History Of Kawasaki Disease Research Articles

Sudden Death in Patients with a History of Kawasaki Disease under School Supervision.

We investigated the incidence of sudden death in students with a history of Kawasaki disease (KD) while under school supervision. Reports of sudden death in students with a history of KD during 1990–1999 and 2000–2009 were retrieved from the mutual aid system data. The student’s grade, sex, circumstances at the time of sudden death, final diagnosis, recommended restrictions on school activities, and intensity of physical activity at the time of sudden death were investigated. There were 11 cases from 1990 to 1999 and 3 from 2000 to 2009; KD was complicated with coronary artery aneurysm (CAA) in nine and one cases, respectively. The incidence of sudden death decreased by approximately 50% for KD history and 80% for KD with CAA between the two decades; however, the difference was not statistically significant. Of the 14 cases, 12 occurred during moderate-to-strenuous exercise; the restriction on exercise for students with KD complicated with CAA was not followed in at least five cases during 1990–1999, while three cases during 2000–2009 occurred without recommended restriction. Cases of sudden cardiac death decreased during 2000–2009, compared with those during 1990–1999. Special attention is required for students with a history of KD, particularly when complicated with CAA.

Tolerability of COVID-19 Infection and Messenger RNA Vaccination Among Patients With a History of Kawasaki Disease

Kawasaki disease (KD) symptoms significantly overlap with multisystem inflammatory syndrome in children due to COVID-19. Patients with KD may be at risk for adverse outcomes from exposure to SARS-CoV-2 infection or vaccination. To describe the outcomes of patients with KD to SARS-CoV-2 infection or vaccination. This case series evaluated 2 cohorts using an existing KD database and reviewed individual electronic medical records for the period spanning January 1, 2020, through January 31, 2022, via electronic medical records that include Washington state immunization records. Vaccine cohort inclusion criteria consisted of being 21 years or younger at immunization and receiving 1 or more BNT162b2 (Pfizer-BioNTech) or messenger RNA (mRNA)-1273 (Moderna) vaccine doses. The COVID-19 cohort included patients 21 years or younger with positive polymerase chain reaction or nuclear capsid IgG findings for SARS-CoV-2. Participants included 826 patients from a preexisting KD database. One hundred fifty-three patients received at least 1 BNT162b2 or mRNA-1273 vaccine dose and were included in the mRNA vaccine cohort. Thirty-seven patients had positive test results for SARS-CoV-2 and were included in the COVID-19 cohort. SARS-CoV-2 vaccination and/or infection. Adverse events after mRNA vaccination and/or COVID-19, including clinician visits, emergency department encounters, or hospitalizations. Among the 153 patients included in the mRNA vaccination cohort (mean [SD] age, 13.0 [4.3] years; 94 male [61.4%]), the BNT162b2 vaccine was provided for 143 (93.5%), and the remaining 10 (6.5%) received mRNA-1273 or a combination of both. Among patients in the vaccine cohort, 129 (84.3%) were fully vaccinated or received a third-dose booster. No clinically severe adverse events occurred, and there were no reports of vaccine-related hospitalizations or outpatient visits. The COVID-19 cohort included 37 patients (mean [SD] age, 11.0 [5.5] years; 22 male [59.5%]). No patients required hospitalization due to COVID-19. The most common symptoms included low-grade fever, fatigue, cough, and myalgia with resolution within a few days. Two patients, aged 9 and 19 years, had extended cough and fatigue for 3 to 4 weeks. One patient developed COVID-19 within 6 weeks of receiving intravenous immunoglobulin for KD. These findings suggest that the mRNA vaccines may be safe and COVID-19 may not be severe for patients with a history of KD.

Practical Workflow for Cardiovascular Assessment and Follow-Up in Kawasaki Disease Based on Expert Opinion.

BackgroundApproximately 25% of the patients with a history of Kawasaki disease (KD) develop coronary artery pathology if left untreated, with coronary artery aneurysms (CAA) as an early hallmark. Depending on the severity of CAAs, these patients are at risk of myocardial ischemia, infarction and sudden death. In order to reduce cardiac complications it is crucial to accurately identify patients with coronary artery pathology by an integrated cardiovascular program, tailored to the severity of the existing coronary artery pathology.MethodsThe development of this practical workflow for the cardiovascular assessment of KD patients involve expert opinions of pediatric cardiologists, infectious disease specialists and radiology experts with clinical experience in a tertiary KD reference center of more than 1000 KD patients. Literature was analyzed and an overview of the currently most used guidelines is given.ConclusionsWe present a patient-specific step-by-step, integrated cardiovascular follow-up approach based on expert opinion of a multidisciplinary panel with expertise in KD.

Giant Coronary Aneurysms After Pediatric Heart Transplant: Giant Problem

<h3>Introduction</h3> Cardiac allograft vasculopathy (CAV) is a leading cause of death and retransplant after heart transplantation (HTx). Coronary aneurysms are not typically seen in CAV. We present a case of atypical CAV with coronary aneurysms resulting in premature death. <h3>Case Report</h3> A 4-year-old male underwent HTx after failed single ventricle palliation. His first year after HTx was uneventful with no cellular rejection score greater than 1R and no antibody mediated rejection on multiple surveillance endomyocardial biopsies. Coronary angiography was normal at 3 months. At 1-year post-HTx, surveillance coronary angiography demonstrated a diffusely dilated appearance with multiple aneurysmal segments of the right and left coronary systems (Figure). No filling defects or areas of focal stenosis were appreciated. His pulmonary capillary wedge pressure was elevated at 15 mmHg, but stable from previous assessments. There was no known history of Kawasaki disease in the donor. The patient had no history of prolonged fevers or other symptoms of vasculitis. He was placed on enoxaparin and aspirin for anticoagulation. He remained asymptomatic with no regional wall abnormalities and normal systolic function by echocardiogram. He denied episodes of palpitations and had a Holter without any ectopy or arrhythmias. While awaiting further evaluation with additional advanced imaging, he had a sudden cardiac arrest and died 10 months after coronary aneurysms were found. His autopsy revealed segmental fusiform aneurysmal expansion with severe concentric intimal fibrosis and luminal narrowing of multiple segments of the right and left coronary systems consistent with severe CAV. Left ventricular histopathology exhibited chronic ischemic changes to the subendocardium. <h3>Summary</h3> Our patient with severe CAV presented with coronary aneurysm formation within 1 year of HTx. Coronary aneurysm formation may be an atypical and more severe form of CAV that should prompt close monitoring and early consideration for retransplant.

Heart Transplant, Kawasaki Disease, and Bone Marrow Transplant: Are There Consequences?

This article reviews the current landscape of cardiovascular disease (CVD) risk factors, focusing on dyslipidemia, which contribute to atherosclerosis in three unique populations: youth less than 18years-of-age with a history of Kawasaki disease, and those who have undergone orthotopic heart and bone marrow transplants. Atherosclerosis, the major cause of CVD, begins in childhood. Acquired and genetic disorders of lipid and lipoprotein metabolism, present at an early age, are major contributors to early precursors of atherosclerosis, which accelerate after age 20. Treatment of the underlying medical condition and optimum management of all risk factors is critical in improving outcomes. Nonetheless, limited data is available to assist clinical decision-making, with the aim of improving outcomes. Atherosclerosis, beginning in childhood, is multifactorial in origin with complex interplay of inflammation, infection, endothelial dysfunction, and dyslipidemia. Future studies are needed to help elucidate the specific roles of disease mechanisms, with an emphasis on early intervention and prediction of subclinical disease. In addition to a heart healthy lifestyle, there may be a role for use of lipid-lowering medications beginning at an early age.

Follow up of Kawasaki disease based on nationwide survey data in Japan: Mortality among those with a history of Kawasaki disease in Japan: Results of a 30-year follow up: Mortality among those with a history of Kawasaki disease in Japan: Results of a 30-year follow up.

The long-term prognosis of those with a history of Kawasaki disease (KD) is still unknown. Using a permanent registry system in Japan (koseki), 6,576 persons with a history of KD were followed up. The average follow-up period was 30 years. The endpoint was death. With a 99.5% follow-up rate, 68 deaths (48 males and 20 females) were observed. The overall standardized mortality ratio, of which reference was vital statistics in Japan, was not elevated. However, the observation according to the presence or absence of cardiac sequelae showed that the standardized mortality ratio for those with cardiac sequelae significantly elevated. Nine persons, all of whom were males, died of KD (including those cases where KD was suspected), but all deaths occurred in individuals who were under 30 years of age. This study revealed the long-term prognosis for KD, but almost all participants were younger than 40 years. Continuing follow up of this cohort is required to clarify whether a history of KD relates to the development of atherosclerosis when participants become middle aged or older.

Acute Myocardial Infarction Caused by Thrombotic Occlusion by a Right Coronary Giant Aneurysm in a 6-Year-Old Boy with No History of Kawasaki Disease

Acute Myocardial Infarction Caused by Thrombotic Occlusion by a Right Coronary Giant Aneurysm in a 6-Year-Old Boy with No History of Kawasaki Disease

Characteristics of coronary flow velocity reserve on transthoracic Doppler echocardiography in patients with a history of Kawasaki disease.

The coronary flow velocity reserve of the left anterior descending artery was examined by transthoracic Doppler echocardiography in 189 patients (male 125, female 64) with a history of Kawasaki disease and 10 volunteers (control). Their ages ranged from 6 to 40 years (median 22 years). The patients were divided into nine groups based on the left anterior descending artery lesions. The coronary flow velocity reserve was measured by intravenous administration of adenosine triphosphate (0.15 mg/kg/minute) while fasting. The coronary flow velocity reserve was calculated as the ratio of hyperaemic to basal mean diastolic flow velocities. The respective groups were as follows: control, no coronary artery lesions (n = 39), no coronary artery lesions in the right coronary artery (n = 29), regression (n = 11), aneurysm at the bifurcation of the left coronary artery (n = 26), aneurysm of the left anterior descending artery (n = 15), localised stenosis <75% (n = 12), localised stenosis ≥75% (n = 17), segmental stenosis (n = 5) and coronary artery bypass grafting (n = 36). One-factor ANOVA followed by Tukey's test was used to compare the coronary flow velocity reserve among the groups. The coronary flow velocity reserve was significantly lower in the localised stenosis ≥75%, segmental stenosis and coronary artery grafting groups than in the other groups (p < 0.05). The endothelial function in the epicoronary artery was preserved in patients with a history of Kawasaki disease and dilated coronary artery lesions.

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.

Multiple Giant Coronary Artery Aneurysms in Kawasaki Disease

A 35-year-old man with a history of Kawasaki disease and coronary artery aneurysms (CAAs) at the age of 1 year, for which a coronary artery bypass graft surgical procedure (saphenous vein graft to the left anterior descending [LAD] artery because of the occluded left internal thoracic artery) was performed, presented with dyspnea on exertion. On arrival at our institution, his vital signs were stable, but electrocardiography revealed T-wave inversions in leads V1 through V3. Chest radiography revealed an abnormality of the contour of the left cardiac border (Supplemental Figure 1, available online at http://www.mayoclinicproceedings.org), and chest computed tomography revealed bulky calcified masses on the left ventricle (Supplemental Figure 2, available online at http://www.mayoclinicproceedings.org).

Coronary artery aneurysm complicated by pneumothorax with a history of Kawasaki disease

Kawasaki disease (KD) is a common vasculitis disorder of childhood. It can sometimes complicate coronary artery aneurysms, and treatment is required depending on the condition of stenosis. A 20-year-old man was referred for surgery with a coronary artery aneurysm and stenosis in the left coronary artery as sequelae of KD. He had a surgical history of left pneumothorax and bullae remaining on the right lung. We simultaneously performed off-pump coronary artery bypass for coronary artery stenosis and bullectomy. Coronary artery aneurysms with KD complicated by pneumothorax are rare, and we treated them using one-stage surgery.

Myocardial fibrosis in patients with a history of Kawasaki disease.

ObjectivesCardiac magnetic resonance (CMR) measurements of myocardial extracellular volume fraction (ECV) and late gadolinium enhancement (LGE) in patients with a history of Kawasaki disease (KD) were analyzed to determine whether fibrosis was increased compared to controls.MethodsIn this single center retrospective study, patients with KD who had a CMR with ECV measurement and LGE assessment were included. The ECV was calculated in the mid-left ventricle by measuring T1 values for blood pool and myocardium before and after gadolinium administration with a Look-Locker technique. CMR findings were compared to 20 control subjects.ResultsKD patients (n = 13) had a median age at CMR of 14.9 years (range, 7.5–36.0). Control subjects (n = 20) had a median age at CMR of 16 years (range, 11.0–36.0). Twelve KD patients had coronary aneurysms. The KD patients had a significantly lower indexed LV mass (p = 0.03) and LV mass/volume ratio (p = 0.01). ECV was not significantly different in KD patients and controls (0.26 (range, 0.20–0.30) vs. 0.25 (range, 0.18–0.28), p = 0.28). One KD patient (8%) had an increased (>0.28) ECV. LGE indicating focal fibrosis was found in 5 of 13 (38%) of KD patients. Patients with LGE tended to have a higher maximum coronary dimension z-score (p = 0.09).ConclusionsIn this study of KD patients, most of whom had aneurysms, ECV did not differ significantly from that in normal controls. Focal fibrosis based on LGE was common. Future larger studies should compare ECV in KD patients with and without aneurysms to define the risk of myocardial fibrosis after KD.

Abstract 13323: Identifying the Culprit Lesion in a Symptomatic Patient With Physiologically Corrected Transposition of the Great Arteries

Introduction: Physiologically corrected transposition of the great arteries [(S,L,L) TGA] is a rare congenital heart disease characterized by atrioventricular and ventriculoarterial discordance. Case Presentation: A 28-year-old man with (S,L,L) TGA, pulmonary valve (PV) stenosis, ventricular septal defect (VSD), and history of Kawasaki disease who presented for a gradual decline in exercise tolerance. Oxygen saturation was 96% at rest and dropped to 86% with ambulation. Hemoglobin was 17.7 g/dl, EKG showed no bradyarrhythmias. On stress test, peak heart rate was 159 beats/minute, and peak VO 2 22.6 ml/kg/min (83%, and 57% of predicted value respectively). Spirometry was normal. Transthoracic echocardiogram revealed biventricular hypertrophy with normal systolic function, a bicuspid PV with severe stenosis (peak and mean gradient of 155 and 90 mmHg respectively), large inlet VSD with bidirectional flow and mild systemic TV regurgitation. Coronary CTA showed no coronary artery aneurysms. Cardiac catheterization suggested hypoxia due to pulmonary-to-systemic shunting across the VSD with Q p /Q s = 0.86 (Figure). Consideration should be given to performing a high-risk surgical repair versus medical management. Conclusions: Natural history, symptoms, and timing of intervention are determined by the associated cardiac anomalies and the progressive dysfunction of the systemic right ventricle (SRV). Anatomic repair to restore the left ventricle as a systemic pump is a very high-risk procedure in adults. Functional repair maintains SRV, repair the VSD, and PV stenosis has a poor late outcome. Medical management ensures iron stores are repleted, provide empirical use of pharmacological heart failure therapy, and referral to heart transplantation when SRV systolic dysfunction ensues. Clinicians need to know the potential complications in both unoperated patients and following various surgical repairs to recommend appropriate treatment options.

Stress perfusion cardiac magnetic resonance imaging to identify coronary aneurysms, ischaemia and fibrosis in adult patients with convalescent Kawasaki disease

Abstract Background Kawasaki disease is a common cause of coronary aneurysms in early adulthood. Non-invasive imaging plays a crucial role in the diagnosis and surveillance of these patients who are known to develop ischaemia, premature coronary artery disease and have poorer long-term health outcomes. Stress perfusion cardiac MRI (CMR) is considered the “gold standard” for the assessment of ischaemia and risk stratification of major adverse cardiovascular events in patients with coronary artery disease. Its use in the long-term follow-up of patients with Kawasaki disease is particularly attractive as it avoids the use of ionising radiation. However, data on its utility, together with magnetic resonance angiography (MRA) and late gadolinium enhancement (LGE) to identify ischaemia, coronary aneurysms, and fibrosis, over long-term follow-up in this population remains limited. Purpose To evaluate the diagnostic utility of comprehensive non-invasive stress perfusion CMR to identify inducible ischaemia, coronary aneurysms and myocardial fibrosis in adult patients with convalescent Kawasaki disease. Methods We performed a retrospective review of all adult patients in our Kawasaki service who underwent stress perfusion CMR with MRA and LGE as part of routine clinical care. Data collected included the presence of inducible perfusion defects, coronary anatomy and LGE. Data was compared to contemporary CT coronary angiography (CTCA). In patients who had repeated stress perfusion CMR, changes in perfusion over time were investigated. Results Seventeen patients underwent stress perfusion CMR (total 30 scans). Seven patients underwent ≥2 scans. Median age was 21 years; 14 patients were male. Five patients (30%) had areas of inducible hypoperfusion of which 3 also had LGE. A further 2 patients had LGE with no ischaemia. Coronary MRA was performed in 12 patients (71%) and identified 14 aneurysms. In 8 patients with both MRA and CTCA, detection of aneurysms was significantly correlated (Pearson's coefficient 0.776; P value 0.024). Of the 5 patients with ischaemia, 4 patients had previous CABG. All areas of ischaemia corresponded to persistent calcified aneurysms in the responsible artery. In the 7 patients with multiple perfusion CMR scans (median follow-up 47 months), 3 patients developed new ischaemia. Conclusions Long-term surveillance of coronary arteries in adult patients with a childhood history of Kawasaki disease is crucial in the early identification and management of complications. We show that stress perfusion CMR is a useful radiation-free technique for the long-term follow-up of these young patients and is able to identify the development of new ischaemia. Coronary MRA has good agreement in the identification of aneurysms when compared to CTCA. Little is reported in the literature regarding the use of a comprehensive CMR assessment in the follow-up of adult patients with Kawasaki disease and further studies are needed to validate this approach. Funding Acknowledgement Type of funding source: None

Cardiac events in Patients in their forties with Kawasaki disease and regression of coronary artery aneurysms.

Over a 50-year period from the first description of Kawasaki disease, we encountered three male patients with a history of Kawasaki disease, who had their first cardiac events in their forties. They were considered to have almost normal coronary arteries in the coronary angiograms when they were children and adolescents. They had no follow-up examinations after 20 years old. The 1st patient had an acute myocardial infarction, and the 2nd was a new appearance of coronary aneurysm and stenotic lesions with coronary artery calcification. The 3rd patient had unexpected sudden death. The interval from the onset of Kawasaki disease to the cardiac events ranged from 37 to 38 years. In the former two patients, coronary artery lesions could not be evaluated immediately after Kawasaki disease. Although the 3rd patient had bilateral medium-sized coronary artery aneurysms, his coronary aneurysms regressed 1 year after acute Kawasaki disease. The intimal thickening at a previous coronary aneurysm at the age of 19 was mild. The patients with regressed coronary aneurysms were asymptomatic for about 40 years after Kawasaki disease, prior to their cardiac events. Coronary artery calcification of the proximal portion of the major coronary arteries was a predictable marker in such patients. To prevent serious cardiac events in middle-aged adult patients, reevaluation of coronary artery lesions and restarting of anti-thrombotic therapy are needed. We must be aware that there are some differences in the clinical course and time of cardiac events between patients with giant aneurysms and those with medium aneurysms.

Assessing left ventricular systolic function in children with a history of Kawasaki disease

BackgroundThe incidence of Kawasaki disease (KD) is increasing. Indeed, KD has become the most common cause of acquired heart disease in children. Previous studies have well summarized the acute phase left ventricular (LV) systolic dysfunction using speckle tracking echocardiography (STE); however, changes in LV systolic function after long-term follow-up remain unclear.MethodsOne hundred children with a history of KD, but without coronary artery aneurysms, were enrolled. These children were divided into two subgroups based on the presence or absence of coronary artery dilatation (CAD). The duration of follow-up was > 7 years. The control group consisted of 51 healthy children. The LV myocardial strain were measured by two- and three-dimensional STE.ResultsTwo-dimensional STE not only revealed that LV longitudinal strain decreased in part of segments in both KD groups, but also showed that global strain decreased in the KD group with CAD compared to the controls (P < 0.05). Global longitudinal strain (GLS), global circumferential strain (GCS), global radial strain (GRS), and global area strain (GAS) were obtained by 3D STE. Compared to the controls, GLS and GAS decreased in both KD groups (P < 0.05). GCS and GRS decreased in the KD group with CAD, but was unchanged in the KD group without CAD (P < 0.05).ConclusionsLV systolic dysfunction in children with KD and CAD was more severe than KD children without CAD compared to healthy children. This dysfunction can be assessed by LV regional and global myocardial strain using two- and three-dimensional STE.

Recurrent Kawasaki disease and cardiac complications: nationwide surveys in Japan

IntroductionBased on data obtained before high-dose (2 g/kg) intravenous immunoglobulin (IVIG) therapy prevailed in Japan, children with a history of Kawasaki disease (KD) were highly susceptible to disease recurrence and...

Coronary Artery Aneurysms: Analysis of Comorbidities from the National Inpatient Sample.

IntroductionCoronary artery aneurysms (CAA) are not commonly seen in the general population, with an incidence of approximately 0.37% to 2.53%. Patients are typically asymptomatic but symptomatic presentation varies from dyspnea and angina to myocardial infarction or even sudden cardiac death.MethodsWe conducted a retrospective analysis using the National Inpatient Sample Healthcare Cost and Utilization Project (NIS-HCUP) database to query individuals with the diagnosis of CAA with the International Classification of Disease (ICD) code 414.11 in all discharge diagnoses for the years 2006-2014. History of Kawasaki disease was determined by ICD code 446.1.ResultsFrom 2006 to 2014, there were 23,033 patients identified with CAA, correlating to approximately one case per 10,000 patients or an incidence of close to 0.01%. Of this, 1,405 or approximately 6.1% of these patients had Kawasaki disease. The mortality rate of CAA was 1.79%. In terms of demographics, Caucasians were the most likely to develop CAA, with 73.8% of cases. The mean age was 61.2 years, with a mean length of stay of 5.1 days. The average cost of admission was $70892. The presence of perivascular disease (15.5% vs 4.5% p<0.05), hypertension (66.1% vs 39.1% p<0.05), chronic lung disease (20.2% vs 15.1% p<0.05), diabetes (21.7% vs 15% p<0.05), renal failure (11% vs 8.8% p<0.05), coagulopathy (6.2% vs 3.4% p<0.05), and obesity (13.1% vs 8.2% p<0.05) were all risk factors for CAA as compared to those without. It was noted that weight loss (3.28% vs 1.91% p<0.05), electrolyte abnormalities (18.2% vs 15.5% p<0.05), and blood loss (2.1% vs 0.9% p<0.05) were protective of CAA.ConclusionCAA risk factors appear to be similar to those of coronary artery disease risk factors, with hypertension, diabetes, perivascular disease, and renal failure. Additionally, obesity was noted to be a risk factor but weight loss appeared to be protective. Interestingly, Kawasaki disease was seen at almost similar rates as these cardiac risk factors. The incidence of CAA we found, of almost 0.01%, is much less than in the quoted literature, however, previous studies did not have as many cases as our study.

The radial augmentation index in children with Kawasaki disease without acute coronary artery lesions during the convalescent period.

PurposeWe recently reported that children and adolescents with a history of Kawasaki disease (KD) had slight but significant elastic arterial stiffness even when no coronary artery lesions (CALs) were present. Moreover, we hypothesized that KD-related arteriopathy may also cause peripheral artery dysfunction. The objective of this study was to assess the involvement of radial artery pulse waves, especially reflection waves from the peripheral arteries using the radial artery augmentation index (rAI) in patients without CALs after KD.Materials and methodsWe first collected the rAI data from 312 subjects (149 consecutive patients of KD and 163 control subjects). Next, 225 cases between 6 and 15 years old were selected. Finally, 41 pairs were included for analysis. The rAI values of these two groups were compared and analyzed. Acute-phase data were also collected to reveal the possible correlation with rAI in the convalescent period.ResultsMultivariable analysis revealed the history of KD was positively correlated with rAI@75 value in children from 6 to 15 years old. After pairing the height and gender, the KD group also had significantly higher rAI and rAI@75 than the control group (rAI 60.63±13.77 vs 54.56±13.17, p=0.028; rAI@75 63.61±15.21 vs 55.68±14.86, p=0.003). With regard to acute-phase condition, nonresponse to initial treatment was also linked to elevated rAI during the convalescent period.ConclusionsDuring the convalescent period, the rAI increased in KD patients without acute CALs. Furthermore, nonresponse to initial treatment in acute phase conferred higher rAI to KD subjects than respondent cases. Elevated rAI means the reflection wave from the peripheral vascular is stronger or earlier. This small but significant change may indicate the existence of peripheral artery stiffness during the convalescent period.

Kawasaki Disease Outcomes and Response to Therapy in a Multiethnic Community: A 10-Year Experience

To describe the epidemiology, response to therapy, and outcomes of Kawasaki disease in a multiethnic community with a large Hispanic and Asian population. We analyzed prospectively collected data from 788 unselected patients with Kawasaki disease diagnosed and treated at a single medical center over a 10-year period. The average incidence of Kawasaki disease in children <5 years in San Diego County over the 10 years from 2006 to 2015 was 25 per 100 000 children, with the greatest incidence (50 per 100 000) for Asian/Pacific Islanders. Compared with other race/ethnicities, Asian/Pacific Islander patients with Kawasaki disease were younger, were diagnosed earlier in the course of their fever, had higher levels of inflammatory markers, and were more likely to develop aneurysms. There was no difference across race/ethnicity groups in response to intravenous immunoglobulin therapy. Filipino children had the highest recurrence rates (9.1%; 95% CI, 3.0%-22.6%) and 12 of 788 patients (1.5%) had a first- or second-degree relative with a history of Kawasaki disease. After correcting for age of onset, sex, and illness day at diagnosis, Asian/Pacific Islander children had an increased risk of developing aneurysms (aOR, 2.37; 95% CI, 1.37-4.11; P = .002). Overall, 180 of 788 patients (22.8%) had a maximal Z score of 2.5-10.0 and 14 of the 788 patients (1.8%) had a maximal Z score ≥10.0 despite 84% of these patients being treated within 10 days of fever onset. Our data provide new insights into the natural history of treated Kawasaki disease in a multiethnic population. Patient race/ethnicity influenced susceptibility to Kawasaki disease, timing of diagnosis, coronary artery outcome, and recurrence rates.