Complete transposition of the great arteries with ventricular septal defect and coarctation or atresia of the aortic arch is a relatively rare anatomoclinical entity and in general not well understood. This association accounted for six out of 50 cases (12%) of our clinical experience and six out of 64 cases (9%) of the post-mortem series of complete transposition. Relevant clinical manifestations were cyanosis, early and pronounced congestive heart failure, with a decrease in amplitude of femoral pulses and, in the most severe cases, isolated left ventricular hypertrophy on the electrocardiogram. Inlet or outlet right ventricular obstruction and pulmonary artery dextroposition are the pathological substrates of unequal partitioning of the blood stream in favour of the pulmonary artery and ductus arteriosus, probably accounting for fetal underdevelopment of the aortic arch. The medical and surgical prognosis of these cases is definitely poor.
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