Pediatric Heart Expert Forum
corrected transposition of great Arteries
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corrected transposition of great Arteries

my daughter is 2 years old has been diagonsed with corrected transposition of great Arteries High secundum ASD,L-R Ebsteins Anomaly of tricuspid valve. i would like to know want teatment to give and what  to expect.and if ASD can be closed in her case.
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Dear sheenaad:  Congenitally corrected transposition of the great arteries (also known as L-TGA, CC-TGA, or ventricular inversion) is an uncommon form of congenital heart disease.  The basic anatomic issue is that the “right ventricle” (which usually pumps blood flow to the low pressure lungs) and the “left ventricle” (which usually pumps blood to the high pressure body) are in the wrong position in this disorder.   Instead, the “left ventricle” is now pumping blood to the lungs and the “right ventricle” is now pumping blood to the high pressure of the body.  This mal-positioning of the pumping chambers can occur by itself, or more commonly, there are other congenital abnormalities of the heart as well.  This includes holes between the upper chambers (Atrial Septal Defect- ASD), holes between the lower chambers (ventricular septal defect- VSD), blockage of the pulmonary artery (pulmonary stenosis- PS) and Ebstein’s anomaly of the tricuspid valve.  In Ebstein’s anomaly, the malformed tricuspid valve is not in its normal position, and can have a variable amount of leakage (often moderate to severe over time).   Usually, the timing of diagnosis of congenitally corrected transposition of the great arteries depends upon the other associated heart anomalies.  The more severe cases can be diagnosed in the newborn period, or even before birth on fetal ultrasounds.  In addition to the structural heart abnormalities, there is also a much higher incidence of cardiac rhythm abnormalities in this group of patients.  They are more prone to develop “heart block” where there is incomplete or complete blockage of the electrical transmissions from the upper heart chambers to the lower heart chambers.  This requires treatment with a pacemaker.

Unfortunately, there is no single approach to managing patients with this form of complex congenital heart disease. Each case must be managed individually.  Some patients with mild associated abnormalities and good heart function, require no interventions.  Other patients with large holes or severely leaking valves require early interventions.  The major, long term concern is that a large number of patients with this disorder can develop heart failure as they get older.  It seems that the “right ventricle” may not be able to perform the high degree of work over many years that the “left ventricle” can do.  This congestive heart failure may need treatment with medications, pacemakers and even heart transplantation.  There are complex surgical procedures that can be performed during childhood where the blood is re-routed so that the “left ventricle” becomes the pumping chamber for the body.  These surgical procedures carry high risk, and the long term results are not available, so we are not yet sure if this is going to change the long term outcome for these patients.  A thorough discussion of medical and surgical options for your child’s particular case with your cardiologist is always recommended.  
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
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