Authors: Kate Holmes, MSN, RN, CPNP-AC - Lucile Packard Children’s Hospital, Stanford Medicine, Children’s Health AND Kaitlin M. Flannery, MD, MPH - Stanford University
An 18-month-old with history of Kawaski Disease (KD) at 4-months old, resulting in a giant coronary artery aneurysm (CAA) of the right coronary artery (RCA), currently managed on aspirin and warfarin, presents with irritability and is diagnosed with CAA thrombosis and myocardial infarction (MI). Which of the following factors is MOST likely associated with MI following KD?
EXPLANATION
Kawasaki disease (KD) is an acute vasculitis that primarily affects children, targeting medium-sized muscular arteries. It is the leading cause of acquired heart disease in developed countries. In the United States, the incidence of KD is reported to be 18–25 cases per 100,000 children under five years old. In contrast, Northeast Asian countries, including Japan, South Korea, China, and Taiwan, experience an incidence that is 10 to 30 times higher.
KD is diagnosed clinically, when a patient presents with a fever lasting four days or more in conjunction with four out of five principal clinical features: distinct polymorphous rash, bilateral conjunctival injection, erythema and swelling of the hands and feet, and unilateral cervical lymphadenopathy, and oral changes, specifically “strawberry tongue”. The standard treatment consists of aspirin and IVIG and should be initiated as soon as possible and within ten days of fever onset to reduce risk of coronary artery aneurysm development. For patients exhibiting high-risk features, such as age < 6 months or coronary artery aneurysm z-score ≥ 2.5, additional anti-inflammatory therapies should be considered.1
Echocardiography plays a critical role in guiding treatment by enabling rapid visualization of proximal coronary artery abnormalities, particularly the proximal left anterior descending (LAD) and proximal right coronary arteries (RCA), which are the most common sites for aneurysm formation. Infants under six-months-old, as well as those with a Z-score ≥2.5 in the LAD or RCA at diagnosis, are at the highest risk for developing giant aneurysms and therefore should receive intensified treatement.1
Myocardial infarction (MI) is the leading cause of death in patients with KD. A retrospective single-center study published in 2025 examined patients with KD who experienced MI over a 30-year period. The findings revealed that almost half (48%) of all KD-associated MIs occurred within the first three months, and 81% occur within the first two years after acute illness. Notably, MIs only occurred in patients with giant coronary aneurysms, defined as a Z-score ≥10 or an absolute diameter of 8 mm or greater. In the current era of diagnosis, imaging, and treatment intensification, about 1% of KD patients develop giant coronary aneurysms. This study highlighted the high prevalence of missed MI diagnoses attributed to nonspecific symptoms. Only one-third of patients reported symptoms, while the remainder were diagnosed retrospectively on cardiac MRI. The two deaths that occurred in this patient cohort occurred in the 1980s.2
Research on anesthesia outcomes in patients with KD is limited. A retrospective single-center study published in 2005 analyzed anesthetic encounters over a 15-year period involving patients diagnosed with KD. The study highlighted the risks of decreased ventricular function, arrhythmia, and pericardial effusion during the acute phase of illness, all of which could lead to hemodynamic compromise under general anesthesia. Of the patients diagnosed with KD, approximately 25% underwent anesthesia, with 15% occurring during acute KD hospitalization but prior to diagnosis. These included cervical lymph node explorations, hip aspirations, and exploratory laparotomies. Notably, there were no reported perioperative deaths, although one patient developed congestive heart failure following a hip aspiration.3
The conclusion is that giant coronary artery aneurysm is critically linked to MI in KD patients, as MIs have only been observed in those with such aneurysms. Infants diagnosed with KD under six months of age are particularly at risk for developing these aneurysms, which most commonly occur in the RCA or proximal LAD.
REFERENCES
1. Jone PN, Tremoulet A, Choueiter N, et al. Update on Diagnosis and Management of Kawasaki Disease: A Scientific Statement From the American Heart Association. Circulation. 2024;150(23):e481-e500. doi:10.1161/CIR.0000000000001295
2. Ghelani SJ, Baker AL, Friedman K, et al. Myocardial Infarction in Kawasaki Disease. J Pediatr. 2025;284:114638. doi:10.1016/j.jpeds.2025.114638
3. Morrison JE, Anderson M, Chan KC, Pietra B, Zuk J, Gnadinger P. A 15-year review of children with Kawasaki's Syndrome having general anesthesia or deep sedation. Paediatr Anaesth. 2005;15(12):1053-1058. doi:10.1111/j.1460-9592.2005.01645.x