Incidence Of Coronary Artery Aneurysms Research Articles

Kawasaki Disease and Coronary Artery Aneurysms: From Childhood to Adulthood

Kawasaki disease is an acute, systemic vasculitis of childhood and confers a 25% risk of developing coronary artery aneurysms. Its etiology is unknown, but the incidence is increasing rapidly with linked gene polymorphisms having been identified. A constellation of symptoms, epidemics and seasonality all implicate an unidentified infective or environmental cause. Intravenous immunoglobulin therapy, aspirin and steroids all form the mainstay of acute treatment and reduces the incidence of coronary artery aneurysms if given before 7days. However, in some, these lesions persist and require ongoing management during follow-up during childhood and into adult life. Evidence for further investigations in order to minimize complications is presented in order to minimize the myofibroblast proliferation and stenosis in the long term.

Abstract 125: Steroid Treatment in the Acute Phase of Kawasaki Disease in Mexican Children. Are they Useful?

Background: Intravenous immunoglobulin (IVIG) and aspirin is the standard initial therapy in the treatment of Kawasaki disease (KD), which is proven to decrease the incidence of coronary artery aneurysms from 25% to less than 5%. There is increasing evidence for steroid therapy as adjunctive primary therapy with IVIG especially in those patients who are at increased risk of coronary artery aneurysms and in patients with risk of IVIG resistance. However, clinical trials evaluating the use of corticosteroids plus IVIG have produced confusing results. Objective: To evaluate the clinical efficacy and safety of steroids plus intravenous immunoglobulin (IVIG) combination therapy (IVIG+S) for the initial treatment of patients with KD to prevent coronary artery aneurysms (CAA) compared with the standard treatment with intravenous immunoglobulin plus aspirin (IVIG+A) in a Children’s Hospital in Mexico City. Material and Methods: An observational, comparative, retrospective and case-control study of all patients treated with IVIG for KD in our Institution from August 1995 to May 2014. The clinical presentation, laboratory results and coronary artery abnormalities in the IVIG+S and the IVIG+A groups were analyzed and compared. Results: We studied 295 patients with KD treated with IVIG, 136 (46.1%) received IVIG+A treatment and 159 (53.9%) received IVIG+S treatment. We didn’t found adverse reactions in the patients treated with steroids. The IVIG+S group were older 43.25 ± 43.04 than the non-steroid group 32.07 ± 24.51 (p < 0.008). Steroids were commonly use in incomplete cases (p < 0.059) and in patients with cardiac complications at diagnosis: pericardial effusion (p < 0.056) and pericarditis (p < 0.013). The steroid group has slightly more days of fever after the IVIG treatment 1.27 ± 1.51 days vs. 0.93 ± 0.924 days (p < 0.028). We found no difference in the development of CAA in both groups. (p = 0.221) Conclusions: There were no differences in the development of CAA with the use of steroids in the initial treatment of KD. Nevertheless steroids were used more commonly in incomplete forms of KD and in more severely affected patients, which could reflect the lack of difference in the development of CAA in both groups.

Guideline-Concordant Treatment of Kawasaki Disease With Immunoglobulin and Aspirin and the Incidence of Coronary Artery Aneurysm.

Current guidelines for Kawasaki disease (KD) recommend intravenous immunoglobulin infusion and echocardiography. However, no previous studies have evaluated the relationship between the treating hospital's concordance with guidelines and the prevention of coronary artery aneurysm in patients with KD. KD patients between 2010 and 2013 were identified in a Japanese national inpatient database. Guideline concordance was defined as the proportions of patients who received echocardiography as well as treatment with both immunoglobulin and aspirin, which were divided into quartiles. Multivariable logistic regression analysis was conducted to examine the association between the guideline concordance and the occurrence of coronary artery aneurysm with adjustment for patient backgrounds. In sum, 20 156 patients with KD were identified. The very high (>83.3%) concordance group had a lower rate of coronary artery aneurysm than the very low (<59.0%) concordance group (4.9% vs 9.9%; odds ratio, 0.45; 95% CI, 0.27 to 0.74; P = .002). All patients with KD should be treated according to the guidelines.

Management of Kawasaki disease in resource-limited settings.

Management of Kawasaki disease in resource-limited settings.

PO-1008 Clinical Features Of Incomplete Kawasaki Disease With Cervical Lymphadenopathy And Fever As Initial Manifestations

<h3>Objective</h3> This study sought to evaluate the clinical features of the incomplete Kawasaki disease (KD) with cervical lymphadenopathy and fever as initial manifestations. <h3>Methods</h3> We conducted a retrospective analysis of the clinical records of consecutive KD patients admitted to our hospital. Children presenting with cervical lymphadenopathy and fever as the initial manifestations, with none or only one another clinical feature of KD were defined as incomplete KD (IKD) group. <h3>Results</h3> A total of 1420 patients were included. Thirty-one (2.2%) were classified as IKD. There were no differences in age and gender ratio between two groups. The duration of fever in IKD group (10.3 ± 5.7 d) was longer than that in KD group (6.9 ± 3.8 d, p &lt; 0.05). Ten (32.3%) children in IKD group presented with bilateral conjunctival congestion. WBC and alanine transaminase (ALT) in IKD group were lower in IKD group. No differences were identified in other laboratory study results between two groups. The rate of non-responders of IVIG in IKD group is lower than KD group (3.3% vs 18.3%). Coronary artery lesions in IKD include coronary dilatation in 22(71%), aneurysm in 5 (16.1%) and giant aneurysm in 3 (9.7%), which is higher than KD group (27.7%, 5.2% and 1.1%). <h3>Conclusions</h3> Children with incomplete KD and cervical lymphadenopathy and fever as initial manifestations presented with longer period of fever and higher incidence of coronary artery aneurysm. Thus awareness of IKD should be kept in mind in this special group of children.

Endovascular management of giant coronary artery aneurysm

Coronary artery aneurysms defined as localized coronary dilations with diameters atleast 1.5 times the diameters of adjacent normal coronary segments.1 It is commonly seen secondary to atherosclerosis but has also been reported in patients who have vasculitis (Kawasaki syndrome), connective tissue disorders (Ehlers-Danlos or Marfan syndrome), and in patients who have undergone interventional procedures.2–5 A growing incidence of coronary artery aneurysms have been recently described after implantation of drug-eluting stent (DES).6–8 We report one such case which was treated successfully using a covered stent.

Clinical outcomes and risk factors of coronary artery aneurysms after successful percutaneous coronary intervention and drug-eluting stent implantation for chronic total occlusions

ObjectiveThe study aimed to analyze the risk factors and long-term outcomes associated with coronary artery aneurysms (CAAs) after successful percutaneous coronary intervention (PCI) and drug-eluting stent (DES) implantation in patients with CTOs. BackgroundThere are sporadic data available on post-procedure CAAs after transcatheter revascularization for CTOs. Methods and resultsA total of 141 patients with 149 CTOs who underwent successful CTO-PCI and DES implantation with angiographic follow-up from 2004 to 2010 were included. Patients were divided into CAA group and non-CAA group according to the presence of CAAs in the follow-up angiography. The independent predictors and major adverse cardiac events (MACEs) including cardiac death, myocardial infarction (MI) and target-vessel revascularization (TVR) were compared between two groups. The incidence of CAAs was 11.4% (17/149) after index procedure. Multivariate analysis showed that age (OR: 0.925, CI 0.873–0.980, P = 0.008), ostial occlusion (OR: 6.715, CI 1.473–30.610, P = 0.014), the parallel wire technique (OR: 6.167, CI 1.709–22.259, P = 0.005) and DES length (OR: 1.030, CI 1.002–1.058, P = 0.036) were the independent predictors of CAAs after successful CTO-PCI and DES implantation. MACEs were similar between two groups (adjusted hazard ratio 0.670; 95% CI 0.160–2.808; P = 0.584) during the 5-year follow-up. ConclusionsThe independent predictors of CAAs after successful CTO-PCI and DES implantation are age, ostial occlusion, the parallel wire technique and DES length. CAAs after index procedure are not frequently associated with adverse clinical events under dual antiplatelet therapy. Further large clinical studies are warranted to explore the clinical implications of patients with this distinct new entity.

Generalized aneurysmal disease in association with autosomal dominant polycystic disease.

A 78-year-old man with end-stage kidney disease secondary to autosomal dominant polycystic disease (ADPKD) on haemodialysis, presented to our institution following a myocardial infarction. He was known to have cystic involvement of his liver and spleen, significant mitral regurgitation secondary to mitral valve prolapse, and a distant history of abdominal aortic aneurysm repair. There was no family history of ADPKD or history of cerebral aneurysms. Coronary angiogram revealed widespread large coronary artery aneurysms affecting both branches of the left and right coronary artery, as well as significant atherosclerotic disease (Figure 1). During work up for coronary artery bypass surgery and mitral valve replacement, multiple other sites of aneurysmal disease were identified, including a 5 cm thoracic aortic aneurysm and bilateral femoral artery aneurysms (Figure 2). Fig. 1. Aneurysmal involvement of the mid to distal left anterior descending coronary artery (black arrow). Fig. 2. Bilateral femoral artery aneurysms (white arrows) together with large polycystic kidneys. Note incidental finding of calcified cyst in left polycystic kidney (black arrow). Whilst waiting for cardiac surgery the patient developed septic shock secondary to an infected renal cyst, requiring prolonged intensive care admission and extended rehabilitation. Given his age and frailty a decision was made to manage his widespread aneurysmal disease conservatively with regular radiographic surveillance. Previous case series have suggested a high incidence of coronary artery aneurysms in association with ADPKD. Hadimeri et al. [1] observed coronary aneurysms in 4 out of 30 (13%) ADPKD patients. This is markedly higher than the incidence observed in the general population, of ∼1.5% of patients studied by coronary angiography or autopsy [2]. There is less evidence regarding the link between ADPKD and aortic, thoracic and femoral aneurysms. While Bailey et al. [3] studied the association between aortic aneurysms and ADPKD, the study was unable to conclusively link the two conditions. Evidence linking thoracic or femoral aneurysms with ADPKD appears predominantly limited to case studies [4]. Given that the gene responsible for ADPKD has been linked with abnormalities of vascular smooth muscle, myofibroblasts, extracellular matrix and collagen defects [5], it seems reasonable for there to be widespread vascular abnormalities in this patient population, although it seems to be seldom described. In summary, we present a rare case of a patient with ADPKD and associated coronary artery aneurysms as well as aorto/iliac aneurysms.

A case of stent aneurysm following everolimus eluting stent implantation

Coronary artery aneurysm commonly occurs secondary to atherosclerosis,but aneurysm formation after stenting is a rare phenomenon specially after drug eluting stent implantation. Drug-eluting stents (DES) elute antiproliferative drugs, can inhibit neointimal growth and may delay healing after vascular injury, and DES implantation may be associated with a risk of coronary artery aneurysm formation. Coronary aneurysms have been reported from 3 days to up to 4 years after DES implantation procedures, with varying clinical presentations. Incidence of coronary artery aneurysms after DES implantation is low within the first 9 months, with a reported incidence of 0.2% to 2.3%, a rate similar to that reported after bare-metal stent (BMS) implantation (0.3% to 3.9%). In our case,the patient is a nondiabetic ,nonhypertensive 68 year old male who underwent coronary angioplasty in the RCA with everolimus eluting stent following acute coronary syndrome.After few weeks the patient came with exertional angina and coronary angiography revealed stent aneurysm formation with new disease proximal to the stent.

Primary Treatment of Kawasaki Disease with Corticosteroids

Kawasaki disease (KD), is the leading cause of acquired heart disease in children in the developed world. The use of aspirin and intravenous immunoglobulin as the initial therapy in KD is the standard of care, as they reduce the incidence of coronary artery aneurysms, the major cardiac morbidity from this disease. The place of corticosteroids in the initial therapy is; however, controversial. We describe the course of a one-year-old child with Kawasaki disease who was treated with aspirin and corticosteroids as the initial therapy, and discuss pertinent issues.

A Case of Kawasaki Disease with Coronary Aneurysm Responding to the 4th IVIG Treatment.

Kawasaki disease is an acute febrile illness that usually occurs in children younger than 5 years of age. The use of intravenous immunoglobulin (IVIG) within the first 10 days of illness has been shown to reduce the incidence of coronary artery aneurysms significantly. The relative roles of repeated doses of intravenous immunoglobulin (IVIG) are controversial in refractory Kawasaki disease (KD). Most experts recommend the second retreatment with IVIG, 2 g/kg in refractory KD. However, the dose-response effect of the third or fourth IVIG was uncertain. Although there have been a significant number of reports on new therapeutic options for refractory KD, such as steroid, infliximab, methotrexate, and other immunosuppressants, their effectiveness in reducing the prevalence of coronary artery aneurysms was unproven. We present here KD patient with small coronary artery aneurysm who is resistant to the third IVIG and steroid pulse therapy but showed improvement immediately after the infusion of the 4th IVIG on fever day 18.

The clinical profile of Kawasaki disease of children from three Polish centers: a retrospective study

Kawasaki disease (KD) is one of the most common vasculitides of childhood. The aim of this retrospective study is to determine the incidence of KD and to evaluate its presenting symptoms, clinical course, laboratory tests, and treatment in patients with complete KD and incomplete KD at three pediatric rheumatology centers in Poland from January 2011 to December 2012. A total of 27 Caucasian children (12 boys and 15 girls) with median age of 3 years (range 4 months–12 years) were included in this study. The incidence of complete versus incomplete KD was 17 (63 %) versus 10 (37 %) children, respectively. Patients with incomplete KD significantly less presented cervical lymphadenopathy (20 vs. 88.2 %; p = 0.00075), changes in extremities (30 vs. 76.5 %; p = 0.04), and bilateral nonpurulent conjunctivitis (60 vs. 100 %; p = 0.01). Cardiac assessments show that the majority of patients with KD have not got coronary artery aneurysms (CAA). The median time from the onset of symptoms to intravenous immunoglobulin (IVIG) infusion was 7 days for complete KD and 11 days for incomplete KD. IVIG delay in the incomplete KD had no effect on the incidence of CAA. In conclusion, there were no differences in demographic features, age of onset, and laboratory tests of patients with complete and incomplete KD. Patients with incomplete KD significantly rarely presented cervical lymphadenopathy, changes in extremities, and conjunctival injection. Electrocardiography is a sensitive test to recognize cardiac involvement in the acute phase of KD. Despite the fact that incomplete forms of presentation often delay diagnosis, in most patients treatment with IVIG can avoid complication of CAA.

Therapeutic immunomodulation in systemic vasculitis: Taking stock

Current data on therapeutic immunomodulation used to treat systemic vasculitides are reviewed in this paper, which also discusses ongoing and future developments in the field. In vasculitides associated with anti-neutrophil cytoplasmic antibodies, rituximab is a validated induction treatment that can serve as an alternative to cyclophosphamide and must be followed by maintenance treatment. In addition, the usefulness of rituximab as maintenance treatment was established recently. Immunoglobulins can be helpful adjuncts, most notably in patients with severe immunodepression. Plasmapheresis is indicated in patients with severe renal failure and may have a role in the treatment of alveolar hemorrhage syndromes. Mepolizumab has produced encouraging preliminary results in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Rituximab can be used in cryoglobulinemic vasculitis associated with hepatitis C virus infection when antiviral therapy fails or the disease is severe. Very low doses of interleukin-2 may be helpful in refractory forms. Rituximab is also an option in essential mixed cryoglobulinemia with uncontrolled vasculitis despite glucocorticoid and/or immunosuppressive treatment. In polyarteritis nodosa associated with the hepatitis B virus, a combination of short-course glucocorticoids, plasmapheresis, and antiviral therapy produces excellent outcomes. Intravenous immunoglobulins are used to treat Kawasaki disease, in which they diminish the incidence of coronary artery aneurysms. Several prospective controlled trials are currently assessing tocilizumab in giant-cell arteritis. Rituximab has useful effects in systemic vasculitis associated with rheumatoid arthritis. In Goodpasture's syndrome, plasmapheresis is indicated to clear the antibodies to glomerular membrane antigen, which can induce glomerulonephritis.

Management of acute and refractory Kawasaki disease

Acute Kawasaki disease (KD) is treated with high-dose intravenous immunoglobulin (IVIG), which is proven to decrease the incidence of coronary artery aneurysms from 25% to less than 5%. Aspirin is also given, although the evidence base is less secure. There is increasing evidence for steroid therapy as adjunctive primary therapy with IVIG, especially in Asian children. Approximately 10–30% of patients fail to respond to the initial IVIG and are at increased risk of coronary artery aneurysms. The optimal treatment for IVIG-nonresponsive KD remains controversial. Management options include further dose(s) of IVIG, corticosteroids, TNF-α blockade, cyclosporin A, anti-IL-1 and anti-CD20 therapy. In this article, the authors review the current evidence for treatment of acute KD and discuss options for IVIG nonresponders.

“Angiographic late catch-up” phenomenon after sirolimus-eluting stent implantation

BackgroundAlthough several randomized trials have shown that sirolimus-eluting stent (SES) substantially reduces in-stent restenosis, recent studies have suggested the possibility of late catch-up after SES implantation. We investigated long-term angiographic outcomes after SES implantation in real-world practice. MethodsThis study was conducted on 195 patients with 253 lesions who underwent the first and long-term angiographic follow-up after SES implantation. First follow-up was done at near 6months after SES implantation. Long-term angiographic follow-up was defined as that performed at least 36months after index procedure. Angiographies in patients who experienced target lesion revascularization at the time of the first angiographic follow-up were excluded from the current analysis. ResultsMinimal luminal diameter at long-term angiographic follow-up was significantly smaller compared with that at the first follow-up (2.21±0.65 vs. 2.40±0.55, p<0.001). In-stent late lumen loss between the first and long-term follow-up tended to be larger compared with that between SES implantation and the first follow-up (0.19±0.47 vs. 0.15±0.39, p=0.298). There was a trend for increased incidence of coronary artery aneurysm (1.6% and 7.5% at the first and long-term follow-up) and stent fracture (4.3% and 10.3%). Two stent aneurysms and one stent fracture were related with definite very late stent thrombosis. ConclusionAn “angiographic late catch-up” phenomenon and a trend toward increased incidence of coronary artery aneurysm and stent fracture were found at a median 46.5-month angiographic follow-up compared with a median 6-month follow-up.

Delayed diagnosis of Kawasaki disease: risk factors and outcome of treatment

Kawasaki disease (KD) is associated with a high incidence of coronary artery aneurysms in untreated children. Treatment with intravenous immunoglobulin (IVIG) within the 1st 10 days of illness reduces by approximately fivefold the prevalence of coronary artery abnormalities (CAA). Data regarding delayed diagnosis of KD in Thailand have not been reported in the literature. To determine the prevalence, risk factors and outcome of delayed diagnosis of KD in Thai patients. We retrospectively reviewed the medical records of patients at Chiang Mai University Hospital diagnosed as KD during 2000-2008. Patients were classified into two groups: Group I were diagnosed ≤10 days of fever and Group II were diagnosed >10 days of fever. Of 170 patients, 150 were in Group I [mean (SD) fever 7 (1·45) d] and 20 (11·7%) in Group II [mean (SD) fever 15 (4) d]. There were no statistical differences between the two groups in age, gender, number of KD clinical manifestations or laboratory results, except that Group II were of lower weight (p = 0·01). Group II were younger (p = 0·09) and had more incomplete criteria (p = 0·09) but the differences were not statistically significant. Group II had a higher incidence of CAA (75% vs 19%) (p<0·001), more severe CAA and more resistant cases (31·2% vs 9·5%) (p = 0·04). Patients with delayed diagnosis of KD have a higher risk of developing CAA and of a more severe outcome for coronary artery disease. Education is needed to make healthcare providers and physicians more aware of KD, especially in small children or those with incomplete KD.

Refractory Progression of Coronary Aneurysms, a Case of Delayed Onset Kawasaki Disease as Depicted by Cardiac Computed Tomography Angiography

Kawasaki disease (KD) is an immune-mediated vasculitis of unknown etiology with self-limited clinical course that was first described in 1967 by Dr. Tomisaku Kawasaki. It is a disease of early childhood and rare past late adulthood but one that can have detrimental consequences when there is a delay in diagnosis and treatment. Cardiovascular complications causing increased morbidity and mortality may include coronary artery aneurysms, myocardial infarction, heart failure, arrhythmias, and peripheral artery occlusion. Here, we present an atypical case of delayed onset KD in a young teenager. DS had visited three different emergency departments during the course of 2 weeks for unrelenting fevers. Despite multiple treatment protocols including immunoglobulin, steroids, and tumor necrosis factor-alpha antagonists, he continued to have progression of cardiovascular complications. While echocardiographic findings were suspicious for cardiac complications, a cardiac computed tomography (CT) angiography was able to clearly distinguish giant coronary aneurysms. Without prompt therapy, fever and manifestations of acute inflammation can last for several weeks to months with increased risk toward complications. The incidence of coronary artery aneurysms has been noted to be 25% in untreated patients with a mortality rate of up to 2%. Using low-dose protocols along with high spatial and temporal resolution of cardiac CT angiography may provide a useful and complimentary imaging modality in accurate diagnosis and follow-up of patients with KD.

The strategy of immune globulin resistant Kawasaki disease: A comparative study of additional immune globulin and steroid pulse therapy

We compared the clinical utility of additional intravenous immune globulin (IVIG) therapy with the clinical utility of steroid pulse therapy in patients with IVIG-resistant Kawasaki disease. We enrolled 164 patients with Kawasaki disease who were treated with a single dose of IVIG (2 g/kg) and aspirin (30 mg/kg per day). Twenty-seven of these patients (16%) were resistant to the initial IVIG treatment. We compared the effectiveness of treatment strategies for the initial IVIG-resistant 27 patients, 14 of these patients were treated with additional IVIG therapy, and the other 13 patients were treated with steroid pulse therapy (methylprednisolone 30 mg/kg per day for 3 days). Three patients in the group receiving additional IVIG treatment had coronary artery aneurysms (21.4%), no patients had coronary artery aneurysm in the steroid pulse therapy group; the difference in the incidence of coronary artery aneurysm was not statistically significant. The duration of high fever after additional treatment in the steroid pulse therapy group (1 ± 1.3 days) was significantly shorter than that in the additional IVIG treatment group (3 ± 2.4 days; P < 0.05). The medical costs were significantly lower in the steroid pulse therapy group than in the additional IVIG treatment group. Steroid pulse therapy was useful to reduce the fever duration and medical costs for patients with Kawasaki disease. Steroid pulse therapy and additional IVIG treatment were not significantly different in terms of preventing the development of coronary artery aneurysm.

Facial palsy in Kawasaki disease: report of two cases.

Facial palsy is an unusual complication associated with Kawasaki disease, with only a few published case reports. We report two patients with typical Kawasaki disease and facial palsy. Both had coronary artery aneurysms and were treated with intravenous immunoglobulin. The facial palsy resolved completely over the next several months in both patients. Coronary artery aneurysms resolved completely in one patient, and the other has not regressed to normal till now. The incidence of coronary artery aneurysm appears to be higher in the handful of reported cases of Kawasaki disease with facial palsy.

Left main coronary artery aneurysm: report of three cases

Left main coronary artery (LMCA) aneurysm is a rare coronary abnormality defined as localized coronary artery dilatations>1.5 to 2 times the diameter of the adjacent segments. The incidence of coronary artery aneurysm varies between 0.15% and 4.9%. It can be recognized by coronary angiography or multidetector computed tomography. Here, we present three cases of LMCA aneuryms, of one which firstly diagnosed by multidetected computed tomography.