, pulmonary vascular obstructive disease, atrial septal defect, patent ductus arteriosus, thrombocytopenia, congestive heart failure, cyanosis, tachypnea, tachycardia, diaphoresis, tetralogy of Fallot, hepatomegaly, dextro-transposition of the great arteries, levo-transposition of the great arteries, L-TGA, pulmonary vascular obstructive disease, treatment, diagnosis
