Utility of Coronary Computed Tomography Angiography in the Diagnosis and Management of Acute-Phase Adult-Onset Kawasaki Disease

An 18-year-old woman presented with a 7-day history of fever, polymorphic exanthema, bilateral conjunctivitis, and cervical lymphadenopathy. Although Kawasaki disease (KD) in adulthood is rare, we suspected incomplete KD, and performed transthoracic echocardiography (TTE) on day 13 and coronary computed tomography angiography (CCTA) on day 14. We observed a coronary artery aneurysm (CAA) along the proximal left anterior descending artery (LAD; diameter, 8.0 mm, z-score, 7.1; Figure A,B; Movie S1) and ectatic changes of other coronary arteries, thereby confirming the diagnosis of KD. Given that the response to aspirin, i.v. immunoglobulin, methylprednisolone, and infliximab was not complete, we prescribed cyclosporine, which eventually reduced the inflammation. I.v. unfractionated heparin was also started, then switched to warfarin. On day 34, a thrombus in the left main coronary artery (LMCA) was suspected on TTE (Movie S2), but the patient was asymptomatic. CCTA on day 35 indicated no thrombus in the LMCA (Figure C,D), and hence urgent coronary angiography was not required. Moreover, CCTA showed no additional enlargement in the CAA compared with day 14, but stenoses in the mid-LAD and proximal right coronary artery (RCA) were detected. On day 77, however, CCTA showed partially regressed coronary arteries and progressed stenoses in the mid-LAD and proximal RCA (Figure E,F). Adult-onset KD is rare, but it needs to be considered in the differential diagnosis of fever of unknown origin, and CCTA is useful to diagnose and manage adult-onset KD in the acute phase.

Figure.

Coronary computed tomography angiography (A,C,E) volume-rendering view and (B,D,F) angiography view on day 14 (A,B), day 35 (C,D), day 77 (E,F) showing (A) coronary artery aneurysm (CAA) in the proximal left anterior descending artery (LAD; diameter, 8.0 mm; arrow) and ectatic changes in the coronary arteries; (B) CAA in the proximal LAD (arrow); (C) non-enlarged CAA in the proximal LAD and stenoses in the mid-LAD (arrow) and proximal right coronary artery (RCA; arrowhead); (D) stenosis in the mid-LAD (arrow); (E) non-enlarged CAA in the proximal LAD, partial regression in the other coronary arteries, unchanged stenosis in the mid-LAD (arrow), and slightly progressed stenosis in the proximal RCA (arrowhead); and (F) right anterior oblique-caudal view.

Acknowledgments

Japanese Red Cross Ashikaga Hospital, Cardiology Wards.

Disclosures

The authors declare no conflict of interest.

Supplementary Files

Supplementary File 1

Movie S1. Transthoracic echocardiography showing ectatic changes in the left main coronary artery and left anterior descending artery, short-axis view.

Supplementary File 2

Movie S2. Transthoracic echocardiography showing a high echogenic mass in the left main coronary artery, short-axis view.

Please find supplementary file(s);

http://dx.doi.org/10.1253/circj.CJ-18-0143