Middle and long-term follow-up of 101 children with giant coronary artery aneurysm of Kawasaki disease

Objective To explore whether the warfarin and aspirin combination therapy can prevent cardiovascular events in patients with giant coronary artery aneurysm (GCAA) caused by Kawasaki disease(KD). Methods Children who had been diagnosed as GCAA secondary to KD in Beijing Children's Hospital Affiliated to Capital Medical University between Jan.1998 and Aug.2012 were enrolled in this study.They were divided into the warfarin plus aspirin group (combination group) and aspirin group.The combination group used the therapy of warfarin and small dose aspirin in the long-term anticoagulation treatment, while the aspirin group used small dosage of aspirin without warfarin.Both groups were followed at the time points of 2nd week, 1st month, 3rd month, 6th month, and 1st year after discharge of the acute stage.Then these children were followed every 6 months.Data on each followed-up included clinical manifestations, coronary artery aneurysm recovery situation and complications. Results (1)The onset age of GCAA caused by KD ranged from 3 months to 13 years and 3 months.Infants who were ≤1 year old and children who were ≥5 years old were more susceptible to this disease, their proportion were both 23.1%. (2)The distribution of GCAA in both groups were similar.GCAA most commonly occurred in the right coronary artery, then the left anterior descending coronary artery, and then the main trunk of left coronary artery, the left circumflex artery was rarely affected.(3) Coronary artery aneurysm in 17 cases(53.1%) retracted in the warfarin combined with aspirin group, while 5 cases(41.7%) in the aspirin group.Fifteen cases(46.9%) in the combination group hadn't obvious change, while the aspirin group got 7 cases(58.3%). (4)During the follow-up, 2 children(6.3%)complicated with intracoronary thromboses in the combination group, while 3 cases(25.0%) in the aspirin group.One case(3.1%) in the combination group suffered myocardial infarction, while 3 cases(25.0%)in the asprin group.Two cases (16.7%) in the aspirin group died, while none in the combination group.Coronary artery stenosis occurred in 2 cases (16.7%) in the aspirin group, while 1 case (3.1%) in the combination group.One child had coronary artery occlusion in the aspirin group, while none in the combination group.(5)The combination group had 1 case of serious bleeding event, subarachnoid hemorrhage.In addition, there were 8 cases of nasal bleeding, a total of 19 person-time.There was no serious bleeding event in the aspirin group, only 3 person-time small mount of nasal bleeding. Conclusions Althought warfarin plus aspirin therapy for the long-term anticoagulation treatment in GCAA caused by KD can not affect the retraction of GCAA, it may decrease the incidence of thrombosis, myocardial infarction and mortality.Bleeding complication is more common during the application of wafarin.Therefore the dose of warfarin should be tailored in various children according to the clinical situation, and bleeding complication should be monitored. Key words: Kawasaki disease; Giant coronary artery aneurysm; Warfarin; Anticoagulation therapy; Prognosis

Objective To observe the mid- and long-term changes of giant coronary artery aneurysm(GCAA) caused by Kawasaki disease(KD) and to provide preventive basis for cardiac accidents of KD-affected adolescents. Methods Twelve cases of KD with GCAA [10 boys, 2 girls; 1-10 years old; mean age: (4.05±2.50) years old] were followed up by coronary angiography (CAG), echocardiography and electrocardiogram for 2-10 years to and the changes of the coronary artery lesion were analyzed. Results The findings showed that 2 cases were single coronary artery aneurysm and 10 cases had multiple coronary artery aneurysms on both sides by echocardiography.Among them 8 cases showed segmental stenosis in the right coronary artery with varying degrees by coronary angiography, including 3 cases with coronary recanalization after right coronary artery complete occlusion, 4 cases with segmental local stenosis, and 1 case with thrombotic occlusion and tufted neovascularization.And in 9 cases of coronary artery aneurysms were still present after treatment, and among them the blood flow filling in 4 cases was slowing down in the left anterior descending arteries.Only 1 case with coronary artery aneurysms on both sides became expanded mildly, without stenosis or other lesions.In this observation, all of the cases had no symptoms or any activity limitation.One case was found pathologic Q wave and the other 11 cases left normal by resting electrocardiogram.The results of resting electrocardiogram in 11 cases were normal by pharmacologic stress electrocardiogram except that 1 case showed multi-lead S-T segment depression and T wave flat.Compared with coronary angiography in the same period, 12 cases showed proximal morphological changes in coronary artery and 5 cases with wall motion abnormalities by echocardiography.This study showed that echocardiography was not sensitive enough for mid- and distal-stenosis examination because it failed to provide any useful information for 4 cases affected by segmental stenosis and neovascularization. Conclusions The GCAA caused by KD will be lasting for a long time after acute stage, including persisting GCAA, recovering GCAA, occlusion, stenosis and neovascularization.The sensitivity of echocardiography and electrocardiogram are not enough to find coronary morphology and function of KD sequelae damages.CAG is important for follow-up observations of KD with GCAA. Key words: Kawasaki disease; Giant coronary artery aneurysm; Coronary angiography; Follow-up

Giant Thrombotic Right Coronary Aneurysm in an Infant with Undiagnosed Incomplete Kawasaki Disease and Rapidly Progressive Cardiovascular Collapse

To evaluate the prognosis and clinical features of patients with giant coronary artery aneurysm (GCAA) caused by Kawasaki disease (KD). KD complicated with GCAA was diagnosed in 55 patients between January 2003 and December 2012 in Guangzhou Women and Children's Medical Center.Of the 55 patients, 48 were studied(43 boys, 5 girls). According to the follow-up time, these patients were divided into four groups: ≤ 1 year follow-up group, > 1-3 years follow-up group, > 3-5 years follow-up group, and > 5-10 years follow-up group. These patients were investigated at the acute stage, and were followed up respectively at two weeks, one month, three months, six months, and one year after hospitalization and every 3 to 6 months after a year. All patients regularly accepted echocardiography and electrocardiographic examination, while some cases accepted CT coronary angiography (CTA) examination and coronary angiography (CAG) examination to confirm the condition of GCAA. (1) The age of 48 patients ranged from 2 months to 10 years. Twenty cases were less than 1 age (42%), 30 cases were under 3 years of age (62%) and follow-up time was (4.0 ± 3.1) years. Age at endpoint was 1.5 to 19 years, mean (6.8 ± 4.2) years respectively. (2) In ≤ 1 year follow-up group, the proportions of no significant changes, retraction, and stenosis were 48%, 48% and 4% respectively. In > 1-3 years follow-up group, the proportions of no significant changes, retraction, and stenosis were 39%, 39% and 22% respectively. In > 3-5 years follow-up group, the proportions of no significant changes, retraction, and stenosis were 30%, 35% and 35% respectively. In > 5-10 years follow-up group, the proportions of no significant changes, retraction, stenosis were 30%, 20% and 50% respectively. Compared with the ≤ 1 year group, a significant increase in the proportion of coronary artery stenosis occurred at the other three groups.Significant difference in the stenosis rate could be found between the ≤ 1 year group and the other three groups respectively (χ(2)=6.026, 11.121, 15.652; P=0.019, 0.002, 0.001). (3) The rate of retraction in bilateral GCAA group (20 cases) was lower than unilateral GCAA group (28 cases ); bilateral GCAA group had lower rate than the unilateral group(15% (3/20) vs. 36% (10/28)). There was no significant difference in coronary artery outcome between two groups (P > 0.05) . (4) There were six severe ischemic heart disease in 48 cases including 2 deaths. (5) CTA showed coronary artery wall thickening, mural thrombus and calcification, the CAG could display coronary artery occlusion and recanalization and collateral vessels formation. KD complicated with GCAA may occur in infants under one year of age, especially infants under 6 months of age. A significant increase in the proportion of coronary artery stenosis occurred with the follow-up time extended. The proportion of bilateral GCAA patients who had ischemic heart disease and died was higher than the unilateral cases. The combined application of echocardiography, CTA and CAG may greatly help to discover coronary thrombosis, stenosis and occlusion.

Giant Aneurysm of the Left Atrial Branch of the Left Circumflex Artery With Fistula

Kawasaki disease (KD) is an acute systemic inflammatory illness that occurs predominately in children <5 years of age. The reported incidence varies widely depending on the ethnicity of the population and the method of case ascertainment. Recent reports would suggest the annual incidence is ≈20 to 25 per 100 000 children <5 years of age in North America, with the highest reported incidence of 188 being in Japan, where the disease was first described in 1967.1 The illness is self-limited and of unknown cause, but is complicated by a systemic vasculitis with a predilection for small- to medium-sized arteries, particularly the coronary arteries. The majority of patients will have either transient coronary artery dilation or no coronary artery luminal changes as noted on echocardiography. Long-term prognosis for these patients is considered to be excellent. Coronary artery aneurysms occur in 25% of patients, but the prevalence is reduced to ≈4% for patients treated with intravenous immunoglobulin infusion within 10 days of illness onset. Aneurysms are associated with an intense inflammatory cell infiltrate, destruction of the internal elastic lamina, and smooth muscle cell death. Coronary artery involvement is usually maximal within 6 to 8 weeks after the acute episode. Regression of aneurysms can occur primarily through myointimal proliferation, although the arterial structure and function remains abnormal,2 and there is an important ongoing risk of stenoses and occlusions.3 Long-term cardiology assessment and management is required, and some of these patients may require revascularization procedures or, rarely, cardiac transplantation. KD has become the most prevalent acquired cardiac disease in children in developed countries. Article see p 60 Although coronary artery complications are the predominant cause of morbidity and mortality, other cardiovascular abnormalities can occur. Valvulitis is a less prevalent complication, and there have been case reports of important long-term aortic and …

The coronary arterial lesions of Kawasaki disease are mainly dilative lesions, aneurysms, and stenotic lesions formed before, after, and between aneurysms; these lesions develop in multiple branches resulting in complex coronary hemodynamics. Diagnosis of myocardial ischemia and infarction and evaluation of the culprit coronary arteries and regions is critical to evaluating the treatment and prognosis of patients. This study used hybrid imaging, in which multidetector computed tomographic (CT) images for coronary CT angiography (CCTA) and stress myocardial perfusion single-photon emission CT (SPECT) images were fused. We investigated the diagnosis of blood vessels and regions responsible for myocardial ischemia and infarction in patients with complex coronary arterial lesions; in addition, we evaluated myocardial lesions that developed directly under giant coronary artery aneurysms. The subjects were 17 patients with Kawasaki disease with multiple coronary arterial lesions (median age, 18.0 years; 16 male). Both CCTA using 64-row CT and adenosine-loading myocardial SPECT were performed. Three branches, the right coronary artery (RCA), left anterior descending branch (LAD), and left circumflex branch, were evaluated with the conventional side-by-side interpretation, in which the images were lined up for diagnosis, and hybrid imaging, in which the CCTA and SPECT images were fused with computer processing. In addition, the myocardial lesions directly under giant coronary artery aneurysms were investigated with fusion imaging. Images sufficient for evaluation were acquired in all 17 patients. In the RCA, coronary arterial lesions were detected with CCTA in 16 patients. The evaluations were consistent between the side-by-side and fusion interpretation in 14 patients, and the blood vessel responsible for the myocardial ischemic region was identified in 2 patients. In the left circumflex branch, coronary arterial lesions were confirmed with 3-dimensional CT in 5 patients, and the the culprit coronary arteries for myocardial ischemia/infarction were confirmed with the fusion interpretation but not with the side-by-side interpretation. In the LAD, coronary arterial lesions were present in all patients, and the diagnosis was made with the fusion interpretation in 10 patients. In the LAD, small-range infarct lesions were detected directly under the giant coronary artery aneurysm in 8 patients, but were not confirmed with the side-by-side interpretation. Fusion imaging was capable of accurately evaluating myocardial ischemia/infarction as cardiovascular sequelae of Kawasaki disease and confirming the culprit coronary arteries. In addition, analysis of fusion images confirmed that small-range infarct lesions were concomitantly present directly under giant coronary artery aneurysms in the anterior descending coronary artery.

Objective To investigate the safety and efficacy of Warfarin combined with Aspirin in the treatment of multiple medium and giant coronary artery aneurysms in Kawasaki disease(KD). Methods Clinical and follow-up data of 45 children diagnosed with KD complicated with multiple medium-sized and giant coronary artery aneurysms from April 2014 to December 2018 at Guangzhou Women and Children′s Medical Center were collected.These children were divided into 2 groups.A total of 31 cases received regular oral Warfarin combined with Aspirin called experimental group.There were 14 patients treated with oral Aspirin and Clopidogrel called control group.General information, laboratory examination, electrocardiogram, echocardiography, outcome and bleeding complications of the 2 groups were analyzed retrospectively. Results (1) In experimental group, there were 22 patients found thrombosis under echocardiography.The 10 patients′ thrombosis disappeared, 5 patients′ thrombosis reduced, and 2 patients′ increased after treatment.In control group, there were 5 cases found thrombosis.The 2 cases′ thrombosis reduced and 3 cases′ throm-bosis increased.The number of thrombosis in experimental group was significantly reduced, and the number of new thrombosis was less than that in control group (χ2=6.454, P<0.05). (2) The number of coronary artery aneurysms in experimental group increased slowly than that in control group [12.90%(4/31 cases)vs.14.28%(2/14 cases)]. (3) The number of coronary artery aneurysms in experimental group decreased rapidly than that in control group [23.91%(11/46 cases)vs.10.00%(1/10 cases)]. (4) The number of cases of tumor retraction in experimental group was more than that in control group [74.19%(23/31 cases)vs.42.85%(6/14 cases)]. (5) During the followed-up, there was no abnormality in the blood phosphokinase isozyme and troponin, no abnormality in the electrocardiogram and echocardiogram, no ventricular enlargement and abnormal ventricular wall movement, and the ejection fraction value was within the normal range.No active bleeding and no death occurred in the two groups. Conclusions Warfarin combined with Aspirin is very safe and effective in the treatment of KD coronary tumor, it can reduce thrombosis effectively.Compared with oral Aspirin and Clopidogrel, Warfarin combined with Aspirin can reduce the number of multiple medium-sized and large coronary artery aneurysms and reduce the diameter of coronary artery aneurysms. Key words: Warfarin; Aspirin; Kawasaki disease; Coronary artery aneurysm; Giant coronary artery aneurysm

Objective To analyze the treatment and follow-up of congenital coronary artery fistula (CAF) with giant coronary artery aneurysm (GCAA) in children. Methods The clinical data were analyzed retrospectively in 13 patients who were diagnosed as congenital CAF with GCAA between July 2009 and December 2016 in Guangzhou Women and Children′s Medical Center.There were 8 boys and 5 girls.The median age was 18 months, ranging from 40 days to 12 years old.The body weight ranged from 3.8 kg to 29.0 kg with a median of 8.8 kg.Fistulas originated from right coronary artery accounted in 8 patients, with 5 from left coronary artery.Fistulas drained into right atrium in 3 patients, right ventricular in 8 patients and left ventricular in 2 patients.Single fistula occurred in 12 patients and multiple fistulas in 1 patient.The diameter of coronary artery aneurysm ranged from 8 mm to 16 mm with a median of 9.2 mm. Results One patient had tachypnea and growth retardation without heart murmur.The other 12 patients were asymptomatic with heart murmurs occasionally found in routine physical examination.One patient underwent fistula ligation without cardiopulmonary bypass (CPB). The remaining 12 cases received fistula correction with beating heart CPB.Direct suture was used in 10 patients and autologous pericardial patch in other 2 patients.Two patients were associated with atrial septal defect (ASD) and underwent repair of ASD concurrently.The coronary artery aneurysm remained ori-ginal shape without any intervention during the operation.The mean hospital delay was (11.0±2.5) days.Two patients had decreased ejection fraction as low as 38% within 3 days after the operation, but went up to over 50% in follow-up 1 month later.Transient T wave change occurred in 7 patients, and another 2 patients showed a residual shunt with size of 1 to 2 millimeters through the fistula without further intervention after the surgical closure.All 13 patients had antiplatelet therapy with 12 taking Aspirin and one taking Dipyridamole.The dosage was 3-5 mg/(kg·d) with duration ranging from 3 days to 13 months with a median of 1 month.During the perioperative period and the follow-up period (3 months to 8 years), all patients were asymptomatically alive.Transthoracic echocardiography showed normal cardiac function.Compared with preoperative status, the diameter of dilated coronary arteries was not changed after the operation.There was no formation of thrombus in the coronary arteries.Electrocardiography showed no ST-T changes or arrhythmia or myocardial ischemia. Conclusions GCAA can be combined with congenital CAF in children, so it needs early operation.The evidence-based intervention of coronary artery aneurysm and usage of anticoagulant and antiplatelet therapy in pediatric patients was still lacking, which needs long-term follow-up. Key words: Coronary artery fistula; Child; Giant coronary artery aneurysm; Antiplatelet; Follow-up

Background: Long-term outcomes and life expectancy for children with a previous history of Kawasaki disease (KD), particularly those with coronary artery aneurysms (CAA), remain to be determined. Methods: An inception cohort of all patients with KD assessed at The Hospital for Sick Children in Toronto between 1978 and 2013 was assembled. Patient outcomes were obtained throughout their pediatric and adult clinical follow-up as long as available. Prevalence of outcomes over time was modelled with Kaplan-Meier survival curves. Life tables from Statistics Canada were used to obtain age/gender specific cumulative mortality for the general population. Results: The cohort included 2,623 KD patients, of whom 410 (16%) had coronary artery involvement (215 dilatation, 57 non-giant CAA and 138 giant CAA). Average follow-up for patients with coronary artery involvement was 6.7 years (13.3 years for giant CAA); 57 and 34 patients had at least 15 and 25 years of follow-up, respectively. No patients with coronary artery dilatation or non-giant CAA had revascularization or a myocardial infarct. Freedom from revascularization (14 events) for patients with giant CAA was 90±6%, 87±7% and 80±13% at 10, 20 and 40 years of follow-up. Freedom from myocardial infarct (11 events) was 94±4%, 92±5% and 89±7% at 5, 20 and 40 years. For patients without coronary artery involvement, 3 (0.1%) deaths were recorded, one secondary to complications of macrophage activation syndrome during the acute phase of KD and 2 from cancer. No deaths were noted for patients with coronary artery dilatation or non-giant CAA, although clinical follow-up was more limited. For patients with giant CAA, 3 deaths (2.2%) were noted, 2 related to CAA complications and 1 from non-medical cause. Cumulative mortality for patients with giant CAA was 1.5% at 10 years of age (expected mortality 0.7%, HR: 2.2 (0.3-11.5), p=0.08) and 3.1% at 40 years of age (expected 2.3%, HR: 1.3 (0.4-4.0), p=0.37). Conclusions: Despite risks of myocardial infarction and revascularization, patients with giant CAA had life-expectancy similar to that of the general population up to the fourth decade of life. Additional follow-up will be necessary to determine if these trends continue into later decades.

We report a case of sudden death due to rupture of a giant right coronary artery aneurysm, likely a sequela of remote Kawasaki disease, in a young woman with Down syndrome. The decedent was a 28 year old female with trisomy 21 who was found deceased in the backyard pool. An autopsy disclosed an enlarged heart with 400 ml of clotted blood in the pericardial sac and undiagnosed giant coronary artery aneurysms involving the left anterior descending, left circumflex, and right coronary artery, with rupture of the latter artery. The histologic findings were consistent with remote Kawasaki disease. Giant coronary artery aneurysms are a rare complication of Kawasaki disease, infectious vasculitis, and atherosclerosis, among other causes. While Kawasaki disease is typically considered a disease of children, late-onset complications should be considered in the differential diagnosis of young adults with myocardial infarction, coronary aneurysm, or sudden death.

&lt;p&gt;Coronary artery aneurysm (CAA) is a rare entity, defined as localized dilation that exceeds the normal vessel diameter by a factor of 1.5. A giant CAA is described as a very large dilation, when diameter exceeds 20 mm. CAA has a preva-lence of 0.02% [Markis 1976]. Different factors may lead to CAA formation, including Kawasaki disease, atherosclerosis, congenital malformations, autoimmune and infectious disor-ders, and percutaneous interventions [Hartnell 1985]. Man-agement of these patients remains controversial due to a lack of data from large series studies.&lt;/p&gt;&lt;p&gt;We reported a case of a young female patient, who pre-sented with an acute inferior infarction and was diagnosed with a giant right coronary artery (RCA) aneurysm. She underwent aneurysmectomy and revascularization on a beat-ing heart through a right lateral thoracotomy. Due to the minimally invasive nature of this procedure, the patient was able to recover quickly without substantial cosmetic changes.&lt;/p&gt;

Giant coronary artery aneurysm (CAA) is a rare disorder, defined as coronary artery dilatation, in which the diameter of the coronary artery exceeds more than 1.5 times of its normal size. The most common cause of CAA is coronary atherosclerosis for adults and Kawasaki disease (KD) for children and adolescents (especially for the giant CAA that occurred in adolescence). CAA complications include thrombus, acute myocardial infarction (AMI), vasospasm, rupture, ischemia, heart failure, and arrhythmia. So, antithrombotic therapy is crucial for patients with giant CAA.Although giant CAA has been reported in some cases before, few of these cases described antithrombotic therapy particularly, let alone informed direct oral anticoagulant (DOAC) use in these patients. Here, we report a case of a young patient with acute coronary artery disease caused by huge CAA. Rivaroxaban combined with clopidogrel was used for his antithrombotic therapy. Moreover, we reviewed the existing reports to provide an overview of antithrombotic treatment in patients with giant CAA.

BackgroundGiant coronary artery aneurysms are rare conditions with potentially devastating consequences. We report a case of the largest documented giant right coronary artery (RCA) aneurysm to date.Case presentationA 57-year-old male patient visited our outpatient clinic for abdominal pain and exertional dyspnea. Computed tomography identified a 10.2 × 9.8 cm RCA aneurysm with RCA pressure waves were similar to aortic pressures. After discussion by the cardiac team, elective resection with ligation of the proximal and distal ends of the RCA was performed due to the presence of adequate collaterals.ConclusionWe highlight the challenges related to the management of patients presenting with giant coronary artery aneurysms. Optimal management strategies and outcomes for such rare cardiovascular conditions implies the need for standardised management guidelines.

Giant coronary artery aneurysms and myocardial fibrosis after Kawasaki disease may lead to devastating cardiovascular outcomes. We characterised the vascular and myocardial outcomes in five selected Kawasaki disease patients with a history of giant coronary artery aneurysms that completely regressed. Five patients were selected who had giant coronary artery aneurysm in early childhood that regressed when studied 12-33 years after Kawasaki disease onset. Coronary arteries were imaged by coronary CT angiography, and coronary artery calcium volume scores were determined. We used endocardial strain measurements from CT imaging to assess myocardial regional wall function. Calprotectin and galectin-3 (gal-3) as biomarkers of inflammation and myocardial fibrosis were measured by enzyme-linked immunosorbent assay. The five selected patients with regressed giant coronary artery aneurysms had calcium scores of zero, normal levels of calprotectin and gal-3, and normal appearance of the coronary arteries by coronary computed tomography angiography. CT strain demonstrated normal peak systolic and diastolic strain patterns in four of five patients. In one patient with a myocardial infarction at the time of Kawasaki disease diagnosis at the age of 10 months, CT strain showed altered global longitudinal strain, reduced segmental peak strain, and reduced diastolic relaxation patterns in multiple left ventricle segments. These patients illustrate that regression of giant aneurysms after Kawasaki disease is possible with no detectable calcium, normal biomarkers of inflammation and fibrosis, and normal myocardial function. Individuals with regressed giant coronary artery aneurysm still require longitudinal surveillance to assess the durability of this favourable outcome.