Demonstration of Extensive Ischemia in a Patient With Kawasaki Disease
A 15-year-old boy without cardiovascular risk factors or autoimmune diseases was referred for a 4-year history of chest pain on exertion. His history was remarkable for a febrile illness at the age of 4 months, associated with heart failure and mildly depressed left ventricular function, from which he had made a full recovery.
Four years earlier, exercise gated single photon emission computed tomography was performed because of chest pain on exertion. This did not demonstrate any myocardial ischemia (Figure 1). The boy continued to have limited physical activity because of progressive worsening of chest pain. Dipyridamole first-pass myocardial perfusion stress cardiovascular magnetic resonance (CMR) (Siemens 1.5 T Espree, Erlangen, Germany) was performed and demonstrated a large myocardial perfusion defect in the left ventricular myocardium (Figure 2 and Movies I to III in the online-only Data Supplement) associated with chest pain. Inversion-recovery late contrast-enhanced CMR confirmed the absence of myocardial scar (Figure 3). The 12-lead ECG performed immediately after magnetic resonance showed a diffuse 3-mm ST-segment depression indicative of severe ischemia (Figure 4). The patient underwent conventional coronary angiography that showed an occluded left main coronary artery with proximal coronary aneurysms on the left anterior descending and the left circumflex arteries (Figure 5, black arrows). The distal left main coronary artery was supplied via collaterals arising from the right coronary artery (Figure 5, white arrows). The course of the collateral circulation was demonstrated in front of the trunk of the pulmonary artery on 64-slice computed tomography (Figure 6). The patient underwent coronary artery bypass grafting on the left anterior descending and left circumflex arteries with internal mammary arteries. His recovery was uneventful, and the patient was doing well without angina at the last follow-up visit 6 months after surgery. The occlusion of the proximal aneurysmal left coronary artery was responsible for severe and diffuse myocardial ischemia, which corresponded to a late cardiac manifestation of Kawasaki disease.
Cardiac sequelae of Kawasaki disease occur in 10% to 15% of patients, with coronary artery dilatation and aneurysms being the most common.1,2 Twenty percent of patients with coronary aneurysms will develop coronary artery stenosis and myocardial infarction.1,2 Although severe obstructive lesions are uncommon, myocardial ischemia may be observed in ≈2% to 4% of children during the follow-up of Kawasaki disease.1,2 In this setting, treadmill stress testing is known to have limited accuracy for detection of coronary artery obstruction.3 Myocardial scintigraphy has shown improved sensitivity for the detection of myocardial ischemia and tissue damage and has become the noninvasive method of choice for the assessment of ischemia and prognosis in these patients.3,4 However, obstructive coronary lesions secondary to giant aneurysms in Kawasaki disease are often proximal, and the presence of diffuse and balanced ischemia may lead to a false-negative scintigraphy. In our patient, dipyridamole first-pass perfusion CMR showed diffuse myocardial ischemia in the setting of total occlusion of the left main coronary artery. The time difference between single photon emission computed tomography and CMR studies precludes direct comparison between the 2 modalities. Because of the lack of radiation and its diagnostic accuracy, stress CMR may play an important role in the detection of obstructive coronary disease, tissue damage, and risk stratification in patients with Kawasaki disease.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/121/20/e409/DC1.