When children with Kawasaki disease grow up: Myocardial and vascular complications in adulthood

Abstract

Kawasaki disease (KD) is an acute, self-limited vasculitis that typically occurs in young children and was first described by Japanese pediatrician Tomisaku Kawasaki in 1967. Although originally thought to be a rare condition, KD has become the most common cause of acquired heart disease in the pediatric age group in developed countries. The majority of patients with KD appear to have a benign prognosis, but a subset of patients with coronary artery aneurysms are at risk for ischemic events and require lifelong treatment. In the 4 decades that have passed since the initial recognition of KD, the number of patients reaching adulthood has continued to grow. Adult cardiologists will be increasingly involved in the management of these patients. Currently, there are no established guidelines for the evaluation and treatment of adult patients who have had KD. We review here the current literature that may be helpful to clinicians who care for adults who experienced KD in childhood.

Figure 1. Sequential angiographic evaluation over a 10-21year-period of Japanese patients with KD who developed coronary artery aneurysms (Adapted from Kato et al., Ref. 12)

Figure 2. KD Histology

Autopsy findings in a 19 year-old man who developed severe cardiomyopathy 17 years after Kawasaki disease at age 2 years treated with intravenous immunoglobulin. Echocardiography during the acute and convalescent phase showed normal internal dimensions of the coronary arteries. A. Masson trichrome stain of a branch of the right coronary artery showing late organization of a thrombus. Original maginification x40. B. Masson trichrome stain of left ventricle showing endocardial fibrosis with an organizing subendocardial microinfarct. Original magnification x100. Photos courtesy of Dr. Henry F. Krous, Director of Pathology Research, Rady Children’s Hospital, San Diego, CA.

Figure 3. Calcification of coronary artery

Multislice computed tomography angiogram showing extensive calcification of a left anterior descending coronary artery aneurysm in a 12 year-old boy 7 years after acute KD.

Figure 4. Right coronary artery occlusion

Angiogram of occluded right coronary artery (RCA) aneurysm in a 33 year-old man who suffered a KD-compatible illness at age 6 years (diagnosed as non-specific viral illness) and was asymptomatic until his myocardial infarction. A. Injection of the occluded RCA B. RCA after percutaneous transluminal coronary angioplasty with re-established distal flow. Note smooth appearance of distal branches of the RCA C. Patent RCA aneurysm one month after PTCA, aspirin, and warfarin therapy maintaining an INR of 2-2.5.