Abstract 13850: Severity Assessment of Coronary Artery Aneurysm by Z-score of the Internal Diameter in Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Masaru Miura ◽  
Naoya Fukushima ◽  
Tetsuji Kaneko ◽  
Tohru Kobayashi ◽  
Kenji Suda ◽  
...  

Introduction: The severity of coronary artery aneurysms (CAA) in patients with Kawasaki disease (KD) has been recently classified according to the z-score. However, it is not known whether this classification can predict coronary events such as stenosis, obstruction, and thrombosis. Methods: In this multicenter retrospective study, data on height, weight, CAA diameter measured by echocardiography in the acute phase, and the clinical course in KD patients 18 years of age or younger who received a coronary angiography between 1992 and 2011 were collected. Time-dependent occurrence of coronary events was analyzed by Kaplan-Meier method according to small (z-score, < 5.0), medium (≥ 5.0 to < 10.0), and large (≥ 10.0) CAA using a 5 increment scale scheduled to be included in the new American Heart Association criteria. Cox regression analysis was used to identify risk factors for coronary events. The occurrence rate of major cardiac events such as angina pectoris, myocardial infarction, and cardiac death was also analyzed. Results: Data were analyzed for 1,002 patients from 44 institutions. Both the body surface area and CAA diameters were available in 741 cases for the right coronary artery (RCA) and 609 cases for the left anterior descending artery (LAD). Coronary events occurred in 83 (11.2%) of the RCA group and 57 (9.4%) of the LAD group, while major events occurred in 30 cases (3.0%). The 10-year event-free survival rate for coronary events for small, medium, and large aneurysms was 100, 95.5, and 64.9% in the RCA group, and 100, 94.4, and 63.5% for aneurysms in the LAD group, respectively. The rate of major cardiac events was 98.5, 98.1, and 87.6% for the RCA group, and 100, 97.5, and 86.8% for the LAD group, respectively. Cox regression analyses showed that the z-score of the CAA diameter was an independent risk factor for coronary events for the RCA [large versus medium aneurysm; hazard ratio (HR) 2.8, 95% confidence interval (CI) 1.5 to 5.3, p = 0.002] and the LAD [HR 3.2, 95% CI 1.6 to 6.5, p = 0.015] groups. Conclusions: The severity assessment of CAA using the 5-increment z-score for coronary arterial diameter can predict the time-dependent occurrence of coronary events in patients with KD.

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Naoya Fukushima ◽  
Masaru Miura ◽  
Toru Kobayashi ◽  
Shigeto Fuse ◽  
Tsutomu Saji ◽  
...  

Background: The standard values of normal coronary artery internal diameters in Japanese children have been recently established, making it possible to calculate Z-scores based on body surface area. The aim of this study was to clarify the appropriate cut-off points of coronary artery aneurysm (CAA) Z-scores to predict coronary events such as stenosis, obstruction, and thrombosis in patients with Kawasaki disease (KD). Methods: In this multicenter retrospective study, we investigated height, weight, CAA diameters measured by echocardiography in acute phase KD, and coronary events in CAA patients with KD (age 18 years or younger) who had coronary angiography from 1992 to 2011. Results: Interim analysis was performed on data of the 928 patients recruited from 45 institutions. Body surface area (calculated from height and weight) and CAA diameters were available in 702, 680, and 539 cases of right coronary artery (RCA), left main trunk (LMT), left anterior descending artery (LAD), respectively. Coronary events occurred in 62 RCA cases (8.8%), 8 LMT cases (1.2%), and 45 LAD cases (8.3%) . Areas under the ROC curves to predict coronary events were similar for actual diameter, Z-score, and the ratio of actual diameter to that showing a Z-score of zero in each segment. The cut-off points for the actual diameter, Z-score, and ratio which yielding the highest sensitivity plus specificity were 6.3 mm, 9.6, and 3.9 times for RCA; 7.4 mm, 11.1, and 2.8 times for LMT; and 5.3 mm, 8.9, and 3.5 times for LAD. Conclusions: We identified cut-off Z-scores for CAA diameters useful for coronary events prediction. Attention should be paid to coronary events when the Z-score for CAA diameter is over 10.


2017 ◽  
Vol 4 (2) ◽  
pp. 403
Author(s):  
Satyaki Das ◽  
Swapan Kumar Ray ◽  
Sukanta Bhattacharya ◽  
Kripasindhu Chatterjee ◽  
Pradyut Kumar Mandal ◽  
...  

Background: Congenital coronary anomalies may be isolated, or they may accompany other congenital heart defects, such as Fallot's tetralogy, transposition of the great arteries or pulmonary atresia. The most common cause of acquired abnormalities of the coronary vessels in children is kawasaki disease (KD). The aim of this study was to find the best model to obtain valid and normally distributed Z-scores for coronary artery (CA) diameters in a large, heterogeneous population of healthy children.Methods: Echocardiography was performed on 300 healthy children. Linear regression models were tested with height, weight, body surface area, and aortic valve diameter. The computed Z scores were tested for normal distribution and stability. Results: CA diameter was best predicted using regression with the body surface area and age in month. The weighted least squares method yielded normally distributed and very stable Z-score estimates for 3 principal CAs.Conclusions: This study showed valid methods to estimate Z scores for CA size in children of all ages. Such Z scores are important for risk stratification in patients with Kawasaki disease.


Author(s):  
Takayuki Suzuki ◽  
Nobuyuki Kakimoto ◽  
Tomoya Tsuchihashi ◽  
Tomohiro Suenaga ◽  
Takashi Takeuchi ◽  
...  

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Michael Khoury ◽  
Michael A Portman ◽  
Cedric Manlhiot ◽  
Anne Fournier ◽  
Rejane F Dillenburg ◽  
...  

Background: Statins have been considered as therapy for children with coronary artery aneurysms (CAA) after Kawasaki disease (KD), due to potential beneficial pleiotropic effects which might influence chronic vascular processes and inflammation. Methods: The North American Kawasaki Disease Registry was queried to identify patients who have received statins in the first 6 months following the convalescent phase of KD. Each identified patient was matched by age, gender and CAA z score to 3 patients who were statin-naïve (controls). Linear regression models adjusted for repeated measures and maximum coronary involvement were used to determine an association of statin use with longitudinal changes in coronary artery diameter z-score. Kaplan-Meier analysis was used to compare freedom from angiographically-confirmed stenosis or interventions. Results: Of 29 patients with KD and CAA (maximum coronary artery z-score >10) who received statins at any time (of n=621, 5%), 10 (9 males) patients were started within 6 months of the acute KD episode. The mean age at KD was 6.3±3.4 years (5.4±3.5 for controls, p=0.57). Mean maximum CAA z-score was 36±14 (vs. 29±16, p=0.20); 90% of statin patients and 87% of matched controls had CAAs in 3 or more branches. Linear regression analysis of 442 serial echocardiograms showed that maximum CAA z-score decreased by -1.5 (95%CI: -2.7; -0.4) SD/year (p=0.008) for control patients compared to -2.9 (95%CI: -4.4; -1.4) SD/year (p<0.001) for statin treated patients. The difference between the rate of change of CAA z-score for statin vs. control patients did not reach statistical significance (controls vs. statins: +1.4 SD/year, 95%CI: -0.6; +3.4, p=0.18). n=7 patients (3 on statin, 4 controls) developed stenosis or had revascularization, with no significant difference between groups (HR for statin group: 2.2 (0.4-11.4), p=0.41). Conclusions: This underpowered pilot study suggests that equipoise likely exists with regards to statin therapy in children with KD and CAA, and that a formal registry-nested trial might be considered.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Emily P Williams ◽  
Michael S Kelleman ◽  
William T Mahle

It has been previously reported that African American race may be protective against coronary artery aneurysm development in Kawasaki Disease (KD). We aimed to test this with our own cohort of KD patients from a large pediatric cardiology practice. Data from 250 subjects diagnosed with KD and followed as outpatients with surveillance echocardiography over a two-year period were analyzed. Twelve patients were excluded due to incomplete records or an unconfirmed diagnosis. Race designated by parent was recorded. Charts were reviewed for any coronary involvement (ectasia or aneurysm) and coronary Z-score greater than 2.5 at the time of diagnosis and at subsequent follow-up visits. Odds rations were calculated comparing each racial group to others for any coronary involvement and for coronary Z-score > 2.5. Of 238 included patients, 44.5% were African American, 37.4% were non-Hispanic white, 10.5% were Hispanic, and 7.6% identified with other racial designations. Approximately 21.9% of African American patients had any coronary involvement and 9.5% had a coronary Z-score > 2.5. Approximately 21.4% of non-Hispanic whites had any coronary involvement and 13.5% of non-Hispanic whites had a coronary Z-score > 2.5. Twenty-eight percent of Hispanic patients had any coronary involvement and 12% had a coronary Z-score > 2.5%. Of patients that identified with other racial designations, 38.9% had coronary involvement and 22.2% had a coronary Z-score > 2.5. No statistically significant odds ratios were identified. Relative to reference group (non-Hispanic whites) African American patients had nearly identical rates of 1) any coronary involvement, or 2) coronary Z-score > 2.5. KD occurs commonly in African-American children. Given equal risk for late coronary sequelae vigilance and strict adherence to consensus guidelines is essential.


2020 ◽  
Vol 30 (6) ◽  
pp. 834-839
Author(s):  
Piaoliu Yuan ◽  
Danyan Su ◽  
Krishna D. Mandal ◽  
Suyuan Qin ◽  
Na Zhao ◽  
...  

AbstractBackground:Giant coronary aneurysms are the most severe complications of Kawasaki disease. There are few reports of outcomes from China. Most previous studies were based only on absolute aneurysmal dimensions. The aim of the present study was to catalog the outcomes of Kawasaki disease with giant coronary aneurysms in southwest China based on absolute dimensions and the z-score adjusted for body surface area.Methods and results:All patients diagnosed with giant coronary aneurysms (z-score ≥ 10 or absolute dimension ≥ 8 mm) between December, 2002 and December, 2018 were included. We retrospectively analysed patient characteristics and clinical data from 38 patients with giant coronary aneurysms. Over a median follow-up period of 30.5 months (range from 1.7 months to 22.3 years), including patients in chronic phase who had been diagnosed prior to 2002, eight patients had myocardial infarction, including two deaths and one patient with coronary artery bypass grafting. The 1-, 2-, and 5-year event-free rates were 0.63, 0.63, and 0.53 for thrombosis, respectively, and 0.86, 0.81, and 0.81 for major adverse cardiac events, respectively. The 1-, 2-, and 5-year regression-free rates were 0.94, 0.85, and 0.67, respectively. A total of 73.7% of patients remained active.Conclusion:In the early stages of Kawasaki disease, patients with giant coronary aneurysms often experience major cardiovascular events; however, they are also likely to have normalisation of the coronary internal luminal diameter. With long-term anticoagulation, close cardiologic monitoring, and prompt thrombolytic therapy, most patients can achieve disease-free periods.


2021 ◽  
Vol 9 ◽  
Author(s):  
Jinling Hu ◽  
Weidong Ren

Abstract: Kawasaki disease (KD) is one of the most common forms of systemic vasculitis in children. Pathological features include extensive inflammation of small and medium blood vessels throughout the body. The primary complication of KD is coronary artery lesions (CALs). A total of 640 children with KD were admitted to the Department of Pediatric Cardiology at Shengjing Hospital of China Medical University from January 2017 to December 2019. These patients comprised 52 coronary artery aneurysm (CAA) cases and 47 coronary artery dilation (CAD) cases. Echocardiography was performed during the acute KD phase and then at 1, 3, 6, 12, and 24 months after KD onset. Patients were divided into a poor prognosis group (n = 30) and a normal group (n = 69) based on CALs prognosis. Differences in laboratory data, clinical manifestations and coronary artery damage rates were compared between the two groups. Univariate analysis was performed on these data, and an ROC curve was used to analyze the efficacy of each risk factor. Univariate analysis revealed that age (months), number of coronary arteries involved (NACI), IgM, IgA and brain natriuretic peptide (ProBNP) levels were higher in the poor prognosis group compared with the normal group, procalcitonin (PCT) levels in the poor prognosis group were lower than in the normal group (P &lt; 0.05).Conclusion: Age ≥ 18 months, IgM ≥ 1.07g/L, IgA ≥ 0.728g/L and NCAI ≥ 3 were poor prognostic factors of KD children with CALs. These parameters can be used as a reference indicator of early prediction where combined detection might improve the accuracy and sensitivity of prediction. Follow-up should be maintained to monitor changes in the coronary artery by echocardiography.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Soha Rached-d'Astous ◽  
Ibtissama Boukas ◽  
Anne Fournier ◽  
Marie-Josée Raboisson ◽  
Nagib Dahdah

Background: Coronary artery dilatations are almost always secondary to Kawasaki disease in the pediatric population. The presence of CA involvement is used as a criterion to diagnose incomplete KD disease, which may be challenging. It has been recently demonstrated that febrile patients had larger CA size than non-febrile children in a pilot study. As there is almost always a myocarditis in the acute phase of KD disease we sought to investigate whether viral myocarditis may cause CA dilatations. Method: This retrospective study reviewed 14 consecutive patients with a diagnosis of viral myocarditis at Saint-Justine Hospital, Montreal, from April 2000 trough December 2010. Kawasaki disease was excluded in all patients. All echocardiogram studies were reviewed by an independent experienced technician for CA size and function parameters. Patients were classified in three categories: definite CA dilatation (Z-score ≥ 2.5 in one or more CA), occult CA dilatation (Z score variation ≥ 2 for the same CA on 2 different echogrardiograms, but absolute Z score always < 2.5) and normal coronary artery. Demographics, laboratory values, microbial etiology testing, diagnostic studies were also collected. Results: Mean age at presentation was 1.67 ± 3.22 years, the majority < 2 years old, and 11 (78%) were girls. Of the 14 patients 8 (57.1%) had normal CA, 3 (21.4%) had occult CA dilatation and 3 (21.4%) had definite CA dilatation. When present, CA dilatation was detected within the first 8 days of presentation. Eleven (78%) patients presented with acute onset features and the remaining 3 presented with subacute characteristics. There was no correlation between CA involvement and the intensity of LV dysfunction however (p = 0.84). Conclusion: Patients with viral myocarditis can present CA dilatation during the acute phase of the illness. This finding should be taken into account when diagnosing patients with incomplete KD on the basis of the CA involvement as the two illnesses may present with similar features.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Elif Seda Selamet Tierney ◽  
Andrew S Mackie ◽  
Brian W McCrindle ◽  
Mathew Mathew ◽  
Kathryn R Armstrong ◽  
...  

Background: The pharmacological management of coronary artery aneurysms (CAA) associated with Kawasaki disease (KD) is based on imperfect evidence, which may lead to considerable practice variation. Methods: Pharmacological management of patients included in the North American Kawasaki Disease Registry was reviewed. The Registry included data for 621 patients with CAA after KD (280 patients with maximum CAA z-score between 2.5-5.0, 139 with z-score 5.0-10.0 and 202 with z-score >10.0) followed at 20 medical centers. Practice variation regarding acute treatment, anti-inflammatory agents, statins, beta-blockers, antiplatelet therapy and anticoagulation were assessed. Results: Considerable practice variation existed between centers. During the acute phase, 93% of patients received at least one dose of IVIG (range: 80-100%), with 23% (range: 12-50%) receiving additional immunomodulatory treatment (22% additional IVIG, 17% steroids, 4% infliximab). Use of a 3 rd course of IVIG was infrequent (2%). All centers reported using additional IVIG or steroids for IVIG-resistant patients, but only 6 centres reported any experience with infliximab (2 commonly, 4 infrequent). Routine use of non-steroidal anti-inflammatory agents was limited to 2 centres, with 4 additional sites reporting infrequent use (10% of patients). Statins (5%), beta blockers (4%) and abciximab (3%) were mostly used by a single centre and was limited to patients with giant CAAs. Aspirin was the primary antiplatelet modality for 97% of patients, clopidogrel (10% of all patients, 23% in giant CAA) was routinely prescribed to patients with giant CAAs at 6 centres, with 2 more centres reporting infrequent use and the remainder not reporting any use. For patients with giant CAA (z-score>10.0), 46% were maintained on an antiplatelet agent alone, 17% additionally were on low molecular weight heparin(LMWH), 12% on warfarin and 25% had initially received LMWH and were later switched to warfarin. Conclusions: Given the important variations in management between centres and the poor evidence base, randomized controlled trials examining outcomes and nested in a high-quality collaborative registry may be an efficient strategy to address this gap.


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