Congenital Thrombaortitis with Complete Obliteration of the Aortic Arch and the Great Vessels

Summary

Thrombosis of the great arteries rarely occurs in the neonate. We report a case of thrombosis of the aortic arch, the brachiocephalic truncus, the left carotid and subclavian arteries, seen in a one-day-old neonate. The clinical findings were at first misdiagnosed as interrupted aortic arch syndrome, though, in retrospect echocardiography was very suggestive of the correct diagnosis. On the basis of the histological examination we assume, as the most likely cause for thrombosis, an aortitis in the prenatal period. Following thrombectomy and Gore-Tex bypass from the ascending to the descending aorta, combined with ligature of a PDA, the child developed a low output state and died on the table. The postmortem examination revealed that the latter was due to multiple infarcts of the left ventricular myocardium.

Key words

Thrombaortitis - Aortic arch - Congenital thrombaortitis