Nagib Dahdah

Acute coronary syndrome (ACS) remains underrecognized in patients with Kawasaki disease (KD). The population at risk for KD-associated ACS is increasing, yet current ACS guidelines are largely derived from atherosclerotic disease and do not adequately address the distinctive mechanisms, anatomy, and thrombosis risk associated with KD. Because the pathophysiology and management of KD-associated ACS differ in fundamental ways from those of atherosclerotic coronary artery disease, its management requires a coordinated team response, with collaboration between pediatric and adult cardiologists. Recent advances in coronary imaging, interventional techniques, and antithrombotic therapies provide an opportunity to establish a more disease-specific approach. This review synthesizes current evidence on the epidemiology and pathophysiology of coronary artery aneurysms, interventional coronary imaging, acute management, revascularization strategies, antithrombotic therapy, and post-ACS surveillance in patients with KD to guide clinical decision-making and treatment for patients with KD presenting with ACS.

Background: Kawasaki disease is an acute childhood vasculitis that may cause coronary artery aneurysms, raising concern for long-term myocardial dysfunction. Exercise stress echocardiography provides a noninvasive assessment of systolic and diastolic reserve, yet data comparing exercise responses in Kawasaki disease patients with contemporary coronary risk stratification are limited. Materials and methods: We retrospectively analysed exercise stress echocardiograms in patients with a history of Kawasaki disease-related coronary artery aneurysm (maximum coronary artery z -score ≥2.5) and age-matched controls without cardiac disease. Two-dimensional echocardiography was performed at rest and during early recovery following a standardised ergometer exercise test. Left ventricular and left atrial strain and mitral inflow velocities were measured. Within-group pre- and post-exercise changes were assessed using paired t -tests. Linear mixed-effects models evaluated differences in the magnitude of exercise-induced change between groups. Results: There were 34 and 27 patients in the Kawasaki disease and control cohorts, respectively. The Kawasaki disease cohort had a mean maximal coronary z -score of 3.8 ± 2.1. Both groups demonstrated significant pre- to post-exercise changes in left ventricular longitudinal strain and mitral A velocity. Additional systolic and diastolic parameter changes were observed within the Kawasaki disease cohort. Mixed-effects modelling revealed no significant differences in the magnitude of exercise-induced change between Kawasaki disease patients and controls. Conclusions: In paediatric patients with a remote history of Kawasaki disease and mild to moderate coronary artery aneurysms, exercise stress echocardiography demonstrated no differences in the magnitude of exercise-induced changes in systolic and diastolic indices compared to controls.

Background: Bacillus Calmette–Guérin (BCG) scar reactivation (BCGitis) is a distinctive clinical sign reported in Kawasaki disease (KD), yet evidence remains scarce across the literature. We aimed to summarize the epidemiology, chronology, clinical correlates, and coronary outcomes associated with BCGitis in KD. Methods: We conducted a PRISMA‐ScR–guided scoping review of studies describing BCGitis in KD. Data were synthesized descriptively across retrospective cohorts and case reports/series. Results: Forty studies published between 1997 and 2024 were included (10 retrospective cohorts; 30 reports of 36 cases). BCGitis was most frequently described in infants and young children (< 2 years, often < 6–12 months) and was commonly associated with earlier recognition and treatment. Reported BCGitis prevalence varied across cohorts, from 11.8% to 49.9%. Coronary artery lesions (CAL) and IVIG resistance were reported at lower rates in some cohorts; however, these findings were not statistically significant. In the case reports, most patients were infants, male predominance was observed, and incomplete KD was frequent. BCGitis was often reported during the early febrile phase, sometimes preceding complete KD diagnostic criteria. Most reported cases responded to IVIG. Coronary abnormalities were usually mild or transient, although two giant aneurysms, one case of Kawasaki disease shock syndrome, and one mortality were reported. Conclusions: BCGitis may support earlier recognition of KD, particularly in incomplete presentations, potentially facilitating timely recognition and treatment. Incorporation of BCGitis into diagnostic algorithms may improve patient outcomes. Future studies should standardize reporting of BCGitis chronology, coronary outcomes, and evaluate the incremental diagnostic value of BCGitis in prospective cohorts.

Kawasaki disease (KD) is a systemic inflammatory condition, likely triggered by a respiratory infection, inducing myocardial inflammation, and complicated with coronary aneurysms. The objectives are to measure the late cardiopulmonary exercise response and associate the severity of the acute episode with the long-term aerobic capacity. Data on the acute KD episode were collected retrospectively. Children and adolescents with past KD underwent standardized ergometer cardiopulmonary exercise testing. Regression analysis was used to associate coronary Z-scores and acute biomarkers, including maximum N-terminal pro-B-type natriuretic peptide (NT-proBNP), with the exercise parameters. Of 43 patients diagnosed with KD (mean age 4.97 ± 2.88 years, 42% females), 30 were age-and-sex matched after a mean of 6.78 ± 3.00 years (range 1.5–16 years) with 15 controls. Peak oxygen consumption (peakVO₂) and ventilatory threshold were significantly decreased in KD patients (34.1 ± 11.1 versus 39.4 ± 7.2 mL·kg⁻¹·min⁻¹, p = 0.025, and 50.7 ± 13.7 versus 56.3 ± 11.8%, p = 0.036, respectively). Acute NT-proBNP and acute coronary Z-score were not associated with peakVO₂ (odds ratio OR [95% confidence interval] 0.974 [0.734–1.293] and 1.023 [0.719–1.455], respectively). Instead, peakVO₂ in KD patients with a persistent coronary aneurysm, defined by a coronary segment lumen greater than 1.5-fold that of the adjacent segment, was lower than controls (26.2 ± 6.4 versus 39.4 ± 7.2 mL·kg⁻¹·min⁻¹, p = 0.013). Conclusion: Children with KD had reduced aerobic capacity 7 years after the diagnosis. Maximum acute NT-proBNP and acute coronary Z-score were not associated with the late impaired exercise capacity, but persistent coronary aneurysms were. What is Known: • Acute coronary Z-score and blood NT-proBNP are markers of disease severity in Kawasaki disease. • Kawasaki disease is associated with late coronary ischaemic complications. What is New: • In Kawasaki disease patients, several years after the diagnosis, peak exercise mass-related oxygen consumption and ventilatory threshold are decreased, and body mass index is increased. • Persistent coronary aneurysms, but neither acute coronary Z-score nor acute maximum NT-proBNP, are associated with the late impaired exercise capacity.

BACKGROUND: Cardiovascular dimensions measured during pediatric echocardiograms must be normalized for body size. However, other variables may confound their interpretation, such as age and abnormal body habitus. This retrospective cross-sectional study of the Canadian Congenital and Pediatric Cardiology Research Network aimed to create Z score equations for commonly measured dimensions in pediatric 2-dimensional echocardiography that were free of residual confounding effects of body size, body mass index, and age. METHODS: The reference sample consisted of >20 000 children without heart disease from 9 institutions who underwent clinical echocardiography that was reported as normal. A generalized additive model for location, scale, and shape (GAMLSS) was used to model the expected distributions of measurements as a function of sex, height, weight, body mass index, and age. RESULTS: Compared with a model that only considered body surface area, the proposed Z scores demonstrated less bias in subgroups of overweight, young, and early school-aged children. CONCLUSIONS: The proposed Z score equations may improve diagnostic and therapeutic accuracy by ensuring that body size, body mass index, and age do not confound the interpretation of measurements.