Giant coronary aneurysms are rare in Kawasaki disease and their outcomes after timely and appropriate treatment of KD are scarcely known in French population.

Over a 20years period, we identified 46 cases of giant coronary aneurysms in KD, defined as a diameter of at least one coronary artery≥8mm or with a z-score≥10. Mean age at diagnosis was 2.2±2.9years (min 0.1 – max 14.3) with 30% of patients being younger than 6months of age. Mean number of day fever between IVIG was 10±5days. Mean LMCA Z-score was 8.2±4.5, LAD 16.6±8.8, circumflex 6.7±5.2 and RCA 13.1±6.7. Sixty-three percent had bilateral lesions. All the patients have had at least one coronary angiogram or CT scan during follow-up. All patients received vitamin K antagonist and aspirin after discharge. Cardiac complications were defined as myocardial infarction, coronary stenosis, thrombosis or occlusion and aneurysm of the left ventricle.

In 33/46 patients, coronary artery imaging shows normal luminal dimensions (n=23) or reduction of coronary aneurysm (n=10), and 13 had persistent giant aneurysms after a mean follow-up of 1±0.2years. Mean follow-up was 7±5.6years. Thirteen cardiac events occurred in 9 patients: 3 early myocardial infarction, 5 coronary stenosis, 2 coronary thrombosis, 1 coronary occlusion and 2 left ventricle aneurysms. There were no reported deaths. One patient had a double coronary by-pass (LAD and RCA at 17.8years) preceded by a RCA stenting at 16.1years and one patient had a left ventricle aneurysm surgical repaired at 2.1years.

Giant coronary aneurysms in KD are impressive at diagnosis. Favorable outcome is frequent with normalization of luminal dimensions in half of the patients. Persisting giant aneurysm requiring long-term anticoagulation concerned one fourth of the patients.