Long term consequences of regressed coronary aneurysms after Kawasaki disease: vascular wall morphology and function

Objectives-To investigate the long term consequences of regressed aneurysms after Kawasaki disease, using follow up coronary angiography; to assess th e vascular wall morphology at the site of the aneurysms by intravascular ul trasound imaging; and to evaluate the function of the affected vessels usin g intracoronary infusions of acetylcholine and isosorbide dinitrate. Design-33 patients were studied, 27 with previous Kawasaki disease and six with congenital heart disease. All Kawasaki disease patients were followed for more than 10 years from disease onset. The 33 patients comprised four g roups: group 1 included 13 Kawasaki disease patients with a total of 23 sit es of regressed large sized (greater than or equal to 4 mm) coronary aneury sms; group 2 included 13 Kawasaki disease patients with 22 sites of regress ed small sized (< 4 mm) coronary aneurysms (four patients had sites of both large and small sized aneurysms); group 3 included a further five Kawasaki disease patients with 25 normal coronary angiography sites in the acute st age of Kawasaki disease; and group 4 comprised the six patients with congen ital heart disease as controls, with a total of 27 normal coronary angiogra phy sites. During coronary angiography, 15 mu g of acetylcholine and 0.5 mg isosorbide dinitrate were infused into the coronary artery. The luminal di ameter at the sites was measured using a cine- videodensitometric analyser, to assess the distensibility of the coronary artery wall. Results-Coronary angiography in all 22 patients in groups 1 and 2 and in al l the patients in group 3 was normal, with no stenoses and no irregularity of the arterial wall. However, the intravascular ultrasound imaging in grou ps 1 and 2 showed various degrees of the intimal thickening. In groups 1 an d 2, there was significantly more vascular constriction with acetylcholine, and poorer dilatation with isosorbide dinitrate than in groups 3 or 4 (eac h p < 0.05, respectively). There was no difference between group 3 and grou p 4 in response to either acetylcholine or isosorbide dinitrate, Conclusions-There is evidence of persisting abnormal vascular wall morpholo gy and vascular dysfunction at the site of regressed coronary aneurysms in patients with previous Kawasaki disease. These patients should be counselle d to avoid potential risk factors for atherosclerosis, and long term follow up is needed into adult life.