Clinical InvestigationAcute Ischemic Heart DiseaseA survey of the 3-decade outcome for patients with giant aneurysms caused by Kawasaki disease
Section snippets
Background
National surveys of Kawasaki disease (KD) in Japan have been performed every 2 years since 1976. Recently, about 10,000 patients with KD have been reported annually, and 1 month after the onset, giant coronary aneurysms (GAs) were present in less than 0.4% of patients. Until 2010, 272,749 patients had had acute KD, with 436 deaths. Although the mortality of KD exceeded 0.2% in the 1970 to 1984, it had decreased to less than 0.1% by the 1990s.1 Most deaths occurred early after the acute illness
Definitions
In this study, GA implies a coronary aneurysm due to KD with a diameter of more than or equal to 8 mm. In coronary artery lesions, localized stenosis (LS) means stenosis equal to or more than 25%, and occlusion means complete occlusion or segmental stenosis (SS) with new small vessels indicating recanalization.5 Stenotic lesions include LS, occlusion, and SS. Death, AMI, coronary revascularization procedures such as CABG and percutaneous coronary catheter intervention (PCI), syncope, and
Number and background of patients with GA
A total of 261 patients with GA were reported by the 9 institutions; 16 were excluded because they failed to meet the definition of GA for the study. Data on 245 patients with GA were analyzed including 187 men (76%) and 58 women (24%). The outpatient department (OPD) records were available for all 245 patients. Age at the latest OPD visit ranged from 1 to 56 years (median 23 years), and the interval from the onset of KD to the latest OPD attendance ranged from 2 months to 51 years (median 20
Discussion
Acute MI is the most important determinant of the long-term outcome of patients with GA. Although most AMIs occurred within 2 years of the onset of KD, a decreased survival rate was found more than 20 years later.8 Acute MI after KD in most patients is triggered by sudden coronary occlusion due to thrombosis in the GA predisposed to by endothelial dysfunction, turbulence or stasis within the GA, and hypercoagulability caused by accelerated coagulant and thrombolytic systems, which complicate
Conclusions
The long-term outcome was significantly different between bilateral GA and unilateral GA, and these differences are important in making treatment decisions in both the early and late stages. The results focus attention on the need to preserve myocardial perfusion, especially in high-risk patients with bilateral GA through the judicious use of revascularization procedures. The optimal CABG approach would be useful in bilateral GA. The study appears to confirm the value of appropriately used
Acknowledgements
We thank Professor Peter for his kind English language consultation.
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