Transposition of the Great Arteries: Background, Pathophysiology, Etiology 18/11/20 23.

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Transposition of the Great

Arteries
Updated: Apr 11, 2017
Author: John R Charpie, MD, PhD; Chief Editor: Howard S Weber, MD, FSCAI more...

OVERVIEW

Epidemiology
United States data

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.

Race-, sex-, and age-related demographics

No racial predilection is known, but transposition of the great arteries has a 60-70% male
predominance.

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

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