Coronary Artery Aneurysm Complications Research Articles

Giant Coronary Artery Aneurysm: A Successful Diagnosis

Coronary artery aneurysms (CAAs) are rare, with giant CAAs being even rarer. The precise pathophysiology of this phenomenon is still unknown. CAAs are seldom reported life-threatening abnormalities of the cardiovascular system. We herein present a case of a 74-year-old man who presented at the hospital complaining of chest pain. An adenosine thallium scan revealed a small, reversible defect in the inferior wall of the left ventricle extending into the apex, consistent with ischemia. Echocardiography uncovered a large right coronary artery (RCA) aneurysm, measuring 5.6 × 7.5 cm. Diagnostic coronary angiography confirmed the presence of a large RCA aneurysm and aneurysmal dilation of the left anterior descending and circumflex arteries with no flow-limiting lesions. A reversed saphenous vein interposition graft was placed from the ascending aorta to the right posterior descending artery. The RCA aneurysmal sac was resected and sent to pathology, which uncovered myxoid degeneration of the media as well as thrombus formation. No complications were encountered during the procedure. Early diagnosis is vital to avoid fatal complications of CAAs, and therapeutic approaches are currently individualized in view of absence of evidence-based management strategies.

History and Future of Treatment for Acute Stage Kawasaki Disease.

Kawasaki disease is a form of vasculitis, mainly in small and medium arteries of unknown origin, occurring frequently in childhood. It is the leading form of childhood-onset acquired heart disease in developed countries and leads to complications of coronary artery aneurysms in approximately 25% of cases if left untreated. Although more than half a century has passed since Professor Tomisaku Kawasaki's first report in 1957, the cause is not yet clear. Currently, intravenous immunoglobulin therapy has been established as the standard treatment for Kawasaki disease. Various treatment strategies are still being studied under the slogan, “Ending powerful inflammation in the acute phase as early as possible and minimizing the incidence of coronary artery lesions,” as the goal of acute phase treatments for Kawasaki disease. Currently, in addition to immunoglobulin therapy, steroid therapy, therapy using infliximab, biological products, suppression of elastase secretion inside and outside the neutrophils, inactivated ulinastatin therapy and cyclosporine therapy, plasma exchange, etc. are performed. This chapter outlines the history and transition of the acute phase treatment for Kawasaki disease.

Urinary Colorimetric Sensor Array and Algorithm to Distinguish Kawasaki Disease from Other Febrile Illnesses.

ObjectivesKawasaki disease (KD) is an acute pediatric vasculitis of infants and young children with unknown etiology and no specific laboratory-based test to identify. A specific molecular diagnostic test is urgently needed to support the clinical decision of proper medical intervention, preventing subsequent complications of coronary artery aneurysms. We used a simple and low-cost colorimetric sensor array to address the lack of a specific diagnostic test to differentiate KD from febrile control (FC) patients with similar rash/fever illnesses.Study DesignDemographic and clinical data were prospectively collected for subjects with KD and FCs under standard protocol. After screening using a genetic algorithm, eleven compounds including metalloporphyrins, pH indicators, redox indicators and solvatochromic dye categories, were selected from our chromatic compound library (n = 190) to construct a colorimetric sensor array for diagnosing KD. Quantitative color difference analysis led to a decision-tree-based KD diagnostic algorithm.ResultsThis KD sensing array allowed the identification of 94% of KD subjects (receiver operating characteristic [ROC] area under the curve [AUC] 0.981) in the training set (33 KD, 33 FC) and 94% of KD subjects (ROC AUC: 0.873) in the testing set (16 KD, 17 FC). Color difference maps reconstructed from the digital images of the sensing compounds demonstrated distinctive patterns differentiating KD from FC patients.ConclusionsThe colorimetric sensor array, composed of common used chemical compounds, is an easily accessible, low-cost method to realize the discrimination of subjects with KD from other febrile illness.

Kawasaki Disease: Paraclinical Evolution and Clinical Outcomes of Iranian Patients

It is well known that Kawasaki disease (KD) is a small to medium vessel vasculitis that affects children six months to four-years-of-age. Diagnosis is based on clinical criteria mostly from the affected skin and mucosa (1). It was very interesting that Sedighi et al. and Soleimani et al. in their recent publications in the Journal of Comprehensive Pediatrics revealed the clinical characteristics of Kawasaki disease, and the liver and renal paraclinical abnormalities, respectively, of this condition in the Iranian population (2, 3). In the study of Sedighi et al., 74 patients less than fiveyears-of-age were discharged with a diagnosis of KD, from 2004-2013, at the Besat Hopsital, Hamadan. Among these patients, in 44 (59.5%) a diagnosis of complete KD was made, while in 30 patients (40.5%) they were diagnosed as incomplete KD. Conjunctivitis (79%) was the most prevalent characteristic, followed by finger desquamation in 54% of patients, leukocytosis in 49.3%, and cervical lymphadenopathy in 40% of patients. Peripheral erythema was the least commonly seen clinical characteristic. Despite the accurate treatment, according to the American Heart Association guidelines, coronary artery aneurysm developed in 11 (14.8%) patients and one possible explanation might be the high frequency of incomplete KD diagnosis in comparison with other studies (2). Soleimani et al. in their study determined the frequency of liver and renal abnormalities in a study of 47 patients with KD in the Ali-Ebne-Abitaleb Hospital, Zahedan, for the period 2006-2013. According to this study, liver involvement was very common and 46.8% of the patients were affected. The manifestations of hepatic involvement included; gallbladder hydrops, cholestatic hepatitis, and asymptomatic increase of liver enzymes. The last one was the most frequent, affecting 42% of the patients in this study and this is very close to other medical reports. Renal involvement is not as frequent as liver impairment, but it is estimated to affect 38.3% of patients and sterile pyuria seems to be the major manifestation (36.2%) of renal involvement. In the Sedighi et al. study, the percentage of pyuria was 20.2% and the difference from the usual frequency, which is usually found in 33-63% of patients, might be explained by the fact that pyuria is a transient symptom and urine analysis should be repeated in suspected patients (2-4). In light of these observations, we should consider that Kawasaki Disease might not fulfill all the criteria, therefore, it is essential to recognize incomplete KD in order to treat the condition properly and to avoid the complication of coronary artery aneurysms. Conjunctivitis and finger desquamation seem to be the most important clinical manifestations of a sick child who has KD.

Successful Treatment of a Coronary Artery Aneurysm that Developed with In-Stent Restenosis after Drug-Eluting Stent Implantation

Coronary artery aneurysms are detected rarely during coronary angiography, and are associated with injury to the mechanical vessel wall during percutaneous coronary intervention. Potential causes also include atherosclerosis, congenital defects, connective tissue disorders, vasculitis, infection, drug-related injury, and trauma; it can also be idiopathic. The complications of coronary artery aneurysms vary, but they rupture only rarely. However, there is no consensus treatment strategy for coronary artery aneurysm after coronary intervention. We report a case of a 55-year-old male who developed a coronary artery aneurysm and in-stent restenosis after percutaneous coronary intervention with a drug-eluting stent. The aneurysm was treated successfully with the implantation of a graft stent. (Korean J Med 2014;86:608-611)

Association between GRIN3A Gene Polymorphism in Kawasaki Disease and Coronary Artery Aneurysms in Taiwanese Children

Kawasaki disease (KD) is pediatric systemic vasculitis with the classic complication of coronary artery aneurysm (CAA). It is the leading cause of acquired cardiovascular diseases in children. Some severe cases present with multi-organ involvement or neurological dysfunction. To identify the role of the glutamate receptor, ionotropic, N-methyl-d-aspartate 3A (GRIN3A) in KD, we investigated genetic variations in GRIN3A in a Taiwanese cohort of 262 KD patients (76 with and 186 without CAA complications). We used univariate and multivariate regression analyses to identify the associations between clinical characteristics and GRIN3A genetic variations in KD. According to univariate regression analysis, CAA formation in KD was significantly associated with fever duration (p < 0.0001), first Intravenous immunoglobulin (IVIG) used (days after day one of fever) (p < 0.0001), and the GRIN3A (rs7849782) genetic variant (p < 0.001). KD patients with GG+GC genotype showed a lower rate of developing CAA (GG+GC genotype: odds ratio = 0.26; 95% CI = 0.14–0.46). Significant associations were identified between KD with CAA complication and the GRIN3A (rs7849782) genetic variant by using multivariate regression analysis. Specifically, significant correlations were observed between KD with CAA complications and the presence of GG+GC genotypes for the GRIN3A rs7849782 single-nucleotide polymorphism (full model: odds ratio = 0.25; 95% CI = 0.14–0.46). Our results suggest that a polymorphism of the GRIN3A gene may play a role in KD pathogenesis.

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.

Sorting nexin 24 genetic variation associates with coronary artery aneurysm severity in Kawasaki disease patients

BackgroundThe sorting nexin (SNX) family is involved in endocytosis and protein trafficking and plays multiple roles in various diseases. The role of SNX proteins in Kawasaki disease (KD) is not known. We attempted to test whether genetic SNX variation associates with the risk of coronary artery aneurysm (CAA) formation in KD.Methods and resultsChi-square tests were used to identify SNX24 genetic variants associated with KD susceptibility and CAA formation in KD; models were adjusted for fever duration and time of first administration of intravenous immunoglobulin. We obtained clinical characteristics and genotypes from KD patients (76 with CAA and 186 without CAA) in a population-based retrospective KD cohort study (n = 262). Clinical and genetic factors were associated with CAA formation in KD. In addition, endothelial cell inflammation was evaluated. Significant correlation was observed between KD with CAA complications and the rs28891 single-nucleotide polymorphism in SNX24. Patients with CC + CT genotypes had lesser CAA complications. In lipopolysaccharide-treated human umbilical vein endothelial cells, siRNA knockdown of SNX24 significantly decreased gene expression of the proinflammatory cytokines IL-1 beta, IL-6, and IL-8.ConclusionsPolymorphisms in SNX24 may be used as genetic markers for the diagnosis and prognosis of CAA formation in KD.

A diagnostic algorithm combining clinical and molecular data distinguishes Kawasaki disease from other febrile illnesses

BackgroundKawasaki disease is an acute vasculitis of infants and young children that is recognized through a constellation of clinical signs that can mimic other benign conditions of childhood. The etiology remains unknown and there is no specific laboratory-based test to identify patients with Kawasaki disease. Treatment to prevent the complication of coronary artery aneurysms is most effective if administered early in the course of the illness. We sought to develop a diagnostic algorithm to help clinicians distinguish Kawasaki disease patients from febrile controls to allow timely initiation of treatment.MethodsUrine peptidome profiling and whole blood cell type-specific gene expression analyses were integrated with clinical multivariate analysis to improve differentiation of Kawasaki disease subjects from febrile controls.ResultsComparative analyses of multidimensional protein identification using 23 pooled Kawasaki disease and 23 pooled febrile control urine peptide samples revealed 139 candidate markers, of which 13 were confirmed (area under the receiver operating characteristic curve (ROC AUC 0.919)) in an independent cohort of 30 Kawasaki disease and 30 febrile control urine peptidomes. Cell type-specific analysis of microarrays (csSAM) on 26 Kawasaki disease and 13 febrile control whole blood samples revealed a 32-lymphocyte-specific-gene panel (ROC AUC 0.969). The integration of the urine/blood based biomarker panels and a multivariate analysis of 7 clinical parameters (ROC AUC 0.803) effectively stratified 441 Kawasaki disease and 342 febrile control subjects to diagnose Kawasaki disease.ConclusionsA hybrid approach using a multi-step diagnostic algorithm integrating both clinical and molecular findings was successful in differentiating children with acute Kawasaki disease from febrile controls.

Coronary Artery Aneurysms and Ectasia: Role of Coronary CT Angiography

Coronary artery dilatations-aneurysms and ectasia-are an uncommon and frequently unrecognized incidental finding in patients with coronary artery disease. Aneurysms and ectasia are associated with a vast group of disorders, and the evaluation and characterization of coronary aneurysms and ectasia represent a great diagnostic task with clinical and therapeutic implications. The underlying etiology is variable and includes degenerative, congenital, inflammatory, infectious, toxic, and traumatic causes. Unlike aneurysms, ectasia is more frequently seen in association with atherosclerosis or as a compensatory mechanism in those cases in which a proximal stenosis is noted in the opposite coronary artery; ectasia is also seen in some coronary artery anomalies, such as anomalous origin from the pulmonary artery, or as a result of a high-flow state, as seen in coronary artery fistulas. The diagnostic approach depends on the clinical scenario, and nowadays, noninvasive evaluation with multidetector computed tomography is possible. Imaging assessment should include evaluation of (a) the distribution, (b) maximal diameter, (c) presence or absence of intraluminal thrombi, (d) number, (e) extension, and (f) associated complications such as myocardial infarction. This article presents an overview of the definition, classification, etiology, clinical manifestations, and potential complications of coronary artery aneurysms and ectasia.

Kawasaki Syndrome in an Adult: Case Report and Review of the Literature in Adolescents and Adults

Kawasaki syndrome in adults is very rare, with fewer than 50 cases reported in the English-language literature. We describe the case of a physician with Kawasaki syndrome and summarize the clinical features and treatment of 11 patients in the literature since the last review in 1994. Our patient presented with high fever, conjunctivitis, and arthralgias, then developed progressive toxicity with oral lesions, cervical adenopathy, and desquamation of the fingers and toes. No exanthematous rash or coronary artery aneurysms were found. Recovery was rapid after therapy with aspirin and intravenous immunoglobulin (IVIg). The diagnosis of Kawasaki syndrome depends on clinical criteria and the exclusion of other diseases. This diagnosis can be challenging to make in an adult, particularly when it presents without all typical features. Kawasaki syndrome must be considered nonetheless in an adult with unexplained fever of more than 5 days duration, because early diagnosis and combination therapy with aspirin and IVIg can prevent the life-threatening complication of coronary artery aneurysms.