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Kaplan-Meier curves for the coronary arterial aneurysm (CAA) regression rate by severity of CAA. Regression rate classified by CAA severity in the right coronary artery (RCA) (A) and the left anterior descending (LAD) (B).
Source publication
Background
Coronary arterial aneurysms (CAAs) associated with Kawasaki disease (KD) significantly affect prognosis. However, the clinical course of CAAs and factors associated with CAA regression have not been well analyzed.
Methods and Results
The cohort of the Z‐Score 2nd Project Stage study, a multicenter, retrospective, cohort study involving...
Context in source publication
Context 1
... the RCA, the regression rates of CAAs 5 and 10 years after diagnosis were 86.8% and 95.5% for small, 75.5% and 83.2% for medium, and 29.6% and 36.3% for large, respectively. In the LAD, the regression rates 5 and 10 years after diagnosis were 87.6% and 95.3% for small, 69.9% and 80.1% for medium, and 20.8% and 28.8% for large, respectively ( Figure 1A and 1B). Figure 2 shows the Kaplan-Meier curves of the regression rate classified by CAA severity in male and female patients. In the RCA, the 10-year regression rate was 96.2%, 79.9%, and 27.3% in male patients and 92.5%, 93.2%, and 50.2% in female patients with small, medium, and large CAAs, respectively. ...
Citations
... Once CAAs have developed, they remain without regression in about 30% of the cases, and persistent CAAs pose a life-long risk of thrombosis beyond childhood. 1,2 Flow turbulence in cavernous CAAs is thought to cause the thrombosis, but atherosclerotic changes in the vessel walls have been reported in the convalescent phase of Kawasaki disease and they might also contribute to the cardiovascular events. 3 Given the rise in newly diagnosed cases of Kawasaki disease, there is presumably a substantial number of adult patients who carry these pathological conditions, which could be an issue when following and treating adult patients with a history of Kawasaki disease. ...
Background
The long-term impact of Kawasaki disease on coronary arteries in vivo is unclear.
Objectives
The purpose of this study was to investigate coronary arteries in the late convalescent phase, we followed patients with Kawasaki disease who developed coronary artery aneurysms (CAAs).
Methods
We followed 24 patients and used optical coherence tomography at a median of 16.6 years after the onset of Kawasaki disease.
Results
Of 72 coronary arteries, optical coherence tomography was performed on 61 arteries: 17 with a persistent CAA, 29 with a regressed CAA, and 15 without a CAA. Between-group comparison was performed by chi-square or Fisher’s exact test, and intimal thickening (17 vs 29 vs 15, all 100%, P = NA) and medial disruption (17 [100%] vs 29 [100%] vs 14 [93%], P = 0.25) were commonly observed in the investigated arteries. Advanced features of atherosclerosis were more frequently seen in arteries with persistent CAAs than in those with regressed CAAs and in those without CAAs: calcification (12 [71%] vs 5 [17%] vs 1 [7%], P < 0.001), microvessels (12 [71%] vs 10 [35%] vs 4 [27%], P = 0.020), cholesterol crystals (6 [35%] vs 2 [7%] vs 0 [0%], P = 0.009), macrophage accumulation (11 [65%] vs 4 [14%] vs 4 [27%], P = 0.002), and layered plaque (8 [47%] vs 11 [38%] vs 0 [0%], P = 0.004).
Conclusions
Long after onset of Kawasaki disease, all arteries showed pathological changes. Arteries with persistent CAAs had more advanced features of atherosclerosis than those with regressed CAAs and those without CAAs.
... Atherosclerosis is accountable for more than 90% of CAA in adults, whereas in children, Kawasaki disease is responsible for most cases. 6 The pathophysiology of CAA remains unclear but is perceived to be similar to that for large vessel aneurysms. In the study done by Berkoff and Rowe, 7 it was hypothesized that the presence of a thin degenerated media, adjacent to intimal plaque was the major pathological prerequisite leading to plaque rupture. ...
A 22-year-old male having cardiomegaly with prominent right atrium (RA) enlargement in his chest X-ray underwent echocardiography which revealed an anterior mediastinal cystic lesion. Contrast-enhanced computed tomography of the thorax showed saccular contrast filling outpouching from ostia of the right coronary artery (RCA) of size 5.9 × 6.4 cm in anterior atrioventricular groove compressing RA and right ventricle along the RCA course. The left main coronary artery also appeared to be dilated with a diameter of 1.1 cm proximally. The patient denied any prior angina, palpitation, and dyspnea on exertion. This case is reported for its rarity and the dilemma involving its appropriate medical and surgical management.
... 2,18 Additionally, the risk of the development of CAA and the clinical and laboratory features differ between younger and older children with KD. 8,14 Older children (over 5 years of age) have a higher incidence of CAA and a lower rate of CAA regression than younger children. 6,19 Thus, making clinical decisions for KD children aged > 5 based on the probability of CAA at 4À8 weeks may be effective. ...
Objective:
Reliably prediction models for coronary artery abnormalities (CAA) in children aged >5 years with Kawasaki disease (KD) are still lacking. This study aimed to develop a nomogram model for predicting CAA at 4 to 8 weeks of illness in children with KD older than 5 years.
Methods:
A total of 644 eligible children were randomly assigned to a training cohort (n = 450) and a validation cohort (n = 194). The least absolute shrinkage and selection operator (LASSO) analysis was used for optimal predictors selection, and multivariate logistic regression was used to develop a nomogram model based on the selected predictors. Area under the receiver operating characteristic curve (AUC), calibration curves, Hosmer-Lemeshow test, Brier score, and decision curve analysis (DCA) were used to assess model performance.
Results:
Neutrophil to lymphocyte ratio, intravenous immunoglobulin resistance, and maximum baseline z-score ≥ 2.5 were identified by LASSO as significant predictors. The model incorporating these variables showed good discrimination and calibration capacities in both training and validation cohorts. The AUC of the training cohort and validation cohort were 0.854 and 0.850, respectively. The DCA confirmed the clinical usefulness of the nomogram model.
Conclusions:
A novel nomogram model was established to accurately assess the risk of CAA at 4-8 weeks of onset among KD children older than 5 years, which may aid clinical decision-making.
Backgrounds:
This study aimed to identify risk factors for the progression of coronary artery lesions (CALs) in children with Kawasaki disease (KD) and to develop a nomogram prediction model.
Methods:
This is a retrospective case-control study in which the participants were categorized into three groups based on the changes of the maximum Z score (Zmax) of coronary arteries at the 1-month follow-up compared with the baseline Zmax: CALs-progressed, CALs-improved, and CALs-unchanged.
Results:
Of total 387 patients, 65 (27%), 319 (73%), and 3 (0.7%) patients were categorized into CALs-progressed group, CALs-improved group, and CALs-unchanged group, respectively. Six independent factors associated with CALs progression were identified, including initial IVIG resistance, baseline Zmax, the number of coronary arteries involved, C-reactive protein, albumin, and soluble interleukin-2 receptor (odds ratio: 7.19, 1.51, 2.32, 1.52, 0.86, and 1.46, respectively; all P-values < 0.01). The nomogram prediction model including these six independent risk factors yielded an area under the curve (AUC) of 0.80 (95% confidence interval, 0.74 to 0.86). The accuracy of this model reached 81.7% after the Monte-Carlo Bootstrapping 1000 repetitions.
Conclusions:
The nomogram prediction model can identify children at high risk for the progression of CALs at early stages.
Impact:
Six independent factors associated with CALs progression were identified, including initial IVIG resistance, baseline Zmax, the number of coronary arteries involved, CRP, ALB, and sIL-2R. The prediction model we constructed can identify children at high risk for the progression of CALs at early stages and help clinicians make individualized treatment plans. Prospective, multi-centered studies with larger sample sizes are warranted to validate the power of this prediction model in children with KD.
The most severe complication of Kawasaki disease, an inflammatory disorder of young children, is the formation of coronary artery aneurysms. It is known that patients with coronary artery aneurysms, particularly those with medium and large lesions, have a higher risk of future major cardiovascular events. In contrast, there is a lack of data on the cardiovascular status in long-term follow-up for Kawasaki disease patients without coronary involvement or with self-limited coronary artery aneurysms, resulting in most patients being discharged after 5 years. Even though some paediatricians may believe these patients should not be followed at all, studies indicating a dysfunctional endothelium show the need for further investigation. Consequently, a review of the most significant aspects of Kawasaki disease, and the necessity of correctly identifying, treating and monitoring these patients, particularly those with a higher risk of complications, was conducted.
