Giant Coronary Artery Aneurysm Research Articles

Variation in the management and treatment of children with giant coronary artery aneurysm following Kawasaki disease.

Giant coronary artery aneurysms are rare but potentially fatal complications of Kawasaki disease. The lack of evidence-based recommendations on their management and treatment cause guidelines and practices to differ. We aimed to assess these variations. An anonymous online survey regarding surveillance, imaging, pharmacological management, and interventional practices was distributed among 134 physicians attending to Kawasaki disease patients worldwide. A p-value of <0.05 was deemed significant. The majority (60%) of respondents were general paediatric cardiologists, and 29% interventional specialists. The average years in practice was 15 ± 9.6. Physicians from Asia had the most experience with giant coronary artery aneurysms. American practitioners preferred combining anticoagulants with aspirin. Beta-blockers and statins were more likely used in teenagers versus younger children. Cardiac catheterisation was most (52%) chosen for coronary surveillance in patients with echocardiogram anomalies, followed by Coronary CT-angiography. The indications for coronary intervention were split among respondents, regardless of geographic region or experience. The preferred treatment of coronary stenosis was percutaneous intervention (69%) versus bypass surgery. For thrombosis, thrombolytics (50%) were preferred over percutaneous (39%) and surgical (11%) interventions. Most (92%) preferred intervening in young children in a paediatric facility but were split between a paediatric and adult facility for older children. Most chose combined management by adult and paediatric specialists for either age-scenarios (70, 82%). As identified by our study, the lack of large studies and evidence-based recommendations cause uncertainty and ambivalence towards certain treatments. International collaborative efforts are needed to provide more robust evidence in the management of these patients.

Surgery for a giant atherosclerotic left main trifurcation saccular coronary artery aneurysm.

Aneurysms of the left main coronary artery are rare clinical entities with an incidence of 0.1%. We herein report a case of left main trifurcation aneurysm in a 57-year-old woman whose coronary angiography demonstrated a giant saccular aneurysm of trifurcation of the left main coronary artery, measuring 32 × 21mm with tight post-aneurysmal stenosis in the intermediate artery. The patient underwent coronary artery bypass grafting following the resection of CAA and reconstruction with saphenous vein roof-plasty, which ensured the preservation of the native coronary vasculature. At the 6-month follow-up, she remained asymptomatic and preserved preoperative left ventricular function.

Successful Surgical Management of Multiple Giant Coronary Artery Aneurysms with Coronary Artery Fistulas.

Successful Surgical Management of Multiple Giant Coronary Artery Aneurysms with Coronary Artery Fistulas.

A School-Goer’s Nightmare: Case Report of CABG in an 8 Year-Old with Kawasaki Disease

Introduction: Kawasaki disease (KD) is an acute vasculitis primarily affecting medium-sized arteries, particularly the coronary arteries (CA). Its impact varies, with some patients developing transient dilation while others experience severe coronary artery aneurysms (CAA), leading to complications such as thrombosis, stenosis, and myocardial ischemia. Male gender, delayed diagnosis, incomplete presentation of KD, and resistance to Intravenous Immunoglobulin (IVIG) therapy are risk factors for CAA. The Japanese Ministry of Health classifies CAAs by size, with giant aneurysms (&gt;8 mm) carrying significant long-term risks, including coronary thrombosis and acute coronary syndrome. Case Report: We report the case of an 8-year-old Malay boy with giant CAA secondary to KD. The patient presented with classical KD symptoms and inflammatory markers, with echocardiography revealing a giant aneurysm (8.7 mm) in the left anterior descending artery (LAD), alongside smaller aneurysms in the left circumflex and right coronary arteries (RCA). Initial management included IVIG, aspirin, and heparin, with warfarin and clopidogrel added subsequently. Coronary angiography indicated severe LAD stenosis (95%) with total occlusion and an aneurysmal segment distal to the occlusion. A multidisciplinary team determined that coronary artery bypass grafting (CABG) using bilateral internal mammary arteries was the optimal intervention. The patient had a favourable recovery and was stable at discharge. Discussion: CABG is recognized as a preferred treatment for severe or multivessel coronary involvement in KD, offering lower reintervention rates compared to percutaneous coronary intervention (PCI). Studies support CABG’s efficacy in managing coronary complications in KD, especially for complex or multivessel cases. Conclusion: This case illustrates the complexities of managing giant CAAs in paediatric KD and underscores the necessity of a multidisciplinary approach and long-term monitoring to

Клиническое наблюдение ретроспективной диагностики болезни Кавасаки

This research was aimed to demonstrating to the pediatric practitioners of a wide range the clinical case of retrospective diagnosis of Kawasaki disease (KD) based on detection of coronary artery (CA) aneurysms and anamnestic data analysis. Mucocutaneous lymphonodular syndrome (KD) is a systemic vasculitis of childhood that was characterized in the observed clinical case by fever, inflammatory changes in the mucous membranes, skin, lymph nodes and CA damage. Features of this clinical case were as follows: 1. the diagnosis was established retrospectively 5 years later than the KD was suffered based on accidental detection of CA aneurysms, 2. the lack of adequate KD treatment had led to the formation of CA aneurysms, including a single one of a giant size, 3. despite the long-term absence of generally accepted thrombosis prophylaxis in a patient with a giant CA aneurysm, only 11 years later did a thrombus in the aneurysm formed for the first time, which had then regressed rapidly despite it is known from the bibliographical data and those of the Authors’ own that the risk for thrombosis is very high in patients with giant aneurysms. Conclusion: late KD diagnosis entails the development of the cardiovascular system complications, in particular, coronary aneurysms, which in its turn in the reported clinical case were an accidental finding during the patient’s checkup.

Surgical management of giant coronary artery aneurysms.

Surgical management of giant coronary artery aneurysms.

Refractory Kawasaki Disease With a Giant Aneurysm Successfully Treated With Infliximab and Enoxaparin: A Case Report

Kawasaki disease (KD) is the most common vasculitis in children, and can result in the development of coronary artery aneurysms (CAAs) if not properly managed. While intravenous immunoglobulin (IVIG) and aspirin are standard first-line treatments, refractory KD may develop, increasing the risk of coronary complications. Herein, we report the case of a young girl with KD who initially responded to IVIG, but later developed a giant CAA, despite additional treatments. Infliximab stabilized her condition, and one year later, the CAA remained stable without thrombus formation. This case demonstrates that clinical appearance, particularly fever, may not fully reflect the patient's condition, as fever can subside immediately after treatment, but may relapse days later, underscoring the need for vigilant monitoring.

New Insights into the Pathophysiology of Coronary Artery Aneurysms.

Coronary aneurysms are typically defined as sections of a coronary artery where the diameter is more than 1.5 times that of an adjacent normal segment. In rare circumstances, these aneurysms can become exceedingly large, leading to the classification of giant coronary artery aneurysms. Despite their occurrence, there is no clear consensus on the precise definition of giant coronary artery aneurysms, and their etiology remains somewhat ambiguous. Numerous potential causes have been suggested, with atherosclerosis being the most prevalent in adults, accounting for up to 50% of cases. In pediatric populations, Kawasaki disease and Takayasu arteritis are the primary causes. Although often discovered incidentally, coronary artery aneurysms can lead to severe complications. These complications include local thrombosis, distal embolization, rupture, and vasospasm, which can result in ischemia, heart failure, and arrhythmias. The optimal approach to medical, interventional, or surgical management of these aneurysms is still under debate and requires further clarification. This literature review aims to consolidate current knowledge regarding coronary artery aneurysms' pathophysiology, emphasizing their definition, causes, complications, and treatment strategies. Recent research has begun to explore the molecular mechanisms involved in the formation and progression of coronary artery aneurysms. Various molecules, such as matrix metalloproteinases (MMPs), inflammatory cytokines, and growth factors, play crucial roles in the degradation of the extracellular matrix and the remodeling of vascular walls. Elevated levels of MMPs, particularly MMP-9, have been associated with the weakening of the arterial wall, contributing to aneurysm development. Inflammatory cytokines such as tumor necrosis factor-alpha (TNF-α) and interleukins (IL-1β and IL-6) have been implicated in promoting inflammatory responses that further degrade vascular integrity. Additionally, growth factors such as vascular endothelial growth factor (VEGF) may influence angiogenesis and vascular remodeling processes. Understanding these molecular pathways is essential for developing targeted therapies aimed at preventing the progression of coronary artery aneurysms and improving patient outcomes.

Kawasaki Disease Diagnosis and Treatment in over 1000 Patients: A Continuum of Dysregulated Inflammatory Responses.

Kawasaki disease (KD) is a pediatric vasculitis, leading to coronary artery aneurysms (CAAs) in ~4-14%. Attention to the etiology and course of KD was generated by the close mimic of a SARS-CoV-2-induced phenotype, called multisystem inflammatory syndrome in children (MIS-C). A total of 1179 cases were collected from 2012 with ~50% of cases retrospectively included. Clinical characteristics were described and risk factors for CAA (persistence) were investigated. Phenotypic patterns of the prospectively included KD patients were evaluated. These patterns were also compared to the seronegative KD and seropositive MIS-C cases identified during the SARS-CoV-2 pandemic. KD mostly affected boys and children < 5 years. IVIG resistance, CAAs, and giant CAAs occurred in 24.5%, 21.4%, and 6.6%, respectively. Giant CAAs were significantly more likely to normalize to a normal Z score in patients that were younger than 2.5 years old at the time of initial giant CAA (χ2 test p = 0.02). In our prospective (SARS-CoV-2-seronegative) KD series, there was a diminishing male predominance over time, whereas the proportions of incomplete presentations (p < 0.001) and patients with circulatory shock (p = 0.04) increased since the COVID-19 pandemic. Pre- and post-pandemic KD cases presented with different levels of C-reactive protein, thrombocyte counts, and hemoglobin levels over the years. Compared to pandemic KD, SARS-CoV-2-seropositive MIS-C patients were older (p < 0.001), and more often required intensive care admission (p < 0.001), with a gradual decrease over time between 2020 and 2022 (p = 0.04). KD carried a substantial risk of CAA development in contrast to MIS-C. the phenotypic changes seen over the last twelve years of our prospective follow-up study suggest a spectrum of hyperinflammatory states with potentially different triggering events within this clinical entity.

Coronary Artery Aneurysm or Ectasia as a Form of Coronary Artery Remodeling: Etiology, Pathogenesis, Diagnostics, Complications, and Treatment.

Coronary artery aneurysm or ectasia (CAAE) is a term that includes both coronary artery ectasia (CAE) and coronary artery aneurysm (CAA), despite distinct phenotypes and definitions. This anomaly can be found in 0.15-5.3% of coronary angiography. CAE is a diffuse dilatation of the coronary artery at least 1.5 times wider than the diameter of the normal coronary artery in a patient with a length of over 20 mm or greater than one-third of the vessel. CAE can be further subdivided into diffuse and focal dilations by the number and the length of the dilated vessels. Histologically, it presents with extensive destruction of musculoelastic elements, marked degradation of collagen and elastic fibers, and disruption of the elastic lamina. Conversely, CAA is a focal lesion manifesting as focal dilatation, which can be fusiform (if the longitudinal diameter is greater than the transverse) or saccular (if the longitudinal diameter is smaller than the transverse). Giant CAA is defined as a 4-fold enlargement of the vessel diameter and is observed in only 0.02% of patients after coronary. An aneurysmal lesion can be either single or multiple. It can be either a congenital or acquired phenomenon. The pathophysiological mechanisms responsible for the formation of CAAE are not well understood. Atherosclerosis is the most common etiology of CAAE in adults, while Kawasaki disease is the most common in children. Other etiological factors include systemic connective tissue diseases, infectious diseases, vasculitis, congenital anomalies, genetic factors, and idiopathic CAA. Invasive assessment of CAAE is based on coronary angiography. Coronary computed tomography (CT) is a noninvasive method that enables accurate evaluation of aneurysm size and location. The most common complications are coronary spasm, local thrombosis, distal embolization, coronary artery rupture, and compression of adjacent structures by giant coronary aneurysms. The approach to each patient with CAAE should depend on the severity of symptoms, anatomical structure, size, and location of the aneurysm. Treatment methods should be carefully considered to avoid possible complications of CAAE. Simultaneously, we should not unnecessarily expose the patient to the risk of intervention or surgical treatment. Patients can be offered conservative or invasive treatment. However, there are still numerous controversies and ambiguities regarding the etiology, prognosis, and treatment of patients with coronary artery aneurysms. This study summarizes the current knowledge about this disease's etiology, pathogenesis, and management.

A heavy ball: the rare giant right coronary artery aneurysm with thrombosis.

A heavy ball: the rare giant right coronary artery aneurysm with thrombosis.

Outcomes Associated with Giant Coronary Artery Aneurysms after Kawasaki Disease: A Single-Center United States Experience

To determine the long-term outcomes among a cohort of patients with Kawasaki disease (KD) and a history of giant coronary artery aneurysms (CAA) at a single US center. There were 60 patients with KD and giant CAAs identified between 1989 and 2023. The majority of patients were male (71.7%) with median age at diagnosis of 0.9 years (0.2-13.3). Patients were followed for a median of 11 years, up to 34.5 years. MACE occurred in 13 (21.7%) patients at a median of 1.4 years (0.04-22.6) after KD diagnosis. The 10-, 20-, and 30-year MACE-free rates were 75%, 75%, and 60%. Patients with maximal CA z-scores ≥20 or bilateral CAA were more likely to have MACE. During follow-up, 26.7% of CAA regressed to normal luminal diameter at a median of 3.6 years (0.6-12.0). The 10-, 20- and 30-year likelihood of CA regression to normal luminal diameter was 36%, 46%, and 46%. Over 30 years, MACE occurred in nearly 22% of patients, more often in those with bilateral CAA or CA z-scores ≥20. Despite regression to normal luminal diameter in over 25% of CAA, patients with a history of KD-associated giant CAA require ongoing surveillance for cardiac complications, even years after the initial disease.

Abstract 2112: Silent Killer: The Rare And Potentially Lethal Right Coronary Artery Aneurysm

Giant coronary artery aneurysms, defined as diameters larger than 20 mm, are exceedingly rare and constitute a life-threatening occurrence. This case involves a patient with a giant right coronary artery (RCA) aneurysm and multivessel coronary artery disease, successfully managed by surgical intervention through quintuple coronary artery bypass grafting (CABG). A 65-year-old male with a history of hypertension presented with acute-onset chest pain. The initial workup revealed an inferolateral ST-segment elevation myocardial infarction (STEMI). The patient underwent emergency coronary angiography revealing a high-grade stenosis in the proximal and mid left anterior descending coronary artery, along with a remarkably tortuous and enlarged mid to distal RCA aneurysm measuring (21mm wide x 20mm long) and featuring significant post aneurysmal stenosis. Attempts at percutaneous angioplasty were unsuccessful due to extensive thrombosis in the RCA. The patient continued to experience active chest pain, necessitating the placement of an intra-aortic balloon pump in the right common femoral artery, the insertion of a temporary pacemaker wire to address bradycardia, and the initiation of pressor support for hypotension. Additionally, intravenous heparin and antiplatelet therapy were administered. Finally, the patient underwent a quintuple CABG to address the multivessel disease. Coronary artery aneurysm is an uncommon yet potentially perilous condition. The selection of appropriate management and intervention is crucial for averting fatal consequences associated with coronary artery aneurysms.

Features of diagnosis of incomplete Kawasaki disease in young children. A clinical case of Kawasaki disease with the formation of giant coronary artery aneurysms

Kawasaki disease (KD) is a mucocutaneous lymphonodular syndrome, a vasculitis of unknown etiology that mainly affects children under the age of 5 years. Today, KD is the most common cause of acquired heart disease in children in developed countries. Given the absence of pathognomonic diagnosis of the disease, the diagnosis is based solely on the identification of clinical signs and the exclusion of other clinically similar diseases. Timely therapy with intravenous immunoglobulin has significantly reduced the incidence of complications, such as coronary artery aneurysms, from 25 to ≈ 4 %. The long-term prognosis of the disease depends on the initial and current level of coronary artery damage. Patients with coronary artery aneurysms remain at high risk of developing myocardial ischaemia due to thrombosis and stenosis of the coronary arteries, which can occur in case of untimely treatment for KD. Therefore, the life of these patients depends on lifelong thromboprophylaxis and timely diagnosis of stenosis. The article presents a clinical case of incomplete Kawasaki disease, which was complica­ted by the development of giant coronary artery aneurysms.

Right Coronary Artery Aneurysm Masquerading as a Pericardial Cyst: A Case Report

Coronary artery aneurysms (CAAs) or ectasias are dilatations of arterial segments, commonly detected as an incidental finding during angiography. The incidence of CAAs varies from 0.3% to 5.3%, mainly caused by atherosclerosis, though infections, inflammatory conditions, and connective tissue disorders can also contribute. Giant CAAs are rare with a reported prevalence of 0.02–0.2%. In our case, a 45-year-old female has been diagnosed with a giant aneurysm in the right coronary artery, which was initially misinterpreted as a pericardial cyst. In addition, she has a bicuspid aortic valve and severe aortic stenosis, hence advised for surgical management.

Acute myocardial infarction due to giant coronary artery aneurysm and arteriovenous fistula: a challenging case report and review of the literature

BackgroundA coronary artery aneurysm (CAA) is an abnormal dilation of a coronary artery segment often accompanied by coronary artery fistula (CAF), leading to communication between a coronary artery and a cardiac chamber or a part of the coronary venous system. Both CAAs and CAFs can present with symptoms and signs of myocardial ischemia and infarction.Case presentationWe describe the case of a 46-year-old woman with non-ST-elevation myocardial infarction (NSTEMI) caused by a “giant” CAA. Various imaging modalities revealed a thrombus-containing aneurysm located at the right-posterior cardiac border, with established arteriovenous communication with the distal part of left circumflex artery (LCx). After initial treatment with dual antiplatelet therapy, a relapse of pain was reported along with a new increase in troponin levels, electrocardiographic abnormalities, reduced left ventricular ejection fraction (LVEF) and thrombus enlargement. Surgical excision of the aneurysm was favored, revealing its true size of 6 cm in diameter. Τhe aneurysm was excised without complications. The patient remained asymptomatic during follow-up.ConclusionsManagement of rare entities such as “giant” CAAs and CAFs can be challenging. Cases such as this can serve as precedents to facilitate treatment plans and develop consistent recommendations, emphasizing the importance of personalized strategies for future patients.

Giant Coronary Artery Aneurysm Associated with Extraluminal Migration of Stent: Role of Multimodal Imaging

Abstract Extraluminal migration of a drug-eluting intracoronary stent into pericardial space with or without aneurysm formation is a rare complication. It is usually symptomatic although rare asymptomatic cases have been described. Herein, we report a 49-year-old male who presented with atypical chest pain 9 years after intracoronary stent implantation in the right coronary artery and the ramus intermediate branch. Echocardiographic examination showed a 7.4 cm × 3.8 cm large oblong cystic mass along the lateral wall of the left ventricle with inward bulging of the adjacent segments. Computed tomography and invasive coronary angiography showed this mass to be a partly thrombosed giant oblong coronary aneurysm arising from the bifurcation of the left main coronary artery with extraluminal migration of the stent from the ramus branch.

Складність діагностики рефрактерної хвороби Кавасакі під «маскою» гемобластозу: клінічний випадок із досвіду гематолога

Kawasaki disease (KD) is an acute systemic vasculitis that is the most common cause of acquired heart disease in children under 5 years of age with hyperthermia. Diagnosis of KD is a clinical challenge, given the wide range of clinical manifestations and similarities with many viral and bacterial diseases. Purpose - to describe a clinical case of refractory КD in a three-month-old girl with an emphasis on the importance of echocardiography and coronary angiography for the final verification of the disease, urgent initiation of treatment with minimal suspicion of КD. Clinical case. The article reports on a three-month-old patient with a difficult diagnosis of refractory form of КD. The disease debuted with hyperthermia, enterocolitis, obstructive bronchitis, and hepatoliver syndrome. The manifestations of skin exanthema were initially considered as an allergic dermatitis to the use of a cephalosporin antibiotic. Multisystemic inflammatory syndrome associated with SARS-CoV-2 infection was suspected. After the use of immunosuppressive therapy with mega-doses of dexamethasone and intravenous human immunoglobulin 2 g/kg/day for 3 days, clinical improvement was achieved. Subsequently, the haemogram showed an increase in neutrophilic hyperleukocytosis to the appearance of blast cells, hyperthrombocytosis and severe anaemia. A differentiation was made between a leukemic reaction, the debut of myeloproliferative disease, juvenile myelomonocytic leukaemia. Against the background of a rapid decrease in the number of leukocytes, the girl developed hyperthermia, migratory intermittent maculopapular rash, and foots edema. Laboratory findings included thrombocytosis and an increase in acute-phase parameters. Echocardiography revealed left ventricular dilation, a small amount of excess fluid in the pericardium, dilatation of the left coronary artery evenly to the bifurcation. Computed tomography revealed giant coronary artery aneurysms, which gave grounds to diagnose KD. The study was performed in accordance with the principles of the Declaration of Helsinki. Informed consent of the parents of child was obtained for the study. No conflict of interests was declared by the authors.

Intermediate and long-term outcomes of transcatheter closure of congenital coronary cameral fistulas in 66 children

Objective: To evaluate the intermediate and long-term outcomes and technical aspects of transcatheter closure (TCC) of coronary cameral fistulas (CCF) in pediatric patients. Methods: This was a case-control study. All pediatric patients with CCF who underwent TCC between January 2005 and December 2019 were retrospectively reviewed. Data was collected from medical records, including demographic characteristics, procedural details, intraoperative and postoperative serious adverse events, follow-up results and prognosis. Patients with serious adverse events and without serious adverse events were compared regarding their clinical features and CCF characteristics. Comparisons between groups were performed with independent sample t test, chi-square test or Fisher exact test. Results: A total of 66 CCF patients (34 boys, 32 girls, 3.9 (1.9, 6.2) years old, 15 (11, 20) kg) underwent attempted TCC. All of the CCF were all medium or large fistulas including 55 proximal fistulas (83%) and 11 distal fistulas (17%). The CCF originated more frequently from the right coronary artery (38 cases (58%)), followed by the left coronary artery (28 cases (42%)). The incidence of coronary artery aneurysms (CAA) was 61% (40/66).Procedural treatment was achieved in 64 patients and procedural success was achieved in 59 patients (92%). Six (9%) serious adverse events occurred in 5 patients during the perioperative period. Acute complications included procedure-related death in one patient and acute myocardial infarction in one patient. Periprocedural complications occurred in 3 patients at one day postoperatively including acute myocardial infarction (2 cases), occluder detachment (1 case), and tricuspid chordae tendinae rupture (1 case). Clinical follow-up data were available in 58 of the 62 patients who underwent initial successful TCC with a follow-up period of 9.3 (6.5, 13.4) years. Ten adverse events occurred in 9 patients including 5 complications consisted of aortic valve perforation (1 case), coronary thrombosis (1 case), progressive aneurysmal dilation after reintervention (1 case), and new-onset tricuspid valve prolapse with significant regurgitation (2 cases) and large residual shunts due to fistula recanalization (5 cases). Therefore, the incidence of intermediate and long-term adverse events was 17% (10/58). During the periprocedural and follow-up period, 16 adverse events occurred in 13 patients, whereas no adverse events occurred in 51 patients. Patients with seriovs adverse events presented with larger proportion of large CCF (11/13 vs. 39% (20/51), P=0.005), giant CAA (10/13 vs.14% (7/51), P=0.030), and higher mean pulmonary artery pressure ((20±9) vs.(16±6) mmHg, 1 mmHg=0.133 kPa, t=2.02, P=0.048) compared to patients without serious adverse events. Conclusions: TCC in CCF children appears to be effective with favorable intermediate and long-term outcomes. Strict indication of TCC is mandatory.

Giant coronary artery aneurysms in Kawasaki disease: A case report

Giant coronary artery aneurysms in Kawasaki disease: A case report