A rare congenital deformity of the heart in which the aorta arises from the right ventricle (instead of the left) and the pulmonary artery arises from both ventricles, in association with a large ventricular septal defect located above the septal limb of the crista supraventricularis and below the pulmonary valve. Cardiomegaly and right ventricular hypertrophy are usually present.
Permanent and progressive cyanosis usually begins at birth. Other features include underdevelopment, cardiomegaly, systolic murmur in left third intercostal space, loud second pulmonic sound. If survival continues for a few years, polycythaemia and clubbing of fingers and toes become evident. A diffuse precordial systolic murmur and thrill are commonly present. retardation of growth and failure to gain weight are other characteristics of these children.
The syndrome was described by Taussig and Bing in 1949, Pernkopf 1920. Very few cases reported. Prognosis is poor.
- H. B. Taussig, R. J. Bing:
Complete transposition of the aorta and levoposition of the pulmonary artery: clinical, physiological, and pathological findings.
American Heart Journal, St. Louis, 1949, 37: 551-559.