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Midaortic syndrome in a premature infant.

Hypertension is an unusual finding in premature infants and warrants an extensive workup. Well-known causes of hypertension include endocrine, renal and cardiac anomalies. Coarctation of the thoracic aorta, a well-recognized cardiac anomaly leading to hypertension can manifest at various ages including the newborn period. In contrast, midaortic syndrome (MAS), also known as midaortic dysplastic syndrome, is a rare clinical syndrome involving hypoplasia of the abdominal aorta (AA) 1 with varying degrees of narrowing of the visceral branches, often presenting as intractable hypertension. Though there are case reports describing this condition in older children and adults, the diagnosis of MAS can be extremely difficult in neonates, especially in preterm low-birth-weight infants. We describe a rare case of a premature infant who presented with congestive heart failure in utero and intractable hypertension postnatally. This is the youngest reported case of MAS with autopsy confirmation in the literature.