CT angiographic evaluation of genetic vascular disease: role in detection, staging, and management of complex vascular pathologic conditions.
OBJECTIVE
Arterial dissection and aneurysm rupture are significant sources of morbidity and mortality in patients with connective tissue diseases. This article provides a detailed analysis of cardiovascular involvement in Marfan syndrome, Loeys-Dietz syndrome, and vascular Ehlers-Danlos syndrome.


CONCLUSIONS
Although these syndromes share some overlapping features, they have discriminating clinical and imaging features, and knowledge of these features enables the radiologist to aid the referring clinician in making the correct diagnosis.
1 The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins Hospital, 601 N Caroline St, Baltimore, MD 21287.
Linda C
LC
Chu
Pamela T
PT
Johnson
Harry C
HC
Dietz
Elliot K
EK
Fishman
D000792:Angiography / Q000379:methods*; D004535:Ehlers-Danlos Syndrome / Q000000981:diagnostic imaging* / Q000235:genetics / Q000628:therapy; D006801:Humans; D055947:Loeys-Dietz Syndrome / Q000000981:diagnostic imaging* / Q000235:genetics / Q000628:therapy; D008382:Marfan Syndrome / Q000000981:diagnostic imaging* / Q000235:genetics / Q000628:therapy; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed