BackgroundThoracic ascending aorta aneurysms (TAA) are an important cause of mortality in adults but are a relatively less studied subject compared to abdominal aortic aneurysms (AAA). The purpose of this review is to explain the main aspects (etiology, pathophysiology, diagnosis) of this disease and to summarize the most recent developments in its management.MethodologyLiterature was obtained through online health related search engines (PubMed, MEDLINE) by including the following keywords: ascending aorta aneurysm, thoracic aneurysms, Marfan syndrome, bicuspid aortic valve, familial thoracic syndrome, aortic dissection, aorta imaging and aortic aneurysm guidelines. We included articles dating from 1980 to 2014.FindingsLiterature revealed how lethal this disease can be and how simple steps such as follow-up and prophylactic surgery can significantly reduce morbidity and mortality. This review also allowed us to realize the many developments that have been made in recent years in the understanding of pathologic mechanisms of this disease.ConclusionTAA is a silent disease that needs to be recognized early in its course and followed closely in order to recommend appropriate preventive and prophylactic therapy in a timely manner.

We report 15 children who developed transient liver dysfunction related to hepatic ischaemia. All patients had cardiocirculatory failure 24 h before the onset of liver injury (day 1). Peak serum values of transaminases occurred between day 1 and day 3: SGOT (mean: 759 IU/l, range: 150-4400); SGPT (418 IU/l, 95-2547). Transaminase values decreased rapidly and normalised from day 6 to day 10. Minimum values of prothrombin test (PT) occurred on day 1 (31%, 10-70) and 13/15 patients had a PT less than 50% (27%, 10-44). PT values normalized from day 3 to day 10. Hypoglycaemia was present in 8/15 patients on day 1. Liver dysfunction improved after correction of the circulatory failure. These results confirm that transient hepatic dysfunction, probably as a consequence of hepatic hypoperfusion, may occur frequently in children after acute circulatory failure. We conclude that the diagnosis of ischaemic liver injury or shock liver syndrome in children can be made on clinical and biochemical criteria, and that liver biopsy is unnecessary.

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