Search

Kawasaki disease (KD) can be associated with high morbidity and mortality due to coronary artery aneurysms formation and myocardial dysfunction. Aim of this study was to evaluate the diagnostic performance of non-invasive myocardial work in predicting subtle myocardial abnormalities in Kawasaki disease (KD) children with coronary dilatation (CADL). A total of 100 patients (age 8.7 ± 5 years) were included: 45 children with KD and CADL (KD/CADL) (Z-score > 2.5), 45 age-matched controls (CTRL) and, finally, an additional group of 10 children with KD in absence of coronary dilatation (KD group). Left ventricular (LV) systolic function and global longitudinal strain (GLS) were assessed. Global myocardial work index (MWI) was calculated as the area of the LV pressure-strain loops. From MWI, global Constructive Work (MCW), Wasted Work (MWW) and Work Efficiency (MWE) were estimated. Despite normal LV systolic function by routine echocardiography, KD/CADL patients had lower MWI (1433.2 ± 375.8 mmHg% vs 1752.2 ± 265.7 mmHg%, p . MWI, MCW and MWE were significantly reduced in KD children despite normal LVEF and normal GLS. These abnormalities seems independent from CADL. Thus, in KD with normal LVEF and normal GLS, estimation of MWI may be a more sensitive indicator of myocardial dysfunction.

BackgroundSystemic right ventricular systolic dysfunction is common late after atrial switch surgery for transposition of the great arteries. Total isovolumic time is the time that the ventricle is neither ejecting nor filling and is calculated without relying on geometric assumptions. We assessed resting total isovolumic time in this population and its relationship to exercise capacity.MethodsA total of 40 adult patients with transposition of the great arteries after atrial switch – and 10 healthy controls – underwent transthoracic echocardiography and cardiopulmonary exercise testing from January, 2006 to January, 2009. Resting total isovolumic time was measured in seconds per minute: 60 minus total ejection time plus total filling time.ResultsThe mean age was 31.6 plus or minus 7.6 years, and 38.0% were men. There were 16 patients (40%) who had more than or equal to moderate systolic dysfunction of the right ventricle. Intra- and inter-observer agreement was good for total isovolumic time, which was significantly prolonged in patients compared with controls (12.0 plus or minus 3.9 seconds per minute versus 6.0 plus or minus 1.8 seconds per minute, p-value less than 0.001) and correlated significantly with peak oxygen consumption (r equals minus 0.63, p-value less than 0.001). The correlation strengthened (r equals minus 0.73, p-value less than 0.001) after excluding seven patients with exercise-induced cyanosis. No relationship was found between exercise capacity and right ventricular ejection fraction or long-axis amplitude.ConclusionResting isovolumic time is prolonged after atrial switch for patients with transposition of the great arteries. It is highly reproducible and relates well to exercise capacity.

Objective We compared the echocardiographic geometry of the preoperative and postoperative left ventricular outflow tract in children and adults with isolated fixed subaortic stenosis with age- and weight-matched controls to elucidate whether the geometry can be modified when surgical intervention is performed at a younger age. Methods The mitral–aortic valve distance, aortic valve diameter, aorto–left ventricular septal angle, degree of aortic valve dextroposition, aortic valve–subaortic stenosis distance, width of left ventricular outflow tract, left ventricle wall thickness, and septal thickness were determined preoperatively and postoperatively in 21 patients and 21 controls. The measurements were indexed to body surface area. Patients were divided into 3 age groups: group 1 comprised 9 patients aged 1 to 10 years, group 2 comprised 8 patients aged 11 to 20 years, and group 3 comprised 4 patients aged 21 years or more. Results Compared with controls, patients had a significantly wider mitral-aortic separation (group 1, P = .003; group 2, P = .02), a steeper aortoseptal angle (group 1, P = .02; group 3, P = .03), a smaller left ventricular outflow tract width (group 1, P = .003; group 2, P = .01), a marked aortic valve dextroposition (groups 1 and 3), an increased left ventricle wall thickness (group 1, P = .03), and an increased septal thickness (group 1, P = .01). There was a significant difference between preoperative and postoperative values in aortoseptal angle and left ventricular outflow tract width in patients up to 10 years of age ( P = .02 and P = .01, respectively). Conclusions Hearts with isolated subaortic stenosis have abnormal left ventricular outflow tract geometry that postoperatively showed changes in left ventricular outflow tract width and aortoseptal angle. Compared with controls, the aortoseptal angle does not "normalize" when surgery is performed in older patients, suggesting that left ventricular outflow tract geometry may be remodeled in younger patients.