The 53rd Annual Meeting of Japanese Society of Pediatric Cardiology and Cardiac Surgery

Presentation information

JCK Poster

JCK Poster 2 (II-JCKP2)
Kawasaki Disease/General Cardiology/Adult Congenital Heart Disease

Sat. Jul 8, 2017 6:15 PM - 7:15 PM Poster Presentation Area (Exhibition and Event Hall)

Chair:Kaiyu Zhou(Department of Pediatric Cardiology, West China Second University Hospital, Sichuan University, Chengdu, Sichuan, China)

6:15 PM - 7:15 PM

[II-JCKP2-01] Progressive Coronary Dilatation for Eighteen Months in Kawasaki Disease: A Case Report

CHIAHUI WU, Ming-Tai Lin, Jou-Kou Wang, Mei-Hwan Wu (Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan)

Background
Kawasaki Disease (KD) is one of the most common vasculitis in childhood and may lead to cardiac sequelae. Progressive coronary dilatation is not uncommon for KD patients with giant coronary aneurysms, but aneurysms rarely keeps enlarging one year after fever onset.
Case report
We, herein, report a 6-year-old KD girl who suffered from KD at her 4.5 years. She had immunoglobulin at 9 days after fever onset and fever subsided soon. The echocardiogram before IVIG demonstrated LMCA 2.9mm (Z-score +1.85), LAD 3.06mm (Z-score +2.18 ), proximal RCA giant aneurysm, 8.3mm (Z-score +6.94). Follow-up echocardiography showed her RCA gradually dilated from the initial 8.3mm to 11.2mm (4Y7M) and gradually to 16.1mm (6Y). She had aspirin, low molecular weight heparin, propranolol, atorvastatin (4Y7M-4Y9M) and captopril. At her age of six, cardiac perfusion scan didn't show any perfusion defect.
Review of Literature
We reviewed the literature and found another three similar cases. One was from Japan and his right coronary aneurysm enlarged gradually from 7.8mm to 19.5mm over the next 17 years. The third cases came from United States. She had a progressively enlarged LAD aneurysm that measured 7mm at the time of initial KD diagnosis and 25mm at the time of surgery 11 years later.
Summary
Giant coronary aneurysms with progressive coronary dilatation lasting longer than 1 year are rare. Optimal managements are still unclear in the unique subgroup of KD patients. Multi-center registry may clarify their real outcomes and suggest the future directions.