Coronary aneurysms in a case of Ehlers-Danlos syndrome

Abstract

An asymptomatic 48 year old man had transient T-wave inversion in the anterolateral leads after nonpenetrating chest trauma. Both the patient and his 6 year old son showed the typical skin hyperelasticity and joint hyperextensibility of the Ehlers-Danlos syndrome, suggesting autosomal dominant inheritance. The coexisting cardiac abnormalities of the patient included multiple coronary aneurysms, mitral valve prolapse with slight regurgitation, fusiform dilatation of the ascending aorta with moderate insufficiency and left anterior hemiblock. A routine noninvasive cardiovascular evaluation should be performed in all patients with Ehlers-Danlos syndrome in order to exclude valvular heart disease and dilatation of the aortic root or of other vessels, including the coronary arteries.