Persistent Coronary Artery Aneurysms Research Articles

Jaundice-predominant manifestation of Kawasaki disease in children.

A jaundice-predominant presentation of Kawasaki disease (KD) is atypical. A total of 12 children with KD with a predominant manifestation of jaundice at MacKay Children's Hospital were reviewed, along with 42 cases reported in the literature since 1990. The median age of the 12 patients was 1.85 years (range: 3 months-4 years), and 66.6% were male. All of the patients had elevated liver function at presentation, 50% had hydrops of the gallbladder, and almost 60% had gastrointestinal symptoms and signs. Complete KD was evident in 11 of the 12 patients (91.7%), and two patients (16.7%) had recurrent episodes. All of the patients received intravenous immunoglobulin (IVIG); however, one-third were refractory to treatment. Corticosteroids were used in five (41.7%) of the patients. Three (25%) of the patients had shock, and seven (58.3%) had coronary artery abnormalities, of whom one (8.3%) had persistent coronary artery aneurysm and the others recovered. A review of the 42 cases in the literature showed that the children with a jaundice-predominant presentation of KD had high rates of IVIG-refractory disease (25%), coronary artery abnormalities (25%), shock (13.2%), and corticosteroid treatment (24.2%). Children with KD presenting with a jaundice-predominant manifestation are at a higher risk of IVIG-refractory disease, coronary artery abnormalities, and more recurrent episodes. Physicians should be aware of the risk of shock in this population.

Evaluation of the Association Between Coronary Artery Aneurysms and Concomitant Infection in Patients With Kawasaki Disease.

We analyzed the relationship between recovery from coronary artery aneurysms (CAAs) and concurrent infections in patients with Kawasaki disease (KD). The estimated median time of aneurysm persistence between patients with and without infections was compared using Kaplan-Meier survival analyses. Risk factors associated with persistent CAAs at 2 years were identified using multivariable analyses. Co-infection was confirmed in 20.5% (106/518) of patients diagnosed with KD. No significant differences regarding treatment or coronary artery outcome were identified between patients with and without infections. The estimated median time of aneurysm persistence was higher in the co-infected group (9 vs. 6 months). A maximum Z-score ≥ 4.00 at 1 month had 78% sensitivity and 83% specificity in predicting CAAs without recovery within 1 year of onset, whereas the predictability was higher within 2 years of onset, with a Z-score ≥ 4.88 (sensitivity, 92%; specificity, 91%). Concomitant infections did not affect the response to treatment or coronary artery outcomes in patients with KD.

Development of a nomogram prediction model for early identification of persistent coronary artery aneurysms in kawasaki disease

BackgroundCoronary artery aneurysms (CAA) persistence prediction is critical in evaluating Kawasaki disease (KD). This study established a nomogram prediction system based on potential risk factors for assessing the risk of CAA persistence in a contemporary cohort of patients with KD.MethodsThis cohort comprised 105 patients with KD who had been diagnosed with CAA during the acute or subacute phase by echocardiography. The follow-up duration was at least 1 year. The clinical and laboratory parameters were compared between the CAA regression and persistence groups. Multivariable logistic regression analysis was used to identify the independent risk factors for CAA persistence, which were subsequently used to build the nomogram predictive model. Decision curve analysis was used to assess the net benefits of different nomogram scores.ResultsOf these patients with CAA, 27.6% of patients presented with persistent lesions. The incidences of CAA persistence were 14.1%, 81.3%, and 100.0% in patients with small, medium, and large aneurysms, respectively. The ratio of neutrophils to lymphocytes, γ-GT, and CAA size at diagnosis were considered as the independent risk factors for CAA persistence in patients with KD. The nomogram predictive models yielded a high capability in predicting CAA persistence, based on either univariable or multivariable analyses-identified parameters, compared with using CAA size as a single predictor.ConclusionThe initial ratio of neutrophils to lymphocytes, γ-GT, and CAA size were the independent risk factors for CAA persistence in patients with KD. Nomogram scores could help elevate predictive efficacy in detecting CAA persistence.

Prognostic nutritional index value in the prognosis of Kawasaki disease with coronary artery lesions.

The prognostic nutritional index (PNI) is a purported predictor of intravenous immunoglobulin (IVIG) resistance and coronary artery aneurysm (CAA) development in patients with Kawasaki disease (KD). However, limited data exist on CAA regression. This study aimed to confirm whether the PNI is a predictor for CAA persistency in patients with KD. This retrospective study grouped 341 patients with KD based on the coronary artery status and time of aneurysm persistence. The clinical and laboratory parameters were compared, and multivariate logistic regression analysis was performed to identify the independent risk factors for persistent CAA. The receiver operating characteristic (ROC) curve was further used to assess the predictive values of the PNI in persistent CAA. Among the study patients, 80 (23.5%) presented with CAA, including CAA persisting for 2 years in 17 patients (5.0%). Patients with CAA were more frequently treated with corticosteroids (p < 0.016). No statistically significant differences were found in the nutritional status and PNI among patients with or without coronary artery lesions, regardless of injury severity. Patients in the persistent CAA group presented with higher rates of overnutrition and showed lower PNI values and a higher incidence of thrombosis than those in the normal group (p < 0.05). The PNI and the maximum Z-score at 1 month of onset were significantly associated with CAA persisting for 2 years and may be used as predictors of persistent CAA. The area under the ROC curve was 0.708 (95% confidence interval, 0.569-0.847), and a 40.2 PNI cutoff yielded a sensitivity and specificity of 41 and 92%, respectively, for predicting CAA persisting for 2 years. Kaplan-Meier survival analysis revealed that the estimated median time of aneurysm persistence was significantly higher in patients with PNI values of ≤40 than in those with PNI values of >40 (hazard ratio, 2.958; 95% confidence interval, 1.601-5.464; p = 0.007). After sampling-time stratification, the PNI differed significantly between patients with and without persistent CAA when sampled on the second (p = 0.040), third (p = 0.028), and fourth days (p = 0.041) following disease onset. A lower PNI value is an independent risk factor for CAA persisting for 2 years in patients with KD, besides the maximum Z-score at 1 month after onset. Furthermore, the PNI obtained within 4 days from fever onset may possess greater predictive power for patients with persistent CAA.

Blood routine risk factors for coronary artery aneurysm in infants younger than 8\u2009months with Kawasaki disease

ObjectiveThe 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).MethodsLaboratory 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.ResultsInfants < 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.ConclusionFactors 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.

Deep Learning-Based Approach to Automatically Assess Coronary Distensibility Following Kawasaki Disease

Kawasaki disease is an acute vasculitis affecting children, which can lead to coronary artery (CA) aneurysms. Optical coherence tomography (OCT) has identified CA wall damage in KD patients, but it is unclear if these findings correlate with any distensibility changes in the CA and how these changes evolve over time. This paper seeks to establish the link between OCT findings and vessel distensibility with a novel deep learning coronary artery segmentation system and use the segmentation framework to automatically analyze the temporal evolution of coronary stiffness over many years. 27 KD patients underwent catheterization with coronary angiography of the left coronary artery (LCA), followed by OCT of proximal and distal segments of the LCA. Changes in the CA caliber over the cardiac cycle were measured automatically and compared against OCT findings suggestive of KD-related vascular damage. In addition, 34 KD patients with regressed or persistent CA aneurysms were followed with serial CA angiography over an average of 14.5years. Distensibility changes were calculated using adeep learning coronary artery segmentation framework and evaluated longitudinally. Distensibility in the coronary arteries after KD negatively correlated with increasing severity of OCT findings of KD-related vessel damage. KD patients have a significant increase in CA wall stiffness at 1year after diagnosis, which then plateaus subsequently, compared to controls. Also, patients with persistent CA aneurysms have a statistically significant increase in wall stiffness over time in comparison to those with regressed CA aneurysms. Distensibility changes in the CA of KD patients calculated using our automated deep learning approach correlates with the severity of OCT findings of KD-related CA damage. This decreased distensibility peaks at 1year in KD patients when following longitudinally and is more severe in those with persistent CA aneurysms.

Impact of Early Treatment With High-Dose Intravenous Immunoglobulin on Incidence of Kawasaki Disease Complications in Iranian Children.

Objective: Kawasaki disease (KD) occurs in five-year-old or younger children. This study aimed to evaluate the impact of high-dose intravenous immunoglobulin plus acetylsalicylic acid therapy on the prevention and treatment of coronary artery lesions and to evaluate the impact of high-dose acetylsalicylic acid (ASA) on the hearing of the patients. Materials and methods: In this retrospective cohort study, 31 patients with KD were followed from January 2012 to December 2015. The clinical, para-clinical, color Doppler echocardiogram and audiometry results were evaluated. Results: Overall, seven cases (22.6%) developed coronary artery aneurysm (CAA) in the acute phase of the disease, of whom only two still had CAA at the end of the treatment (6%). One of the five children with CAA recovery had a delay in the onset of treatment and one of two patients with persistent CAA at the end of treatment was admitted within the first 10 days. There was no evidence-based abnormal liver biochemical test. None of the patients developed sensorineural hearing loss (SNHL) on audiometry tests conducted before and after treatment. Conclusion: Recovery of coronary artery lesions was 71.43% after 28 days of the onset of treatment. The distribution of coronary artery aneurysm was not different in terms of the time of the treatment initiation (P-Value = 0.371). None of the children had a sensorineural hearing loss (SNHL) 48 hours and 4 weeks after treatment.

Biomarkers of inflammation and fibrosis in young adults with history of Kawasaki disease.

BackgroundMyocardial histology from autopsies of young adults with giant coronary artery aneurysms following Kawasaki disease (KD) shows bridging fibrosis beyond the territories supplied by the aneurysmal arteries. The etiology of this fibrosis is unknown, but persistent, low-level myocardial inflammation and microcirculatory ischemia are both possible contributing factors. To investigate the possibility of subclinical myocardial inflammation or fibrosis, we measured validated biomarkers in young adults with a remote history of KD.MethodsWe measured plasma calprotectin, galectin-3 (Gal-3), growth differentiation factor-15 (GDF-15), soluble ST2 (sST2), and serum procollagen type 1C-terminal propeptide (P1CP) in 91 otherwise healthy young adults with a remote history of KD and in 88 age-similar, healthy controls. KD subjects were stratified by coronary artery aneurysm (CAA) status and history of remote myocardial infarction (MI).ResultsAfter correction for multiple testing, calprotectin, Gal-3, and GDF-15 levels were significantly higher in subjects with persistent CAA (n = 26) compared with KD subjects with remodeled CAA (n = 20, p = 0.005, 0.001, 0.0036, respectively). In a multivariable regression model with CA status as the main predictor and adjusting for sex, MI history, and interval from KD onset, CA status was a significant predictor (Persistent CAA vs KD Normal CA) of calprotectin, Gal-3, GDF-15 and sST2 levels (p = 0.004, <0.001, 0.007, and 0.049, respectively).ConclusionsThese results suggest that ongoing inflammation and fibrosis may be occurring in individuals with persistent CAA. Longitudinal follow-up is needed to clarify the clinical significance of these elevated biomarker levels in this patient population that requires life-long monitoring.

Bilateral internal thoracic artery grafting in children under 5 years of age with Kawasaki disease: a case series.

BackgroundAlthough persistent coronary artery aneurysm of Kawasaki disease (KD) is rare, some patients develop a life-threatening myocardial infarction. In paediatric coronary artery bypass surgery (PCABS), the internal thoracic artery (ITA) graft is a reliable graft with favourable coronary outcomes. However, few studies have reported the outcomes and technical considerations of PCABS using bilateral ITAs in small children who have multivessel disease.Case summaryWe present the cases of three children under 5 years of age who underwent PCABS utilizing bilateral ITAs. All three patients had known bilateral giant coronary aneurysms associated with KD. Paediatric coronary artery bypass surgery was indicated after confirming multiple coronary lesions with myocardial ischaemia. One child underwent emergency PCABS because of circulatory collapse. The arterial anastomoses were performed under cardioplegic arrest. The left ITA was anastomosed to the left anterior descending artery (LAD) or the circumflex artery. The right ITA was anastomosed to the right coronary artery or the LAD. Post-operative coronary angiography revealed patent bilateral ITA grafts with an excellent run-off in all patients, and none of them have suffered any subsequent coronary event.DiscussionBilateral ITA grafting is a feasible procedure with favourable coronary outcomes for treating small children with multivessel disease, even in the setting of circulatory collapse.

Abstract 12598: Stress Testing During Long-term Follow-up After Kawasaki Disease

Introduction: The American Heart Association (AHA) guidelines recommend testing for inducible myocardial ischemia in all patients with a history of coronary artery aneurysm (CAA) after Kawasaki disease (KD). Because the prevalence of clinical coronary complications is exceedingly low in patients whose worst-ever CAA dimension was &lt;large/giant, we examined the yield of stress testing in KD over a 20-year period. Methods: Retrospective study including patients &lt;18 yo with KD who underwent cardiac imaging to evaluate for inducible ischemia between 2000-2019. Patients with a prior coronary artery intervention were excluded. Inducible ischemia was defined as stress-induced reversible wall motion abnormalities on echocardiogram or cardiac MRI, or reversible defect on nuclear myocardial perfusion imaging. Results: A total of 588 stress tests were performed in 208 patients at median age of 12.4 [IQR 8.8, 16.8] years, 8.3 [IQR 5.0, 12.9] years after diagnosis (Table). Tests were performed using either exercise stress (545 [93%] tests) or pharmacologic stress with dobutamine (43 [7%] tests). Echocardiography was the most frequently used modality (n=481, 82%), followed by nuclear imaging (n=133, 23%) and cardiac MRI (n=53, 9%). Inducible ischemia was found on 11 (2%) tests in 6 (3%) patients. All patients with inducible ischemia had a history of giant CAA with persistent CAA at time of testing (moderate CAA in 2 (18%) tests, giant CAA in 9 (82%) tests). After finding inducible ischemia on stress test, coronary artery bypass grafting was performed in 3 cases, cardiac catheterization in 4, stress testing using a different imaging modality in 1, and careful clinical monitoring without intervention in 3. Conclusions: Inducible ischemia was found in 2% of test after KD, and only in patients with a history of giant CAA. Recommendations in the 2017 AHA guidelines for KD for testing for myocardial ischemia in patients with non-giant CAA should be reconsidered in light of these findings.

Primary adjunctive corticosteroid therapy is associated with improved outcomes for patients with Kawasaki disease with coronary artery aneurysms at diagnosis

ObjectivePatients with Kawasaki disease (KD) with coronary artery enlargement at diagnosis are at the highest risk for persistent coronary artery aneurysms (CAAs) and may benefit from primary adjunctive anti-inflammatory therapy...

Delayed Development of Coronary Artery Aneurysm in Patients with Kawasaki Disease Who Were Clinically Responsive to Immunoglobulin

To clarify the frequency and characteristics of discrepant outcomes of intravenous immunoglobulin (IVIG) between fever and coronary artery aneurysms (CAAs) in patients with Kawasaki disease. This study included 325 patients who responded to oral aspirin and IVIG alone. The main outcome was CAA 4weeks after disease onset. CAA was defined as ≥2.5 of maximum z score (Zmax) representing the highest value of 4 coronary artery branches. Immunoglobulin dosage and sequential changes in Zmax were reviewed to investigate the effects on fever and timing of CAA development. Logistic regression analyses with receiver operating characteristic curves using clinical and laboratory variables including the initial Zmax were performed to identify predictors of CAA at 4weeks. CAAs were either persistent or appeared de novo 4weeks after diagnosis in 13 of 325 patients who responded to a single or repeated IVIG. Four single-dose IVIG-responders developed CAA although they had pretreatment Zmax of <2.0. The 2 single-dose IVIG responders with the greatest pretreatment Zmax (>4.5) developed persistent CAA. Receiver operating characteristic analysis demonstrated Zmax of 2.57 as the cut-off for predicting CAA. Multivariable analyses identified >2.5 Zmax (OR 9.08, 95% CI 1.26-65.3, P=.028, 50% sensitivity, 91% specificity) as the sole risk factor for CAA at 4weeks in single-dose IVIG responders. Delayed development and persistence of CAA in single-dose IVIG responders indicate that some factors other than those responsible for systemic inflammation may contribute to vasculitis in CAA. Baseline Zmax 2.5 aids in predicting CAAs.

Multisystem Inflammatory Syndrome in Children and Kawasaki Disease: Two Different Illnesses with Overlapping Clinical Features

Multisystem Inflammatory Syndrome in Children and Kawasaki Disease: Two Different Illnesses with Overlapping Clinical Features

Von Willebrand factor parameters as potential biomarkers for disease activity and coronary artery lesion in patients with Kawasaki disease.

Elevated von Willebrand factor (vWF):Antigen plasma levels have been observed in conjunction with cardiovascular diseases or vasculitis. The association of Kawasaki disease, a vascular inflammatory disease and vWF:Antigen, vWF:Collagen binding activity, and vWF multimers is unknown. We therefore investigated vWF parameters in 28 patients with acute Kawasaki disease in association with disease activity and coronary artery lesions. VWF:Antigen and vWF:Collagen binding activity were assessed via enzyme-linked immunoassay. The ratio of both (vWF:Collagen binding activity and VWF:Antigen) was calculated and vWF multimeric structure analysis performed. We analyzed the association between vWF parameters and our clinical data focusing on coronary artery outcome. VWF:Antigen and vWF:Collagen binding activity levels were significantly higher in the acute than in the disease's convalescence phase, and correlated positively with CRP levels. Neither variable was associated with coronary artery lesions. The vWF:Collagen binding activity/vWF:Antigen ratio, however, was significantly decreased in patients with a coronary artery lesion (z-score > 2; N = 10; mean ratio 0.96 vs. 0.64; p = 0.031) and even more so in those with a coronary artery aneurysm (z-score > 2.5; N = 8; mean ratio 0.94 vs. 0.55; p = 0.02). In a sub-analysis, those patients with a very low ratio in the acute phase presented a persistent coronary artery aneurysm at their 1-year follow-up.Conclusion: This study suggests that comprehensive analysis of vWF parameters may help to both monitor KD inflammation and facilitate the identification of those patients carrying an increased risk for coronary artery lesion.What is Known:• Von Willebrand factor (VWF)-parameters represent surrogate markers for vascular inflammation.• Kawasaki disease is a generalized vasculitis in children, which can be complicated by coronary artery lesions.What is New:• In those Kawasaki disease patients with coronary artery lesions, the vWF:CB/vWF:Ag ratio was significantly decreased.• VWF parameters may help to identify patients at risk for coronary artery lesions.

Hemodynamic variables in aneurysms are associated with thrombotic risk in children with Kawasaki disease

BackgroundThrombosis is a major adverse outcome associated with coronary artery aneurysms (CAAs) resulting from Kawasaki disease (KD). Clinical guidelines recommend initiation of anticoagulation therapy with maximum CAA diameter (Dmax) ≥8 mm or Z-score ≥ 10. Here, we investigate the role of aneurysm hemodynamics as a superior method for thrombotic risk stratification in KD patients. Methods and resultsWe retrospectively studied ten KD patients with CAAs, including five patients who developed thrombosis. We constructed patient-specific anatomic models from cardiac magnetic resonance images and performed computational hemodynamic simulations using SimVascular. Our simulations incorporated pulsatile flow, deformable arterial walls and boundary conditions automatically tuned to match patient-specific arterial pressure and cardiac output. From simulation results, we derived local hemodynamic variables including time-averaged wall shear stress (TAWSS), low wall shear stress exposure, and oscillatory shear index (OSI). Local TAWSS was significantly lower in CAAs that developed thrombosis (1.2 ± 0.94 vs. 7.28 ± 9.77 dynes/cm2, p = 0.006) and the fraction of CAA surface area exposed to low wall shear stress was larger (0.69 ± 0.17 vs. 0.25 ± 0.26%, p = 0.005). Similarly, longer residence times were obtained in branches where thrombosis was confirmed (9.07 ± 6.26 vs. 2.05 ± 2.91 cycles, p = 0.004). No significant differences were found for OSI or anatomical measurements such us Dmax and Z-score. Assessment of thrombotic risk according to hemodynamic variables had higher sensitivity and specificity compared to standard clinical metrics (Dmax, Z-score). ConclusionsHemodynamic variables can be obtained non-invasively via simulation and may provide improved thrombotic risk stratification compared to current diameter-based metrics, facilitating long-term clinical management of KD patients with persistent CAAs.

Failure to Predict High-risk Kawasaki Disease Patients in a Population-based Study Cohort in Germany

Diverse scores on high-risk Kawasaki disease (KD) patients have proven a good prognostic validity in the Japanese population. However, data on non-Japanese have been inconclusive. Do the Kobayashi, Egami and Sano scores or application of up-to-date statistical methods (Random Forest) predict response to standard intravenous immunoglobulin (IVIG) therapy and the risk of persistent coronary artery aneurysm (CAA) in patients with KD in a mainly Caucasian population in Germany? Data on 442 children (German population-based survey, 2013 and 2014) were used to assess the prognostic validity of the Kobayashi, Egami and Sano scores for being refractory to IVIG treatment and for predicting the risk of persistent CAA. Additionally, an up-to-date statistical approach (Random Forest) was applied to identify a potentially more valid score. A total of 301 children were eligible for assessment of their response to IVIG treatment. Among those, 177 children were followed-up for 1 year to identify persistent CAA. Although all scores were significantly associated with being refractory to IVIG (relative risk range between 2.32 and 3.73), the prognostic properties were low (likelihood ratio positive: 1.83-4.57; sensitivity in the range of 0.28-0.53). None of the scores was a significant predictor of CAA 1 year after acute illness. Application of statistical analysis such as Random Forest did not yield a more valid score. None of the available scores appears to be appropriate for identifying high-risk Caucasian children with KD who might need intensified therapy.

Coronary artery aneurysm regression after Kawasaki disease and associated risk factors: a 3-year follow-up study in East China.

Kawasaki disease (KD) is the leading cause of acquired heart disease due to its complicated coronary artery lesions. Up to now, few studies were focused on the status of persistent coronary artery aneurysms (CAA) in KD patients. The present study was designed to identify the coronary artery outcomes and seek the risk factors associated with the regression of CAA in KD patients. One hundred and twenty KD patients with CAA hospitalized in Children's Hospital of Soochow University from Jan 2008 to Dec 2013 were prospectively studied by a 3-year follow-up. Data regarding demographic, clinical, laboratory, and echocardiographic characteristics were documented and further analyzed. It was estimated that 39.2% of the patients had complete regression of CAA within 4weeks, 59.2% within 8weeks, and 70.0% within 16weeks. No fatal cardiac events occurred. We found patients who aged ≤ 1year, received initial intravenous immunoglobulin (IVIG) treatment after the 10th day of illness, and IVIG non-responders were associated with the regression of persistent CAA. The relative risks were 1.55, 1.87, and 1.88, respectively. Age, initial IVIG treatment, and IVIG response were risk factors of persistent CAA, and more attention should be paid on these patients.

REGRESSED CORONARY ANEURYSM AFTER KAWASAKI DISEASE: AN OPTICAL COHERENCE TOMOGRAPHY (OCT) STUDY FROM A SINGLE INSTITUTION

Coronary artery aneurysms (CAA) are a serious complication of Kawasaki disease (KD). CAA size regresses in nearly 50% of the cases on follow up. Nevertheless, patients with regressed CAA have an increased risk of ischemic heart disease, myocardial infarction and sudden cardiac death on long-term follow-up. Actual imaging techniques often describe these segments as normal, whereas studies have shown significant endothelial and smooth muscle dysfunction. Optical Coherence Tomography (OCT) is a high-resolution intracoronary imaging modality that characterizes coronary artery wall structure. KD patients scheduled for routine coronary angiography underwent OCT imaging between March 2013 and April 2015. The aim of the study was to compare microstructural coronary changes in coronary artery segments with no history of CAA to segments with regressed CAA, and segments with persistent CAA. OCT was performed on 27 patients at 12.4 ± 5.5 years, 9.3 ± 5.3 years following onset of KD. Overall, 22/27 (81.5%) had a history of CAA. Of those, 11/22 (50.0%) had regressed CAA at time of OCT. Data was analyzed according to echocardiographic and angiographic progress of CAA segments. All 19/19 (100.0%) persistent CAA segments and 20/23 (87.0%) regressed CAA segments had significant intimal hyperplasia, compared to 2/19 (10.5%) with no history of segmental CAA (P<0.001), amounting 475.0 ± 179.1 μm, 305.4 ± 164.2 μm and 79.6 ± 78.2 μm respectively (p<0.001). Also, partial disappearance of the media was found more frequently in segments with persistent CAA, 17/19 (89.5%), followed by segments with regressed CAA, 12/23 (52.2%), and those with no history of CAA, 1/19 (5.3%); p<0.001. Neovascularization was found more often in segments with persistent CAA, 8/19 (42.1%), compared to segments with regressed CAA, 3/23 (13.0%), and no history of CAA, 0/19; p=0.002. Calcifications were identified almost exclusively in patients with persistent CAA, 6/19 (31.6%), compared to 1/23 (4.3%) for segments with regressed CAA and 0/19 for segments with no history of CAA (p=0.004). Macrophage accumulation was identified more often in segments with persistent CAA compared to those with regressed CAA and no history of CAA, in 10/19 (52.6%), 3/23 (13.0%) and 3/19 (15.8%) respectively (p=0.007). Despite normal angiographic appearance, regressed CAA segments displayed significant intimal hyperplasia and disruption of the media, among other anomalies, similarly to segments with persistent CAA. These features represent an increased risk for adverse coronary events. The additional diagnostic value of OCT following KD may bring prognostic potential in future chronological series.View Large Image Figure ViewerDownload (PPT)

Abstract 163: Regressed Coronary Aneurysm after Kawasaki Disease: What are they hiding? An Optical Coherence Tomography (OCT) study

Background: Coronary artery aneurysms (CAA) are a serious complication of Kawasaki disease (KD). Regression of CAA occurs in 50% of the cases on follow up. Actual imaging techniques often described these segments as normal, whereas studies have shown significant endothelial and smooth muscle dysfunction. Method: KD patients scheduled for angiographic follow-up between June 2013 and August 2014 underwent OCT imaging. We compared coronary intimal changes in coronary artery segments with no history of CAA to segments with regressed CAA, and segments with persistent CAA. The intima was measured in sections adjacent to the CAA for the segments with persistent CAA, at the former CAA and adjacent sections for those who regressed, and at various corresponding sections for segments with no history of CAA. Results: OCT was performed on 18 patients at 12.4 ± 5.5 years. Overall 14/18 (77.7%) had a history of CAA. Of those, 7/14 (50.0%) had regressed CAA at time of OCT. Data was analyzed according to echocardiographic and angiographic progress of CAA segments. Accordingly, all 18/18 persistent CAA segments and 11/11 regressed CAA segments had significant intimal hyperplasia, compared to 1/13 with no history of segmental CAA (P&lt;0.001). The intensity of intimal hyperplasia is displayed in Table 1. Conclusion: Despite normal angiographic features, regressed CAA segments displayed significant intimal hyperplasia, similarly to those with persistent CAA. These features may present a risk of adverse coronary events.

Abstract 19: Epidemiologic Surveillance of Kawasaki Disease in Manitoba and Northwestern Ontario 2000-2010

Objective: The purpose of this study is to determine the incidence of persistent coronary artery sequelae due to KD between the First Nation/Metis (FN/M) and non First Nation children (n-FN) in the province of Manitoba and Northwestern Ontario, Canada. Methods: This is a 10-year (2000-2010) retrospective chart review study of all patients &lt; 18 years of age identified with KD and persistent coronary artery aneurysms. The data was obtained from the Children’s Hospital of Winnipeg medical records and the Electronic Echocardiography data based at the Variety Heart Centre. Results: A total of 103 children and adolescents with KD and with at least 3 echocardiography studies were enrolled. Complete or incomplete presentation of KD was determined according to the fulfillment for the disease classic criteria on diagnosis. Age at diagnosis ranged between 2 months to 4 years (median 0.5 years) with 2 patients diagnosed during their early infancy (age&lt; 90 days). The median follow up period was 4 years (1.3 to 6.3 years). Persistent coronary artery aneurysms were detected in 10 children (M/F ratio 3:1). Nine (9) of the studied subjects account for the FN/M children group and the remaining 1 is of Asian origin. We observed no n-FN children with permanent cardiac sequelae secondary to KD. Conclusion: There is a significantly higher occurrence of permanent coronary artery lesions due to KD among the FN/M children in the province of Manitoba and Northwestern Ontario. However, this study does not imply direct association of any risk factor, which might be related with our findings. Socio-environmental or even genetic factors might contribute to these findings. Nevertheless, these speculations are beyond the scope of this study. We suggest that a strategy of early, diagnosis and treatment of KD based on the latest AHA guidelines, when we deal with patients of these ethnic/racial groups, should be implemented.