Transposition of the Great Arteries Workup
- Author: John R Charpie, MD, PhD; Chief Editor: Howard S Weber, MD, FSCAI more...
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.
Note the following:
- 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.
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.
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.
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.
Magnetic Resonance Imaging
Late gadolinium enhancement (LGE) cardiovascular magnetic resonance imaging (MRI) studies may have the potential to help clinicians stratify risk in patients with transposition of the great arteries following atrial switch procedure. In a single-center prospective study of 55 patients with transposition of the great arteries who underwent LGE MRI, investigators found that the presence of systemic right ventricular fibrosis was strongly associated with adverse clinical outcomes, particularly new-onset sustained tachyarrhythmias.
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