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A CASE OF SITUS INVERSUS AND DEXTROCARDIA WITH CONGENITALLY CORRECTED TRANSPOSITION OF GREAT ARTERIES IN AN ELDERLY MALE -A RARE ASSOCIATION

GOVINDA RAJAN B BRAMANAYAGAM

Abstract


Congenitally Corrected Transposition of Great Arteries is a rare cardiac malformation characterized by the combination of discordant atrioventricular or ventriculoarterial connections, usually accompanied by other cardiovascular malformations. Incidence has been reported to be around 133,000 live births, accounting for approximately 0.05 of  congenital heart malformations. Associated malformations may include inter ventricular communications, dextrocardia, obstruction of the outlet from the morphologically left ventricle and tricuspid valve abnormalities. The clinical picture and age of onset depends on the associated malformations. Bradycardia, single loud S1 and a heart murmur are the most common manifestations. In the rare cases where there are no associated malformations, congenitally corrected TGA can lead to progressive AV valve regurgitation and failure of the systemic ventricle. The diagnosis can also be made late in life when the patient presents with complete heart block or  cardiac failure. Etiology is currently unknown and with  increase in incidence among families with previous cases of CCTGA reported. Diagnosis can be made by fetal  echocardiography, but is far more commonly made postnatally with clinical signs and echocardiography. Prognosis is defined by the associated malformations and on the timing and approach to palliative surgical care. This case is presented for its rarity and the age at presentation, this patient being one of the eldest reported.

 


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References


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