Sudden Death Due to Rupture of Coronary Aneurysm in a 26-Year-Old Man
A 26-year-old man, previously in good health, was transferred to the emergency unit of our hospital on July 19, 1995; however, he was dead on arrival. He was an employee of a machine industry with no history of chest pain or palpitation, and at company checkups, no abnormalities were pointed out except for marked left hilar calcification on chest radiograph in June 1991 (Fig 1⇓); moreover, when he was 1 year old, he suffered from an acute self-limited febrile illness with diffuse erythematous macular rash followed by membranous desquamation of the fingertips and notable conjunctival infection resembling Stevens-Johnson syndrome or mucocutaneous lymph node syndrome that occurred 2 years after the first description of Kawasaki disease.1 About half an hour before admission, he had suddenly lost consciousness while playing catch during the noon break. Despite cardiopulmonary resuscitation, he died, and an autopsy was performed immediately.
A small, fresh hematoma was seen on the epicardial surface close to the proximal left anterior descending coronary artery (Fig 2A⇓). The true lumen was heavily calcified and stenotic, and the pseudolumen was coated internally with a smooth intimal membrane (Fig 2B⇓). A postmortem angiogram (Fig 3⇓) revealed that the saccular aneurysm communicated with the true lumen. The circumflex artery was also depicted, suggesting that there was no thrombotic occlusion of the left descending artery, and secondary strangulation of the true lumen due to the aneurysm rupture might have evoked an anteroseptal acute myocardial infarction (Fig 3⇓).
Histologically, diffuse intimal thickening was seen throughout the epicardial artery, and leukocyte infiltration into the adventitia and vasa vasorum was seen around the aneurysm, indicating persistent mild vasculitis of the artery (Fig 4⇓). Rupture of a coronary aneurysm is more common in the acute or subacute phase of Kawasaki disease,2 3 4 but to the best of our knowledge, our report is the first case in the literature of mucocutaneous lymph node disease of aneurysm rupture due to persistent vasculitis at the healed stage more than 10 years after disease onset. Further discussion is needed, including therapeutic options for non–aspirin-treated patients.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
- Copyright © 1998 by American Heart Association
Melish ME, Hicks RH, Larson EJ. Mucocutaneous lymph node syndrome in the United States. Am J Dis Child. 1976;130(6):599-607.
Fujiwara T, Fujiwara H, Nakano H. Pathological features of coronary arteries in children with Kawasaki disease in which coronary arterial aneurysm was absent at autopsy. Circulation. 1988;78:345-350.