Abnormal myocardial mechanics in Kawasaki disease: rapid response to gamma-globulin.
BACKGROUND
The time course and rate of recovery of myocardial dysfunction in association with Kawasaki disease in response to intravenous gamma-globulin is unknown and may provide mechanistic clues.


RESULTS
The acute changes in myocardial contractility in 25 patients with Kawasaki disease were evaluated by noninvasive stress-shortening and stress-velocity analysis. Echocardiograms were performed before and then daily for 4 days during which the patients received gamma-globulin 1.6 to 2 g/kg. Before treatment, contractility was abnormally low (<2 SD) in 14 patients (56%). Contractility increased significantly (2 SD increase) in 17 (68%), including 13 of 14 with depressed contractility and 4 whose initial contractility fell within normal limits. Of the 14 patients with depressed contractility, 8 (57%) normalized within 24 hours and a further 5 (35.7%) normalized within 6 months. A clinical response to treatment (fall in C-reactive protein by 50% and/or resolution of fever within 4 days) was seen in 22 patients (88%). Contractility increased in 17 of the 22 clinical responders and was normal before therapy in the other 5. The 3 patients who did not respond clinically also had no change in contractility with gamma-globulin therapy. Long-term (more than 12 months) follow-up was available in 19 patients. All patients had normal contractility at late follow-up.


CONCLUSIONS
More than half the patients with Kawasaki disease have abnormal contractility at presentation. Myocardial response to gamma-globulin therapy is associated with rapid improvement in myocardial mechanics, with a high concordance between the clinical and myocardial response to therapy. The speed of recovery suggests that depressed contractility in patients with Kawasaki disease is caused by a rapidly reversible process such as circulating toxins or activated cytokines. Long-term outcome is good even in those patients with slow recovery of myocardial function.
Moran
A M
AM
Department of Cardiology, Children's Hospital and the Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA. Colan@A1.TCH.harvard.edu
Newburger
J W
JW
Sanders
S P
SP
Parness
I A
IA
Spevak
P J
PJ
Burns
J C
JC
Colan
S D
SD
D002675:Child, Preschool; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000503:physiopathology*; D009200:Myocardial Contraction / Q000187:drug effects*; D011446:Prospective Studies; D016896:Treatment Outcome; D018487:Ventricular Dysfunction, Left / Q000188:drug therapy*
D016756:Immunoglobulins, Intravenous