A diabetic mother gives bih to a baby who dies in the first week of life. Autopsy reveals a severe cardiac malformation. Which of the following is the most likely diagnosis?
Correct Answer: Transposition of the great aeries
Description: Maternal diabetes is best known for causing large but immature-for-age babies. There is also a specific association between maternal diabetes and transposition of the great vessels. In transposition of the great vessels, the aoa takes off from the anterior pa of the right ventricle and the pulmonary trunk takes off from the posterior pa of the left ventricle. This produces a complete separation of the systemic and pulmonary circulations. Without surgical correction, most affected infants die within the first months of life, although a patent ductus aeriosus, patent foramen ovale, or ventricular septal defect may allow enough mixing of blood to temporarily sustain life. In atrial septal defect blood can pass from one atrium to the other. Associate coarctation of the aoa with Turner syndrome. Eisenmenger's syndrome is a shift from a left-to-right shunt to a right-to-left shunt secondary to developing pulmonary hypeension. Ref: McAnulty J.H., Broberg C.S., Metcalfe J. (2011). Chapter 97. Hea Disease and Pregnancy. In V. Fuster, R.A. Walsh, R.A. Harrington (Eds), Hurst's The Hea, 13e.
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Gynaecology & Obstetrics
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