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J Thorac Cardiovasc Surg 2008;135:1174-1175
© 2008 The American Association for Thoracic Surgery

Brief Communication

Aortic dissection in a young man with Loeys–Dietz syndrome

^a Department of Cardiology and Cardiothoracic Surgery, Institute of Clinical and Experimental Medicine–IKEM, Prague, Czech Republic
^b Department of Radiology, Nemocnice Na Homolce, Prague, Czech Republic
^c Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium

Received for publication October 13, 2007; accepted for publication January 7, 2008.

^_* Address for reprints: Vojtech Melenovsky, MD, PhD, Department of Cardiology, Institute for Clinical and Experimental Medicine–IKEM, Videnska 1958/9, Prague 4, 140 21, Czech Republic. (Email: vojtech.melenovsky@ikem.cz).

The first 20% of the full text of this article appears below.

Aortic dissections occur typically in older hypertensive patients with a peak incidence in the sixth decade, but occasionally they occur in younger patients, frequently with atypical presentation. Aortic dissection in this younger group is most often related to an underlying connective tissue disease, such as Marfan syndrome, vascular Ehlers-Danlos syndrome, nonsyndromic familial throacic aortic aneurysm with dissections, or the recently described Loeys-Dietz syndrome. We report the case of a young patient with Loeys-Dietz syndrome who underwent aortic dissection.

Clinical Summary

A 20-year-old apparently healthy man presented to the emergency department with chest discomfort that had begun the previous day, when he was hit in the chest during karate. He had deep retrosternal discomfort that was relatively mild, nonradiating, and nonpleuritic. The pain intensified during the ensuing hours and led to several episodes of lightheadedness that led him to seek medical attention. On physical examination, the patient had mild kyphosis but otherwise normal habitus (172 cm/60 kg); he was not diaphoretic. Heart sounds were normal and peripheral pulsations were symmetric, blood . . . [Full Text of this Article]