[Reversed Hegglin syndrome in Takayasu arteritis]

To study the "reversed Hegglin syndrome" in Takayasu arteritis, 57 patients were selected, and divided into four groups; Group I: 12 with Takayasu arteritis with aortic regurgitation (AR), Group II: 27 with Takayasu arteritis without AR, Group III: six with annuloaortic ectasia and severe AR, and Group IV: 12 with severe AR of other miscellaneous etiologies. QII-QT intervals were compared among the four groups. The reversed Hegglin syndrome (QII-QT greater than or equal to 40 msec) was observed in 58 percent of Group I patients and 37 percent of Group II patients. This phenomenon was not seen in any patients in Group III or IV. Sixteen of the 17 patients who had the reversed Hegglin syndrome belonged to the thoracic aortic type or diffuse type of Takayasu arteritis. Most patients who did not have this syndrome either had a localized lesion or a prolonged QT interval. The reversed Hegglin syndrome, therefore, is related to widespread lesions involving the thoracic aorta due to Takayasu arteritis, and may be attributed to a delay in closure of the aortic second heart sound due to a decrease in aortic impedance of the diseased proximal aorta.