Delayed Development of Coronary Artery Aneurysm in Patients with Kawasaki Disease Who Were Clinically Responsive to Immunoglobulin

A rare case of a giant circumflex coronary artery aneurysm 10 years after bentall surgery

“…To our knowledge, this is the first case reported in the medical literature describing a giant aneurysm of LCX involving the vessel's length distant from its origin that occurred in a patient who had undergone a Bentall procedure 10 years before, had a previously normal coronary angiogram and did not have a history of Kawasaki disease [21] , inflammatory disease or connective tissue disorder, such as Marfan or Ehlers-Danlos syndromes [22 , 23] . Considering the location and morphology of the aneurysmal dilation diagnosed in our patient ( Fig.…”

Late Coronary Aneurysm Formation after Kawasaki Disease: a Review of Mechanistic and Clinical Aspects

“… 36) Interestingly, 4 out of 13 patients having CAAs at 4 weeks had a pretreatment Z max value of <2.0. 36) Unfortunately, this might imply that KD patients, who have apparently unaffected coronary arteries at the time of diagnosis, might occasionally develop ‘early CAAs’ even after succesful IVIG therapy. 36) It seems noteworthy that some authors raised the possibility of long-term use of certain agents including anakinra in an effort to prevent fibroproliferative complications including luminal stenosis.…”

“… 33) 34) 35) Accordingly, only 13 out of 325 (4%) IVIG responders with KD were reported to develop ‘early CAAs’ at 4 weeks in a recent retrospective analysis. 36) Importantly, a pretreatment Zmax value (the highest Z score in coronary branches) of >2.5 was suggested as the sole clinical variable to predict the evolution of ‘early CAAs’ with a specificity of 91% and a sensitivity of 50%. 36) This might potentially warrant more aggressive antiinflammatory regimens in KD patients who already have a pattern of significant coronary dilatation at diagnosis.…”

“… 36) Importantly, a pretreatment Zmax value (the highest Z score in coronary branches) of >2.5 was suggested as the sole clinical variable to predict the evolution of ‘early CAAs’ with a specificity of 91% and a sensitivity of 50%. 36) This might potentially warrant more aggressive antiinflammatory regimens in KD patients who already have a pattern of significant coronary dilatation at diagnosis. 36) Interestingly, 4 out of 13 patients having CAAs at 4 weeks had a pretreatment Z max value of <2.0.…”

“… 36) This might potentially warrant more aggressive antiinflammatory regimens in KD patients who already have a pattern of significant coronary dilatation at diagnosis. 36) Interestingly, 4 out of 13 patients having CAAs at 4 weeks had a pretreatment Z max value of <2.0. 36) Unfortunately, this might imply that KD patients, who have apparently unaffected coronary arteries at the time of diagnosis, might occasionally develop ‘early CAAs’ even after succesful IVIG therapy.…”