eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Transposition of the Great Arteries

John R Charpie, MD, PhD, Associate Professor, Department of Pediatrics, University of Michigan Medical Center
Kevin O Maher, MD, Assistant Professor of Pediatrics, Emory University School of Medicine; Consulting Staff, Department of Pediatrics, Pediatric Cardiovascular Intensive Care Unit, Sibley Heart Center

Updated: Jun 11, 2009

Introduction

Background

Transposition of the great arteries (TGA) is the most common cyanotic congenital heart lesion that presents in neonates. The hallmark of transposition of the great arteries is ventriculoarterial discordance, in which the aorta arises from the morphologic right ventricle and the pulmonary artery arises from the morphologic left ventricle.

This right ventricular angiogram shows a patient with transposition of the great arteries. The aorta arises directly from the right-sided anterior right ventricle (10° left anterior oblique [LAO]).

This right ventricular angiogram shows a patient with transposition of the great arteries. The aorta arises directly from the right-sided anterior right ventricle (70° left anterior oblique [LAO]).

This left ventricular angiogram shows a patient with transposition of the great arteries. The pulmonary artery arises directly from the left-sided posterior left ventricle (30° right anterior oblique [RAO]).

This left ventricular angiogram shows a patient with transposition of the great arteries. The pulmonary artery arises directly from the left-sided posterior left ventricle (20° cranial).

The major anatomic classifications of transposition of the great arteries depend on the relationship of the great arteries to each other and/or the infundibular morphology. In approximately 60% of the patients, the aorta is anterior and to the right of the pulmonary artery (dextro-transposition of the great arteries [d-TGA]). However in a subset of patients, the aorta may be anterior and to the left of the pulmonary artery (levo-transposition of the great arteries [l-TGA]). In addition, most patients with transposition of the great arteries (regardless of the spacial orientation of the great arteries) have a subaortic infundibulum, an absence of subpulmonary infundibulum, and fibrous continuity between the mitral valve and the pulmonary valve. Despite these useful classifications, several exceptions are noted, and, hence, discordant ventriculoarterial connection is the only distinguishing characteristic that defines transposition of the great arteries.

From a practical standpoint, the presence or absence of associated cardiac anomalies defines the clinical presentation and surgical management of a patient with transposition of the great arteries. The primary anatomic subtypes are (1) transposition of the great arteries with intact ventricular septum, (2) transposition of the great arteries with ventricular septal defect, (3) transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction, and (4) transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease.

In approximately one third of patients with transposition of the great arteries, the coronary artery anatomy is abnormal, with a left circumflex coronary arising from the right coronary artery (22%), a single right coronary artery (9.5%), a single left coronary artery (3%), or inverted origin of the coronary arteries (3%) representing the most common variants.

Pathophysiology

The pulmonary and systemic circulations function in parallel, rather than in series. Oxygenated pulmonary venous blood returns to the left atrium and left ventricle but is recirculated to the pulmonary vascular bed via the abnormal pulmonary arterial connection to the left ventricle. Deoxygenated systemic venous blood returns to the right atrium and right ventricle where it is subsequently pumped to the systemic circulation, effectively bypassing the lungs. This parallel circulatory arrangement results in a deficient oxygen supply to the tissues and an excessive right and left ventricular workload. It is incompatible with prolonged survival unless mixing of oxygenated and deoxygenated blood occurs at some anatomic level.

The following are 3 common anatomic sites for mixing of oxygenated and deoxygenated blood in transposition of the great arteries:

• Atrial septal defect
• Ventricular septal defect
  
  

  

  This 2-dimensional echocardiogram (parasternal long-axis view) shows a patient with transposition of the great arteries and ventricular septal defect. The pulmonary artery arises from the posterior (left) ventricular, dives posteriorly, and bifurcates immediately into left and right branch pulmonary arteries. A large ventricular septal defect is present in the outlet septum.

  
  
  
  

  

  This 2-dimensional echocardiogram (apical 4-chamber view) shows a patient with transposition of the great arteries and ventricular septal defect. The anterior aorta arises from the right-sided right ventricle.

  
  
• Patent ductus arteriosus

One or all of these lesions can be present in concert with dextro-transposition of the great arteries, and the degree of arterial hypoxemia depends on the degree of anatomic mixing.

Frequency

United States

Despite its overall low prevalence, transposition of the great arteries is the most common etiology for cyanotic congenital heart disease in the newborn.¹ This lesion presents in 5-7% of all patients with congenital heart disease. The overall annual incidence is 20-30 per 100,000 live births, and inheritance is multifactorial. Transposition of the great arteries is isolated in 90% of patients and is rarely associated with syndromes or extracardiac malformations. This congenital heart defect is more common in infants of diabetic mothers.

Mortality/Morbidity

The mortality rate in untreated patients is approximately 30% in the first week, 50% in the first month, and 90% by the end of the first year. With improved diagnostic, medical, and surgical techniques, the overall short-term and midterm survival rate exceeds 90%.

Long-term complications are secondary to prolonged cyanosis and include polycythemia and hyperviscosity syndrome. These patients may develop headache, decreased exercise tolerance, and stroke. Thrombocytopenia is common in patients with cyanotic congenital heart disease leading to bleeding complications.

Patients with a large ventricular septal defect, a patent ductus arteriosus, or both may have an early predilection for congestive heart failure, as pulmonary vascular resistance falls with increasing age. Heart failure may be mitigated in those patients with left ventricular outflow tract (pulmonary) stenosis.

A small percentage (approximately 5%) of patients with transposition of the great arteries (and often a ventricular septal defect) develop accelerated pulmonary vascular obstructive disease and progressive cyanosis despite surgical repair or palliation. Long-term survival in this subgroup is particularly poor.

Race

No racial predilection is known.

Sex

TGA has a 60-70% male predominance.

Age

Patients with TGA usually present with cyanosis in the newborn period, but clinical manifestations and courses are influenced predominantly by the degree of intercirculatory mixing.

Clinical

History

• Infants with transposition of the great arteries (TGA) are usually born at term, with cyanosis apparent within hours of birth.
• The clinical course and manifestations depend on the extent of intercirculatory mixing and the presence of associated anatomic lesions.
  • Transposition of the great arteries with intact ventricular septum: Prominent and progressive cyanosis within the first 24 hours of life is the usual finding in infants.
  • Transposition of the great arteries with large ventricular septal defect
    • Infants may not initially manifest symptoms of heart disease, although mild cyanosis (particularly when crying) is often noted.
    • Signs of congestive heart failure (tachypnea, tachycardia, diaphoresis, and failure to gain weight) may become evident over the first 3-6 weeks as pulmonary blood flow increases.
  • Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction
    • Infants often present with extreme cyanosis at birth, proportional to the degree of left ventricular (pulmonary) outflow tract obstruction.
    • The clinical history may be similar to that of an infant with tetralogy of Fallot.
  • Transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease
    • Progressively advancing pulmonary vascular obstructive disease can prevent this rare subgroup of patients from developing symptoms of congestive heart failure, despite a large ventricular septal defect.
    • Most often, patients present with progressive cyanosis, despite an early successful palliative procedure.

Physical

Newborns with transposition of the great arteries are usually well developed, without dysmorphic features. Physical findings at presentation depend on the presence of associated lesions.

• transposition of the great arteries with intact ventricular septum
  • Infants typically present with progressive central (perioral and periorbital) cyanosis.
  • Other than cyanosis, the physical examination is often unremarkable.
• Transposition of the great arteries with large ventricular septal defect
  • Cyanosis may be mild initially, although it is usually more apparent with stress or crying.
  • Upon presentation, infants often have an increased right ventricular impulse, a prominent grade 3-4/6 holosystolic murmur, third heart sound, mid-diastolic rumble, and a gallop rhythm.
  • Hepatomegaly may be present.
• Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction
  • Cyanosis is prominent at birth, and the findings are similar to those of infants with tetralogy of Fallot.
  • A single, or narrowly split, diminished second heart sound and a grade 2-3/6 systolic ejection murmur may be present.
  • Hepatomegaly is rare.
• Transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease
  • Progressive pulmonary vascular obstructive disease is not always evident from physical examination findings.
  • Cyanosis is usually present and can progress despite palliative therapy in the newborn period.
  • No murmur (despite the ventricular septal defect) or early short systolic ejection sounds are heard.
  • The second heart sound is often single, with increased intensity.
  • In later childhood or adolescence, a high-pitched, blowing, early decrescendo diastolic murmur of pulmonary insufficiency and a blowing apical murmur of mitral insufficiency are evident.

Causes

• Etiology for transposition of the great arteries is unknown and is presumed to be multifactorial.
• Embryology likely involves abnormal persistence of the subaortic conus with resorption or underdevelopment of the subpulmonary conus (infundibulum). This abnormality aligns the aorta anterior and superior with the right ventricle during development.

Differential Diagnoses

Pulmonary Atresia With Intact Ventricular Septum
Tetralogy of Fallot With Absent Pulmonary Valve
Tetralogy of Fallot With Pulmonary Atresia
Total Anomalous Pulmonary Venous Connection
Tricuspid Atresia
Truncus Arteriosus

Other Problems to Be Considered

Double-outlet right ventricle with malposed great arteries

Workup

Laboratory Studies

A hyperoxia test (for cyanotic congenital heart disease) may be indicated in patients with transposition of the great arteries (TGA).

• In a patient with arterial hypoxemia, an ABG measurement is obtained on 100% oxygen for 10 minutes.
• Pulmonary disease (not cyanotic congenital heart disease) is suspected if the partial pressure of oxygen increases to more than 150 mm Hg with oxygen.

Imaging Studies

• Chest radiography
  • The chest radiograph may appear normal in newborns with transposition of the great arteries and intact ventricular septum but may demonstrate the classic "egg on a string" appearance in approximately one third of patients.
  • With a ventricular septal defect, cardiomegaly usually occurs with increased pulmonary arterial vascular markings.
• Echocardiography
  • Echocardiographic images should be diagnostic of transposition of the great arteries by demonstrating the bifurcating pulmonary artery arising posteriorly from the left ventricle in the parasternal long-axis view.
  • The parasternal short-axis view shows the relationship of the great arteries to one another. The aorta is usually anterior and rightward of the pulmonary artery in cross-section.
  • Most associated anatomic lesions, including atrial septal defects, ventricular septal defects, and patent ductus arteriosus, are also diagnosed readily by echocardiography.
  • The coronary artery anatomy needs to be ascertained and may be abnormal in nearly one third of patients. The coronary artery origins and branching pattern are often delineated by echocardiography.

Procedures

• Cardiac catheterization
  • Diagnostic cardiac catheterization is usually reserved for the subgroup of patients for whom echocardiography does not adequately delineate the anatomy. Suspected coronary artery abnormalities and additional ventricular septal defects may be confirmed or better delineated by cardiac catheterization with angiography. In addition, cardiac catheterization may be necessary to improve left-to-right shunting.
  • Postcatheterization precautions include hemorrhage, vascular disruption after balloon dilation, pain, nausea and vomiting, and arterial or venous obstruction from thrombosis or spasm.
  • Complications may include rupture of blood vessel, tachyarrhythmias, bradyarrhythmias, and vascular occlusion.

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.
• 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.

Dosing

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

Interactions

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

Contraindications

Documented hypersensitivity, atrioventricular block, idiopathic hypertrophic subaortic stenosis, constrictive pericarditis, hypokalemia, renal failure

Precautions

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.

Dosing

Adult

20-80 mg/d PO/IV/IM divided q6-12h

Pediatric

1 mg/kg/dose PO/IV qd; may increase up to tid

Interactions

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

Contraindications

Documented hypersensitivity; hypokalemia; renal failure

Precautions

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.

Dosing

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

Interactions

Limited data exist; caution with concurrent use of antiplatelet drugs or anticoagulants

Contraindications

Documented hypersensitivity; hyaline membrane disease or respiratory distress syndrome; persistent fetal circulation

Precautions

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

Follow-up

Further Inpatient Care

• Admit patients with transposition of the great arteries (TGA) for preoperative testing and surgical interventions.
• Carefully monitor medication doses and side effects.
• Monitor adequacy of repair and palliation with periodic physical examinations and possibly echocardiograms.
• Periodic electrocardiograms and/or 24-hour Holter monitoring to monitor for atrial arrhythmias should be employed, particularly following atrial-level switch operation (ie, Senning or Mustard procedure).
• Controversy surrounds whether patients should undergo cardiac catheterization every few years following arterial switch operation because of the concern for long-term patency and normal function of the coronary arteries following surgical translocation. No specific recommendations for routine cardiac catheterization will be possible until more information is available, and treatment of coronary artery stenosis is still a matter of debate.

Further Outpatient Care

• 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.²

Inpatient & Outpatient Medications

• Many patients do not require any specific medications. Possible discharge medications might include digoxin, furosemide, or both.
• All patients require preoperative and postoperative antibiotic prophylaxis for dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa. Antibiotic prophylaxis is also recommended for invasive respiratory tract procedures that involve incision or biopsy of the respiratory mucosa (eg, tonsillectomy, adenoidectomy). Antibiotic prophylaxis is not recommended for bronchoscopy unless the procedure involves incision of the respiratory tract mucosa. For invasive respiratory tract procedures to treat an established infection (eg, drainage of abscess, empyema), administer an antibiotic active against Streptococcus viridans. See the endocarditis prophylaxis guidelines that were revised by the American Heart Association (AHA) in the Medication section.³

Transfer

• Transfer may be required for specialized diagnostic, therapeutic, and surgical interventions.

Complications

• Congestive heart failure
• Arrhythmia
• Eisenmenger syndrome (irreversible and progressive pulmonary vascular obstructive disease)

Prognosis

• Prognosis depends on the specific anatomic substrate and type of surgical therapy used (arterial switch operation, atrial switch operation, or Rastelli procedure).
• The overall survival rate following arterial switch operation is 90%.
• The overall mortality rate following an atrial level switch is low; however, long-term morbidity associated with systemic (right) ventricular dilatation and failure, systemic atrioventricular (tricuspid) valve regurgitation, and atrial bradyarrhythmias and tachyarrhythmias is significant.

Patient Education

• Family members should learn cardiopulmonary resuscitation (CPR).
• Educate family members about congenital heart disease.
• Obtain genetics counseling for future pregnancy, despite the relatively low risk of recurrence.
• For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Tetralogy of Fallot.

Miscellaneous

Medicolegal Pitfalls

• Failure to consider transposition of the great arteries (TGA), particularly in a cyanotic newborn
• Failure to adequately delineate coronary artery anatomy preoperatively
• Failure to appropriately interpret diagnostic information, including echocardiography, radiography, and oxygenation studies
• Complications of surgery, including death

Multimedia

Media file 1: This 2-dimensional echocardiogram (parasternal long-axis view) shows a patient with transposition of the great arteries and ventricular septal defect. The pulmonary artery arises from the posterior (left) ventricular, dives posteriorly, and bifurcates immediately into left and right branch pulmonary arteries. A large ventricular septal defect is present in the outlet septum.

Media file 2: This 2-dimensional echocardiogram (apical 4-chamber view) shows a patient with transposition of the great arteries and ventricular septal defect. The anterior aorta arises from the right-sided right ventricle.

Media file 3: This right ventricular angiogram shows a patient with transposition of the great arteries. The aorta arises directly from the right-sided anterior right ventricle (10° left anterior oblique [LAO]).

Media file 4: This right ventricular angiogram shows a patient with transposition of the great arteries. The aorta arises directly from the right-sided anterior right ventricle (70° left anterior oblique [LAO]).

Media file 5: This left ventricular angiogram shows a patient with transposition of the great arteries. The pulmonary artery arises directly from the left-sided posterior left ventricle (30° right anterior oblique [RAO]).

Media file 6: This left ventricular angiogram shows a patient with transposition of the great arteries. The pulmonary artery arises directly from the left-sided posterior left ventricle (20° cranial).

References

1. Rao PS. Diagnosis and management of cyanotic congenital heart disease: part I. Indian J Pediatr. Jan 2009;76(1):57-70. [Medline].

2. 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].

3. [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].

4. 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].

5. 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].

6. 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].

7. Kirjavainen M, Happonen JM, Louhimo I. Late results of Senning operation. J Thorac Cardiovasc Surg. Mar 1999;117(3):488-95. [Medline].

8. Neches WH, Park SC, Ettedgui, JA. Transposition of the great arteries. In: The Science and Practice of Pediatric Cardiology. Vol 1. 1998:1463-1503.

9. 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.

10. 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].

11. Planche C, Lacour-Gayet F, Serraf A. Arterial switch. Pediatr Cardiol. Jul-Aug 1998;19(4):297-307. [Medline].

12. 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].

13. 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].

14. 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].

15. Wren C, Birrell G, Hawthorne G. Cardiovascular malformations in infants of diabetic mothers. Heart. Oct 2003;89(10):1217-20. [Medline].

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

Contributor Information and Disclosures

Author

John R Charpie, MD, PhD, Associate Professor, Department of Pediatrics, University of Michigan Medical Center
John R Charpie, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Coauthor(s)

Kevin O Maher, MD, Assistant Professor of Pediatrics, Emory University School of Medicine; Consulting Staff, Department of Pediatrics, Pediatric Cardiovascular Intensive Care Unit, Sibley Heart Center
Kevin O Maher, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Charles I Berul, MD, Associate Professor of Pediatrics, Harvard Medical School; Senior Associate, Department of Cardiology, Children's Hospital of Boston
Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Ameeta Martin, MD, Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine
Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

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