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Published on: September 8, 2023
A fatal case of ruptured giant coronary artery aneurysm
Yuki Imai1, Keishin Sunagawa, Mamoru Ayusawa
1Department of Pediatrics, Nihon University School of Medicine, 30-1 Oyaguchi-kamicho, Itabashi, Tokyo 173-8610, Japan. yimai@med.nihon-u.ac.jp
Insights
A rare, fatal rupture of a coronary artery aneurysm (CAA) in a child with Kawasaki disease (KD) highlights the need for early intervention. Distinguishing "super-giant" CAAs is crucial for timely, life-saving treatment.
Area of Science:
- Pediatric Cardiology
- Rheumatology
- Vascular Medicine
Background:
- Kawasaki disease (KD) is a leading cause of acquired heart disease in children, often associated with coronary artery aneurysms (CAAs).
- While typically managed with intravenous immunoglobulin and aspirin, some KD cases progress to severe CAA complications.
- CAA rupture is a rare but catastrophic event, necessitating a deeper understanding of risk factors and management strategies.
Observation:
- A 5-year-old boy with KD developed bilateral CAAs, including a rapidly enlarging 18-mm left anterior descending artery aneurysm.
- Despite standard treatment with IV gamma globulin, aspirin, and anticoagulation, the aneurysm continued to expand.
- The patient experienced abrupt cardiac arrest due to a ruptured LAD aneurysm and cardiac tamponade, leading to death.
Findings:
- Microscopic examination of the ruptured aneurysm revealed significant neutrophilic infiltration, indicating active inflammation.
- The case underscores the potential for rapid, aggressive CAA progression even with conventional KD treatment.
- The study proposes a new classification of "super-giant" CAAs ( >10 mm with ongoing vasculitis) to identify high-risk cases.
Implications:
- Early identification and differentiation of "super-giant" CAAs are critical for timely intervention and improved patient survival.
- Aggressive management strategies, potentially including surgical intervention or intensive care, should be considered for "super-giant" CAAs.
- This case highlights the importance of vigilant monitoring and advanced management protocols for complex Kawasaki disease cases.
Abstract:
A 5-year-old Japanese boy died because of a ruptured left coronary artery aneurysm (CAA). He was diagnosed as having Kawasaki disease (KD) on the 5th day from onset, with all of the principal signs. On the 7th day of illness, bilateral CAAs were already found via echocardiography, and he was treated with intravenous (IV) gamma globulin and oral ASA. However, the fever persisted and the CAA progressed rapidly. Echocardiography on the 12th illness day showed a giant (18-mm) left anterior descending (LAD) artery aneurysm. Oral propranolol and nifedipine were administered, in conjunction with warfarin/aspirin anti-coagulation therapy. On the 13th day of illness, cardiac arrest developed abruptly, and, despite cardiopulmonary resuscitation (CPR), the patient remained unresponsive and died one hour later. The final pathological diagnosis was a ruptured LAD artery aneurysm and cardiac tamponade. Microscopic investigation of the ruptured vascular wall revealed marked neutrophilic infiltration, with fewer macrophages and lymphocytes. CAA ruptures are a very rare, but fatal, complication of KD. Based on a review of previous reports on CAA ruptures, we consider it useful to distinguish aneurysms which rapidly dilate and continue to expand beyond a diameter of 10 mm with ongoing vasculitis (these CAAs can be termed "super-giant") from the more common giant CAAs limited to a diameter of 8 or 9 mm, because a decision must be made as to whether to start intensive care or to intervene surgically, in order to ensure the survival of patients with such a potentially critical complication.
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