Predictive Factors of Medium-Large Coronary Artery Aneurysm in Children with Acute Kawasaki Disease: A Retrospective Cohort Study

Background: The severity of the cardiac complications resulting from Kawasaki disease (KD) appears to be directly correlated to the magnitude of the coronary artery aneurysm (CAA). However, there remains some unclear about the risk factors for medium-large CAA identified after acute KD.Methods: We analyzed 90 patients diagnosed with CAA in KD hospitalized from January 2013 through August 2021. Patients were stratified based on the coronary artery z-score adjusted for body surface area as the medium-large CAA group and small-sized CAA group. The association of baseline characteristics was investigated within the groups. Multivariable logistic regression analyses were performed to evaluate potential risk factors associated with medium-large CAA development.Results: In total, 353 pediatric cases with KD were investigated during the study period, of whom 90 (25.5%) presented with CAA, including medium-large CAA in 20 patients (5.7%) after acute KD. The medium-large CAA group showed significantly higher Harada risk scores, the incidence of thrombosis, serum globulin concentration values, proportions of C-reactive protein > 40 mg/L, proportions of albumin < 35 g/L, and lower values of albumin-to-globulin ratio (A/G ratio) than those in the small-sized CAA group (P < 0.05). Medium-large CAA was significantly associated with the A/G ratio (odds ratio, 3.503; 95% confidence interval [CI]: 1.068–11.492). The area under the receiver operating characteristic curve was 0.684 (95% CI: 0.558–0.810), and the cutoff point of 1.35 showed a sensitivity and specificity for predicting medium-large CAA of 80% and 59%, respectively.Conclusions: A lower A/G ratio independently predicts medium-large CAA in patients with KD. Medium-large CAA is associated with greater odds of developing thrombosis. Thus, close monitoring with routine echocardiography is recommended.

Giant coronary fistula aneurysm presenting as a progressing left-sided asymptomatic mediastinal mass with systolic dominant Doppler flow: a case report

Background Cases of giant coronary artery aneurysms associated with coronary fistula are rarely reported, and they present with various symptoms, including coronary steal syndrome. We report an uncommon case of an asymptomatic giant coronary fistula aneurysm presenting as a progressing left-sided mediastinal mass that has been tracked for years. Case summary A 67-year-old healthy asymptomatic woman was referred to our hospital because of an abnormal shadow on her chest radiography revealing a left-sided mediastinal mass that had progressed in size over the past 4 years. Computed tomography revealed mass progression from 4 cm to 5 cm in diameter within 2 years. Coronary computed tomography and coronary angiography identified a giant coronary artery aneurysm in a coronary fistula originating in the left anterior descending artery and draining into the main pulmonary artery. Transthoracic Doppler echocardiography revealed a unique systolic-dominant flow. She underwent coronary artery aneurysmectomy and fistula ligation. The patient has been in good health without any events for 10 months since her discharge. Discussion A giant coronary artery aneurysm in a coronary fistula can present as an asymptomatic left-sided mediastinal mass that has progressed in size for years in older adults. Echocardiography can provide clues of the steal phenomenon in coronary artery fistula. A close investigation of mediastinal abnormalities can facilitate the detection of coronary aneurysms.

Blood routine risk factors for coronary artery aneurysm in infants younger than 8 months with Kawasaki disease

Objective The aims of this study were to characterize the evolution of routine blood values within the first 10 days of illness and coronary artery outcome in infants < 8 months with Kawasaki disease (KD) and to identify risk factors for coronary artery aneurysm (CAA). Methods Laboratory data, clinical features and coronary artery outcomes from 78 infants < 8 months old and 86 patients between 8 months and 7 years old were retrospectively analyzed. Logistic regression analysis was conducted to evaluate the potential risk factors for CAA. Results Infants < 8 months old were more likely to present with incomplete KD (37.2% vs 4.7%, P < 0.001), erythema and induration at the BCG inoculation site (24.4% vs 3.5%, P < 0.001) and CAA (47.4% vs 15.1%, P < 0.001) even with timely diagnosis and treatment with intravenous immunoglobulin (IVIG) compared with patients ≥8 months old. Clinical feature related to diagnostic criteria for KD including bilateral conjunctival injection, oral changes, unilateral cervical lymphadenopathy and extremity changes were less common in the younger group. During the acute phase, the percentage neutrophils and neutrophil to lymphocyte ratio [NLR] peaked on median illness day 3, followed by white blood cell (WBC) and CRP on median illness day 4, hemoglobin on median illness day 7 and platelet count on median illness day 9. CAA occurred on median illness day 6 and regressed on median illness day 28. Multivariate logistic regression analysis revealed that the peak percentage neutrophils (odds ratio [OR] per 0.1: 1.597, 95% confidence interval [CI]: 1.041–2.452, P = 0.032) and the peak platelet count (OR per 10 × 109/L: 1.029, 95% CI: 1.004–1.055, P = 0.024) were independent risk factors for CAA. Hemoglobin on the 5th day was associated with persistent CAA at 1 year after KD onset. Conclusion Factors associated with CAA include a high peak percentage neutrophils, increased peak platelet count, and reduced hemoglobin within 4–6 days during the acute phase of KD. Therefore, this population should receive primary therapy with IVIG and adjunctive anti-inflammatory medications.

Sex differences in treatment strategy for coronary artery aneurysms: Insights from the international Coronary Artery Aneurysm Registry

Introduction Sex disparities exist in coronary artery disease (CAD) in terms of risk profile, clinical management and outcome. It is unclear if differences are also present in coronary aneurysms, a rare variant of CAD. Methods Patients were selected from the international Coronary Artery Aneurysm Registry (CAAR; ClinicalTrials.gov: NCT02563626), and differences between groups were analysed according to sex. The CAAR database is a prospective multicentre registry of 1565 patients with coronary aneurysms (336 females). Kaplan-Meier method was used for event-free survival analysis for death, major adverse cardiac events (MACE: composite endpoint of death, heart failure and acute coronary syndrome) and bleeding. Results Female patients were older, were more often hypertensive and less frequently smoker. They were treated conservatively more often compared to male patients and received significantly less frequently aspirin (92% vs 88%, p = 0.002) or dual antiplatelet therapy (DAPT) (67% vs 58%, p = 0.001) at discharge. Median DAPT duration was also shorter (3 vs 9 months, p = 0.001). Kaplan-Meier analysis revealed no sex differences in death, MACE or bleeding during a median follow-up duration of 37 months, although male patients did experience acute coronary syndrome (ACS) more often during follow-up (15% vs 10%, p = 0.015). Conclusions These CAAR findings showed a comparable high-risk cardiovascular risk profile for both sexes. Female patients were treated conservatively more often and received DAPT less often at discharge, with a shorter DAPT duration. ACS was more prevalent among male patients; however, overall clinical outcome was not different between male and female patients during follow-up.