Giant coronary artery aneurysm caused by Kawasaki disease observed by coronary angiography

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

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.

Objective To investigate the diagnosis and prognosis of patients with coronary artery aneurysm in Kawasaki disease. Methods The data of ultrasonic diagnosis and follow-up of 338 children with Kawasaki disease complicated with coronary artery aneurysms were analyzed. Results The incidence of coronary artery aneurysm in the acute stage of Kawasaki disease was 21.2%(338/1 594). Of all the 338 cases, small aneurysms was 66.6%(225 cases), medium aneurysms was 25.1%(85 cases), and giant aneurysms was 8.3%(28 cases). There were 719 branches involved in 338 cases, 32.8% of them in left main coronary artery and 31.1% in right coronary artery; 25.3% in left anterior descending branch, and 10.8% in left circumflex branch. A total of 382 branches were followed up, including 218 branches of small coronary aneurysm group and 82.1% of them were completely recovered to the normal diameter. The medium aneurysm group was 124 brunches, the proportion of no significant change, retraction, and normal were 23.4%, 68.5% and 8.1%, respectively. There were 40 brunches in the giant anuerysm group, in which the proportion of no significant change, retraction, and normal were 70.0%, 27.5% and 2.5%, respectively. A total of 30 thrombosiswere detected by echocardiography in the acute stage, 20 thrombosis were regularly followed up, 12 thrombosis gradually subsided, and the other remained persist. Five thrombosis were detected in the sequelae stage, and all located in the giant coronary aneurysm. Seven children were clinically diagnosed with ischemic heart disease, of them, acute myocardial infarction in 1 case, 1 died of heart failure. Conclusions Kawasaki disease coronary artery disease are common in small coronary aneurysms, of which the left main artery and right coronary artery lesions are the most common, and the prognosis is better; medium and giant aneurysm need more time to recovery , and are easy to complicated with thrombosis. Key words: Echocardiography; Mucocutaneous lymph node syndrome; Coronary aneurysm

Objective: To summarize the clinical features, middle-and long-term prognosis of Kawasaki disease (KD) with giant coronary artery aneurysm (GCAA). Methods: In this retrospective cohort study, a cross-sectional analysis was conducted on 101 KD children with GCAA in the KD with GCAA database established by Beijing Children's Hospital, Capital Medical University in 2004. GCAA was diagnosed as coronary artery absolute lumen diameter ≥8.0 mm. All patients were followed up regularly. The endpoint was the time of last follow-up or the death time. T test or χ2 test was used for comparison between groups. Results: A total of 101 KD children with GCAA were enrolled, including 82 males (81.2%) and 19 females (18.8%). The age of disease onset was 2.5 (1.0, 4.5) years. The follow-up duration was 4.5 (2.7, 7.5) years, with a longest of 19 years. All children received routine treatment with aspirin and warfarin, and clopidogrel was added in severe cases. At the end of follow-up, 13 cases (12.9%) had cardiac enlargement, 11 cases (10.9%) developed heart failure, 13 cases (12.9%) experienced myocardial infarction, 2 cases (2.0%) underwent coronary artery bypass graft and 6 cases (5.9%) died. A total of 170 coronary arteries were involved, including 24 (14.1%) GCAAs on the main trunk of left coronary artery, 10 (5.9%) GCAAs on left circumflex, 57 (33.5%) GCAAs on left anterior descending, 78 (45.9%) GCAAs on the middle segments of right coronary artery, and 1 (0.6%) GCAA in the distal segments of right coronary artery. Eleven cases (10.9%) recovered with the coronary artery absolute lumen diameter of all GCAAs below 4.0 mm. Among 170 branches with GCAAs, 28 (16.5%) regressed below 4.0 mm. No significant difference was found in the regression rates between right and left GCAA (18.7% (17/91) vs. 13.9% (11/79), χ²=2.473, P=0.116). There was no statistically significant difference in retraction between unilateral GCAA and bilateral GCAA (16.1% (9/56) vs. 4.4% (2/45), χ2=2.381, P=0.123). Conclusions: GCAA of KD occurred more common in the middle segments of right and left anterior descending coronary arteries. The incidence of adverse cardiac events and the mortality rate in children with GCAA complicated with KD was high. Their long-term prognosis was poor.

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

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

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

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

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 …

Objective To explore the clinical value of Z score in assessing coronary artery lesions (CAL) of children with Kawasaki disease. Methods The clinical records of 102 children with Kawasaki disease from January 2012 to December 2016 in Gansu Provincial Hospital were retrospectively analyzed.The internal diameter of left main coronary artery (LMCA) and right coronary artery (RCA) was measured by echocardiography (ECHO), and the incidence of CAL was preliminarily judged.The Z scores of LMCA and RCA were calculated on the basis of the coronary artery diameter, the age of the children and the body surface area, and the incidence of CAL was judged again. Results A total of 22 cases(21.6%) of CAL were found in 102 cases by ECHO examination, of which 18 cases(17.6%) of LMCA lesions, and 22 cases(21.6%) of RCA lesions.A total of 33 cases(32.4%) of CAL were found by calculating the Z score of coronary artery, of which 29 cases(28.4%) of LMCA lesions and 33 cases(32.4%) of RCA lesions.There was significant difference between two methods for determining LMCA lesions (χ2=3.35, P 0.05). Z score of coronary artery was more accurate to detect the CAL in Kawasaki disease, especially LMCA lesions.A large coronary artery aneurysm was found in the patients with the largest Z score by selective coronary angiography. Conclusion The Z score can be more conductive to assess the CAL in children with Kawasaki disease, and the higher the Z score, the more serious the CAL is. Key words: Kawasaki disease; Coronary artery lesions; Z score; Echocardiography

A 20-year-old woman visited our cardiology clinics for regular follow-up of Kawasaki disease. She was diagnosed with a giant coronary aneurysm at age 4. Her last coronary angiography was obtained 6 years before her current admission. To investigate the status of her coronary aneurysm, contrast-enhanced coronary computed tomographic (CT) angiography was performed with a 16-slice CT scanner. The CT was performed with retrospective …

Background: The Etanercept as Adjunctive Treatment for Acute Kawasaki Disease study, a phase 3 randomized placebo-controlled clinical trial, evaluated etanercept, a TNF-alpha inhibitor, as an adjunct to IVIg for Kawasaki Disease (KD). In children presenting with coronary artery (CA) aneurysm, etanercept resulted in reduction in progression of CA dilation and earlier aneurysm regression compared to placebo. Following study conclusion, our institution implemented etanercept as a first line IVIg adjunctive treatment for patients presenting with early CA aneurysm formation. Hypothesis: Real world use with etanercept as adjunct to IVIg in children with KD and CA aneurysm shows high patient compliance, is safe and results in early CA regression. Methods: We reviewed charts and echocardiograms for all children admitted to Seattle Children’s Hospital from 5/2019 to 4/2024 and treated with etanercept for KD with CA aneurysms at presentation (Z-score ≥2.5 per AHA aneurysm definition). Etanercept is injected subcutaneously at 0.8 mg/kg with IVIg at diagnosis and then with 2 repeat weekly doses at outpatient visits. Results: Thirty-six patients with KD and CA aneurysms received at least one dose of etanercept. Sixteen (44%) were &lt;1 year of age, 17 (47%) were diagnosed with incomplete KD and 5 (14%) were ‘IVIg refractory.’ Four patients responded to a second IVIg dose and two patients received additional biologic agents. Thirty-two patients completed the 3-dose course of etanercept, with outpatient doses administered 6.6±1.5 days (mean±SD) and 13.5±2.3 days after the first. Seven patients had giant CA aneurysms (Z-score ≥10) requiring anticoagulation. In patients with giant aneurysms and at least 6-month follow-up, all aneurysms regressed to below the threshold for requiring anticoagulation beyond aspirin. Of 31 children with long-term follow-up echocardiograms available, 19 had complete resolution (Z-score &lt;2.5) of CA dilation by 6 months, 22 by 1 year, and 29 by 2-year follow-up. No severe or serious adverse events were attributed to etanercept. Conclusions: Etanercept for KD with early CA aneurysms was delivered safely and with high compliance. CA aneurysm regression occurred in most patients with marked reduction in giant CA aneurysms by 6 months. These real-world data support the use of etanercept as an intensification therapy for preventing persistence of CA aneurysms in KD.

Resection of Giant Coronary Artery Aneurysms in a Takayasu's Arteritis Patient

Kawasaki disease(KD) without proper treatment, the incidence of coronary artery lesions is up to 25%.Some of them develope of coronary artery aneurysms(CAA), obstructive coronary artery disease(stenosis, occlusion or thrombosis). KD with CAA in children need long term follow-up.Electrocardiogram(ECG) and two-dimensional echocardiography(2DE) are still the basic means of checking for follow-up.KD with CAA who should be preferred magnetic resonance imaging examination.Dobutamine stress echocardiography or coronary angiography should be done with suggestive of myocardial ischemia in ECG or 2DE. Key words: Kawasaki disease; Coronary artery stenosis; Myocardial ischemia; Diagnosis

Left main coronary artery (LMCA) aneurysms are rare with incidence of 1 in 1000 cases among patients undergoing coronary angiography. Coronary artery aneurysm is defined as coronary arterial segment dilation > 1.5 fold greater than normal adjacent coronary segments. Atherosclerosis is the primary cause of coronary aneurysms but also has been reported in other conditions like arterial vasculitis (e.g., Kawasaki disease, Takayasu arteritis), or infection (e.g., syphilis, mycosis). LMCA aneurysms are especially associated with serious complications including thrombosis, distal embolization, spontaneous rupture or dissection or even sudden cardiac death. The optimal treatment for LMCA aneurysm is still controversial and the available results have been based mainly on case reports and not on controlled trials. The lack of consensus about managing LMCA is not the only challenge here. The lack of individualized treatment or precision medicine in this area makes managing these cases at bedside even more challenging.