Background: Coronary artery aneurysms (CAA) develop in up to 30% of untreated patients with Kawasaki disease (KD). This may lead to ischemic heart disease and sudden cardiac death, which is extremely rare nowadays.
Method: A 3 mo infant presented with severe KD 27 days after onset of fever. He died few days later of multi vessel obstruction and aneurysms. OCT was performed post mortem and correlated with pathology findings.
Results: The patient presented with shock, inferolateral ischemia on ECG and high Troponin-i (from 6.45 to 59 μg/L 2 days later). Echocardiography showed severe myocardial dysfunction with diffuse coronary dilation and right CAA. Arterial Doppler demonstrated thrombosis of aneurysmal axillary, subclavian and iliac arteries, which corroborated with clinically ischemic limbs. Withdrawal of support was implemented due to multiorgan failure.
OCT images from the proximal RCA, LAD, carotid and pulmonary arteries were obtained post mortem. The circumflex coronary could not be accessed by the OCT catheter, which was occluded on pathology exam. The carotid (figure TOP) and pulmonary arteries were normal on OCT and histology. RCA and LAD (figure BOTTOM) showed aneurysmal dilatation, with marked intimal hyperplasia and preserved media on OCT. Pathology confirmed these findings, with destruction of internal elastic lamina, luminal myofibroblastic proliferation, neovascularization, and attenuation of the media in some sections.
Conclusion: This is the first report of pathologic correlation in a severe KD case with OCT imaging at the sub-acute stage, which adequately identified structural wall changes.