Myocardial Strain and Strain Rate in Kawasaki Disease: Range, Recovery, and Relationship to Systemic Inflammation/Coronary Artery Dilation

Abstract

Background Kawasaki Disease (KD), a systemic vasculitis of medium sized vessels, is the most common cause of acquired heart disease among children in the developed world. Some KD patients demonstrate echocardiographic evidence of depressed myocardial mechanics. However, the incidence, etiology, and reversibility of abnormal mechanics in KD patients remain undefined. Methods and results We retrospectively studied 41 KD patients and measured myocardial strain and strain rate by velocity vector imaging from pre-treatment and convalescent echocardiograms. Pre-treatment procalcitonin, C-reactive protein (CRP), and coronary artery z-scores were obtained in all patients and compared between the groups with preserved versus depressed acute phase mechanics. The change in mechanics between the acute and convalescent phases was also assessed. Patients with initially low longitudinal strain improved by the convalescent period (mean difference - 4.0%; p<0.005) with the greatest improvement occurring in patients with the lowest initial strain (−7.3%; p<0.05). Patients with higher initial strain did not change significantly by the convalescent period. Patients with lower longitudinal and circumferential strain demonstrated higher median procalcitonin levels (1.2 vs. 0.3 ng/mL; p<0.05 and 1.8 vs. 0.4 ng/mL; p<0.05 respectively) and a trend towards higher CRP, but no difference in coronary artery z-scores. Strain rate was not associated with inflammatory markers or coronary artery z-scores. Conclusions The range of strain found in our cohort was large. Improvement in mean strain was driven primarily by patients with lower initial strain. Lower strain was associated with increased markers of systemic inflammation, but not proximal coronary artery changes.

IL-1 receptor antagonist, anakinra, prevents myocardial dysfunction in a mouse model of Kawasaki disease vasculitis and myocarditis

“…While only a minority of patients are clinically symptomatic [16], in many children with acute KD echocardiography studies show depressed shortening fraction, increased end-systolic and end-diastolic dimension strain abnormalities as well as diastolic dysfunction [33,42]. Increased left ventricular mass, believed to indicate cardiac edema, is also seen in patients with acute KD diagnosis prior to IVIG treatment [34,35].…”

“…These cases are often partially refractory to inotropic support and demonstrate impaired systolic and diastolic mechanical function with significantly decreased ejection fraction [16,33,36]. These cases are often partially refractory to inotropic support and demonstrate impaired systolic and diastolic mechanical function with significantly decreased ejection fraction [16,33,36].…”

“…Myocarditis is ubiquitous in KD patients; myocardial dysfunction and left ventricular enlargement are also often observed [33][34][35], and in some instances can lead to severe cardiac dysfunction and cardiogenic shock [36]. To determine whether IL-1Ra pretreatment also prevents myocardial dysfunction and left ventricular enlargement during LCWE-induced KD vasculitis, we performed MRI on LCWE-injected mice in the presence or absence of IL-1Ra pretreatment (Fig.…”

Kawasaki-betegség gyermekkortól a felnőttkorig. Multimodális képalkotók szerepe a hosszú távú nyomonkövetésben

“…19 éves betegről készült CT-koronarográfiás felvétel, akinél akut szakaszban megfigyelhetők az aneurizmák, és 14 évvel a betegség lezajlása után a már regrediált aneurizma helyén plakk rajzolódik ki. Mellette ugyanezen páciens CT-vizsgálat utáni koronarográfiás felvételén látható a plakk okozta szűkület idejű infekciójának gyakori előfordulása (16 (9,33). A betegek többségénél, mintegy 80%-ánál teljes gyógyulás várható és a halálozási adatok is javulnak: az 1974-ben mért 1%-os letalitáshoz képest ma már ez az adat kisebb, mint 0,1% (9,22).…”

“…A funkcioná-lis és terheléses vizsgálatok szintén a koronária-lézió típusától függőek. A speckle-tracking deformációs analízisről csak korai szakban végzett vizsgálatról történt beszámoló (8,26,(30)(31)(32)(33). A Kawasaki-betegség legjelentősebb szövődményei közé tartoznak a kardiovaszkuláris szövődmények, amelyek szívkoszorúér-betegséghez, vagy akár hirtelen halálhoz is vezethetnek.…”