Surgical Treatment of Giant Coronary Artery Aneurysm Secondary to Kawasaki Disease

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

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 …

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

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.

Patient: Male, 64-year-oldFinal Diagnosis: Huge coronary aneurysmSymptoms: Chest discomfort • dyspneaMedication: Losar 25 mg twice daily • Amlodipine 5 mg twice daily • Aspirin 80 mg daily • Atorovastatin 20 mg daily • Pantaprazole 20 mg dailyClinical Procedure: Coronary artery bypass graft surgerySpecialty: Cardiac Surgery • CardiologyObjective:Rare diseaseBackground:Giant coronary artery aneurysm (GCAA) is a rare disease, with an incidence of 0.02% in the general population. GCAA is defined as when the diameter of the coronary artery is more than 4 times the adjacent part or more than 8 mm. There are several causes of GCAA, with atherosclerosis being the most common. Patients with giant coronary artery aneurysms can be asymptomatic or develop chest pain, dyspnea, and palpitations. Complications of GCCA include myocardial infarction, thrombosis, and sudden death, so early treatment is necessary to prevent mortality. There is no standard surgical approach for a giant coronary artery aneurysm.Case Report:A 64-year-old man with hypertension, opium addiction, morbid obesity (body weight 151 kg and BMI 46), and benign prostate hyperplasia presented with a giant coronary aneurysm in coronary angiography. The patient underwent cardiac surgery, and a 42-mm coronary aneurysm was detected. The aneurysm had many orifices that opened to the left main coronary artery, left circumflex artery, LAD, the diagonal branch of the LAD, and the septal branch of the LAD. Aneurysmectomy and coronary artery bypass graft were successfully performed.Conclusions:Giant coronary artery aneurysms are rare. Patients with giant coronary artery aneurysms may experience sudden death due to myocardial infarction and other cardiovascular complications due to ischemia. Because it is rare, there is no standard surgical approach for a giant coronary artery aneurysm. Further studies need to focus on standardized surgical management of patients with giant coronary artery aneurysms.

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

We report the case of a 66-year-old man with a left anterior descending (LAD) coronary artery aneurysm. Cigarette smoking and hepatitis C virus infection were in his clinical history. Coronary angiography performed in 2002 showed a LAD aneurysm, a 50% stenosis of the right coronary artery, and a 30% stenosis of the left main artery (Figure 1, a through c). The ECG in 2002 was normal (Figure 2b i ). In 2002, the patient underwent successful Jomed polytetrafluoroethylene (PTFE)-coated stenting (3.0×20 mm) to cover the aneurysm (Figure 1d). After stenting, the patient was discharged on ticlopidine 250 mg/d for 1 year, aspirin 100 mg/d, metoprolol 25 mg/d, and transdermal nitrates. Figure 1. LAD …

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

BACKGROUNDGiant coronary artery aneurysms (CAA), entailing thrombosis, myocardial infarction, and sudden death, are the most severe and life-threatening complications of Kawasaki disease (KD). Giant aneurysms rarely regress and can later transform into stenoses. Data on dynamic follow-up are scarce in the literature.AIMTo evaluate clinical features and long-term outcomes of giant CAA in children with KD.METHODSA single-center retrospective study included data from patients with KD and giant CAA in the Irkutsk region (2012-2023). CAA criteria according to the American Heart Association guidelines of 2017 were used: (1) Dilated coronary artery with diameter Z-score > 2 standard deviations (SD) but < 2.5 SD; (2) Small CAA with Z-score > 2.5 SD but < 5 SD; (3) Medium CAA with Z-score > 5 SD but < 10 SD; and (4) Giant CAA with Z-score > 10 SD or ≥ 8 mm.RESULTSThe mean age of children with coronary dilatation/aneurysms was 2.5 years, and the male-to-female ratio was 3:1. Patients with giant/medium CAA had symptoms of cerebral dysfunction more often compared with children with moderate (Z-score < 5 SD but > 2.0 SD) coronary dilatation (62.0% vs 21.0%, P = 0.019). Major cardiovascular events (myocardial infarction, coronary artery bypass grafting, acute coronary syndrome, ischemic cardiomyopathy, left ventricular aneurysm, and giant extracardiac aneurysm) occurred in 55.5% of patients who had giant CAA. At follow-up the complete regression of giant/medium CAA was observed in 58.0% and partial regression in 42.0% after a mean of 2.3 and 5.5 years, respectively. All thrombi detected by echocardiography, CT, and angiography in giant/medium CAA disappeared between 1 year and 5 years (mean: 15 months). All patients survived.CONCLUSIONRisk factors for giant CAA were male sex, early age, and cerebral dysfunction. Complete regression of giant coronary aneurysms occurred in 58.0% of patients after follow-up of 2.3 years.

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

With Kawasaki disease it is important to clarify the mechanisms of coronary artery aneurysm and thrombus to avoid acute myocardial infarction. The authors tested the hypothesis that shear stress is reduced at coronary branching sites and in coronary artery aneurysms, and that this reduction of shear stress can promote formation of coronary artery aneurysms and thrombus. The subjects were 111 children with Kawasaki disease with left coronary artery aneurysms, classified into three groups: giant coronary artery aneurysm (n= 28, diameter of coronary artery >8 mm), aneurysm (n= 44, diameter of coronary artery =8 mm), and normal-appearing coronary (n= 39). Averaged peak flow velocity (APV), flow patterns and shear stress were measured and calculated at normal-appearing coronary vessels, left coronary artery branching sites and intra-coronary aneurysm using flow wire, and coronary angiography. Also, presence and appearance of thrombus were detected by intravascular ultrasonography. The authors found that 90.3% of the coronary artery aneurysms occurred at major left coronary branching sites. APV and shear stress were significantly decreased in giant coronary artery aneurysms (APV, 7.1 +/- 2.1 cm/s; shear stress, 3.8 +/- 2.1 dyne/cm(2)) and at the left coronary artery branching site (APV, 9.1 +/- 1.2; shear stress, 1+/-+/-.2 3.0). In total, 20 of 24 thrombi were detected only in giant aneurysm, and all patients exhibited disturbed flow pattern in their giant coronary artery aneurysms. Reduced shear stress and disturbed flow pattern may lead to coronary artery aneurysm and thrombus formation.

Bilateral congenital coronary artery fistulae complicated with a giant coronary artery aneurysm is a very rare condition. A coronary artery aneurysm is a coronary artery dilatation that exceeds the diameter of normal adjacent segments or the diameter of the patient's largest coronary vessel by 1.5 times. The complications associated with a coronary artery aneurysm include thrombosis, embolization, rupture, vasospasm, congestive heart failure and infectious endocarditis. We report on a 63-year-old woman presenting with severe heart failure related to bilateral coronary artery fistulae. A giant coronary aneurysm was noted in the right coronary artery, and a tortuous coronary artery fistula was noted in the left coronary artery. Symptoms were relieved after surgical intervention for bilateral coronary artery fistulae.

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