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

BACKGROUND: A 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 PRESENTATION: We 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. CONCLUSIONS: Management 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.

[Pulmonary Artery Transection in Surgery for Coronary Artery Fistula].

Coronary artery fistula is a rare abnormality in the communication between a coronary artery and any of the cardiac chambers or major vessels. At present, there is no standard surgical treatment and the most appropriate method is selected on a case-by-case basis. We report one case of coronary artery fistulae in which pulmonary artery transection was required around the left main trunk (LMT). A 62-year-old man who had coronary artery fistulae with an aneurysm which increased from 12 mm to 16 mm in a two-year span. The fistula was located adjacent to the LMT. A complete aneurysm excision under cardiopulmonary bypass was performed, which required pulmonary artery transection. No postoperative complications occurred. Postoperative coronary computed tomography scan showed intact coronary arteries and complete aneurysm removal.

Left main coronary artery aneurysm with fistula to superior vena cava: A challenging case.

Coronary artery fistulas (CAFs) are rare congenital coronary artery abnormalities, with direct communication between a coronary artery and a cardiac chamber, great vessel or other structure. We report here, a rare case of a 25-year-old male with CAF from the aneurysmal left main coronary artery to the superior vena cava detected on echocardiography and computerized tomography (CT) coronary angiography.

Giant coronary aneurysm of Behcet's disease with sudden syncope: a case report.

Behcet's disease(BD) is a chronic inflammatory vasculitis that rarely affects the arteries, making myocardial infarction unlikely. We report a 28-year-old patient who was admitted to our hospital with multiple sudden syncope. Cardiovascular risk factors such as hypertension (HT), diabetes and obesity were not found in her. Preoperatively, imaging examinations suggested thrombosis of the inferior and superior vena cava and right heart combined with coronary artery aneurysm. The patient was finally diagnosed with a huge coronary artery aneurysm proximal to the left anterior descending artery. Syncope is considered to be caused right ventricular outflow tract obstruction. The patient received a successful aneurysm resection and had an uneventful postoperative recovery.

Giant right coronary artery aneurysm in a dominant right system.

Giant coronary artery aneurysms (GCAAs) are unusual and extremely rare. Due to their rarity, there is a lack of data on managing GCAAs. A man in his 70s who presented with worsening shortness of breath and bilateral lower extremity oedema was found to have non-ST elevation myocardial infarction. Coronary angiography showed a tortuous Shepherd's crook right coronary artery with ectasia and a gigantic 4.5×4 cm saccular aneurysm in the mid-right coronary artery with limited flow to the distal vasculature. He subsequently underwent aneurysmal clipping and excision with coronary artery bypass grafting. GCAAs are usually silent and diagnosed incidentally but can also present with variable cardiac symptoms. Treatment options include medical management, percutaneous coronary angioplasty and surgery. As per limited available literature, surgical resection has shown favourable outcomes, especially in symptomatic GCAAs. The patient reported significant symptomatic improvement on the follow-up office visit.

ITPKC polymorphism (rs7251246 T > C), coronary artery aneurysms, and thrombosis in patients with Kawasaki disease in a Southern Han Chinese population.

Objectives: Kawasaki disease (KD) is a commonly acquired pediatric systemic vasculitis disease resulting in coronary artery aneurysm (CAA). The relationship between the ITPKC polymorphism (rs7251246) and the severity and susceptibility to KD in the Han Chinese population in Southern China remains unclear. Methods: We enrolled 262 children as controls and 221 children with KD (46 [20.8%] with intravenous immunoglobulin resistance and 82 [37.1%] with CAA). The relationship between the ITPKC rs7251246 polymorphism, KD susceptibility, and CAA formation was investigated. Results: While the ITPKC rs7251246 T>C polymorphism was not significantly associated with KD susceptibility, it was significantly related to the CAA risk in children with KD [CC/CT vs. TT: adjusted odds ratio [OR] 2.089, 95% confidence interval [CI] 1.085-4.020]. Male children with the rs7251246 CT/TT genotype had a significantly lower risk of thrombosis [CT/TT vs. CC: adjusted OR 0.251, 95% CI 0.068-0.923]. Children with KD, especially those with CAA, had significantly downregulated ITPKC mRNA compared to healthy children. ITPKC mRNA levels were lower in children with CAA who developed thrombosis (P=0.039). In children with KD, the CC genotype showed lower mRNA levels of ITPKC (P=0.035). Conclusion: The ITPKC rs7251246 T>C polymorphism may be a risk factor for CAA and thrombosis in children with KD in the Han Chinese population, likely due to differences in mature mRNA levels caused by interference of RNA splicing. Dual antiplatelet therapy for thrombosis is recommended for male children with the rs7251246 CC genotype.

Successful surgical management of a single coronary artery with giant coronary artery aneurysm with fistula to the right ventricle.

A giant coronary artery aneurysm (GCAA) concurrent with coronary artery fistula is a rare condition, and it becomes even more unusual when combined with a single coronary artery (SCA) anomaly. Here, we report such an extremely rare case, who is a 35-year-old woman presenting with severe chest distress. A GCAA with fistula to the right ventricle was noted, occurring in a single coronary artery, diagnosed by multimodality cardiovascular imaging techniques. Both GCAA and coronary artery fistula can cause severe cardiac complications, which jeopardize life. While an SCA is mostly asymptomatic, it may also lead to sudden cardiac death as well. Therefore, surgical intervention was recommended. We chose a novel thrombus-inducing strategy to eliminate the GCAA and repair the fistula. Symptoms were relieved after the surgery, and the patient remained asymptomatic over 8 months of follow-up.

Coronary artery ectasia associated with IgG4-related disease: a case report and literature review.

BACKGROUND: Coronary artery ectasia is defined as a local or diffuse dilatation of the coronary artery more than 1.5 times the diameter of the adjacent normal segment. The etiology of coronary artery ectasia is diverse, and rarely complicated with immunoglobulin G4-related disease (IgG4-related disease). A limited number of cases have been reported, with insidious onset, slow progression but poor prognosis. CASE PRESENTATION: we report a patient with coronary artery ectasia combined with IgG4-related disease. He has been diagnosed with IgG4-related disease 5 years after his first percutaneous coronary intervention (PCI). Despite routine treatment with steroids, he develops a large coronary aneurysm and eventually died. CONCLUSIONS: It is suggested that a thorough evaluation should be performed when coronary artery ectasia is diagnosed. The factors such as manifestations of coronary artery thickening, typical imaging features, other aortas involvement, increased serum IgG4 level, etc. should be considered for early diagnosis of key etiologies.

Brain natriuretic peptide as a clinical screening tool for the diagnosis of Kawasaki disease.

N-terminal pro-brain natriuretic peptide (NT-proBNP) has been studied as a diagnostic screening tool for Kawasaki disease (KD). However, brain natriuretic peptide (BNP) has been less studied while has less variability among age groups. We aimed to find out if BNP can be used as a diagnostic screening tool for KD in Korea. This was a retrospective cohort study performed in a single pediatric emergency department. Patients younger than 19 years of age who presented with fever and underwent BNP examination for suspected KD was included. The primary outcome was the diagnostic performance of BNP for KD, and the secondary outcome was the diagnostic performance of BNP for coronary artery aneurysm (CAA). We also derived a scoring system for predicting KD and CAA. Of the 778 patients who were finally included, 400 were not diagnosed with KD and 378 were diagnosed with KD. The odds ratio of BNP at the cutoff of 30 pg/mL for KD was 7.80 (95% CI, 5.67-10.73) in the univariate analysis and 3.62 (95% CI, 2.33-5.88) in the multivariable analysis. The odds ratio of BNP at the cutoff of 270 pg/mL for CAA was 3.67 (95% CI, 2.18-6.19) in the univariate analysis and 2.37 (95% CI, 1.16-8.74) in the multivariable analysis. The AUC of KD and CAA were 0.884 and 0.726, respectively, which was the highest AUCs among all variables. Additionally, we proposed a scoring system for KD and CAA. It is important to clinically suspect KD and CAA in children with high BNP levels.

[Congenital Left Internal Mammary Artery to Pulmonary Artery Fistula with Aneurysmal Change Diagnosed Incidentally:Report of a Case].

An internal mammary artery to pulmonary artery (IMA-PA) fistula is a very rare vascular abnormality. Patients with this disease are often asymptomatic, but they may develop symptoms such as heart failure and hemoptysis. A 60-year-old woman was incidentally diagnosed with left IMA-PA fistula by chest computed tomography (CT) during an examination for colon cancer. She was asymptomatic, but we determined that surgery was indicated because of the presence of an aneurysmal change. We performed complete surgical resection of the IMA-PA fistula and aneurysm under cardiopulmonary bypass. Her postoperative course was uneventful. Although a specific management strategy for IMA-PA fistula has not yet been established, surgical treatment should be performed to prevent rupture in cases with aneurysmal change.

Sudden cardiac death caused by a right coronary artery aneurysm complicated with acute myocardial infarction: a case report.

Coronary aneurysms are defined as coronary artery ectasia (CAE) more than 1.5 times the normal adjacent segment diameter or the maximum coronary artery diameter. Although most CAE patients are asymptomatic, some patients present with acute coronary syndrome (ACS), such as angina pectoris, myocardial infarction (MI), and even sudden cardiac death. Sudden death due to coronary artery dilatation is very rare. However, we report a case of a patient with aneurysm-like dilatation of both the left and right coronary arteries, with acute inferior ST segment elevation myocardial infarction and sudden death due to third-degree atrioventricular block. After cardiopulmonary resuscitation, the patient underwent emergency coronary intervention. After thrombus aspiration and intracoronary thrombolysis in the right coronary artery, the atrioventricular block returned to normal on the fifth day of hospitalization. Following anticoagulant therapy, coronary angiography was repeated and showed that the thrombus had disappeared. The patient is recovering well after active rescue at the time of writing.

Presence of coronary aneurysms during Kawasaki Disease (KD) correlates with lower levels of autoantibodies to both full form and spliced variant of immune regulator Del-1.

Kawasaki disease (KD), a rare multisystem inflammatory condition that predominantly affects children under six years of age, is the leading cause of childhood-acquired heart disease in developed countries. The pathogenesis is unknown, but studies support that an infectious stimulus triggers an autoimmune reaction in a genetically susceptible child. Recent studies demonstrated an association with autoantibody response to Del-1 (also known as EDIL3) in children with KD. Del-1 is an extracellular matrix protein that is expressed both in macrophages and vascular endothelium. Del-1 has an anti-inflammatory role by preventing leucocyte migration to inflammatory sites. Del-1 has two expression variants and genetic variants of Del-1 have been associated with the risk of intracranial aneurysms. Due to the physiologic plausibility for a role during KD, we chose to assess if autoantibodies against DEL-1 are seen in a larger cohort of children with KD and to assess if responses correlated to aneurysm formation. Contrary to prior findings, in comparison to febrile controls, autoantibodies were not overall higher in children with KD. Elevation in Post-IVIG samples in comparison to pre-IVIG and convalescent samples supports the commonality of anti-Del-1 antibodies. Autoantibodies were notably lower in children with KD who had coronary Z score elevations in comparison to those who did not.

Ruptured mycotic coronary artery aneurysm.

Aneurysms complicated by rupture of the coronary arteries are exceedingly rare. Literature regarding management of mycotic aneurysms resulting in rupture is limited. Therefore, we describe a fascinating diagnosis, imaging progression and management of a ruptured mycotic coronary artery aneurysm.

Identifying Kawasaki Disease With a Low Coronary Artery Aneurysm Risk.

INTRODUCTION: Kawasaki disease (KD) patients with a high risk of coronary artery aneurysm (CAA) development are well characterized and targeted for intensified primary intravenous immunoglobulin (IVIG) treatment. However, the characteristics of KD patients with a low CAA risk are less well-known. METHODS: The present study was a secondary analysis of Prospective Observational study on STRAtified treatment with Immunoglobulin plus Steroid Efficacy for Kawasaki disease (Post RAISE), a multicenter, prospective cohort study of KD patients in Japan. The target of the analysis was patients with a Kobayashi score <5 who were predicted to respond to IVIG. The incidence of CAA during the acute phase, the primary outcome, was assessed based on all echocardiographic evaluations performed between week 1 (days 5-9) and month 1 (days 20-50) after the start of primary treatment. Multivariable logistic regression was used to identify the independent risk factors of CAA during the acute phase, based on which a decision tree was created to identify a subpopulation of patients with KD with a low CAA risk. RESULTS: Multivariate analysis found that a baseline maximum Z score >2.5, age <12 months at fever onset, nonresponsiveness to IVIG, low neutrophils, high platelets and high C-reactive protein were independent predictors of CAA during the acute phase. The decision tree created by using these risk factors identified 679 KD patients who had a low incidence of CAA during the acute phase (4.1%) and no medium or large CAA. CONCLUSIONS: The present study identified a KD subpopulation with a low CAA risk comprising around a quarter of the entire Post RAISE cohort.

Coronary Artery Aneurysms as a Cause of Acute Coronary Syndrome Presentation - A Focused Review.

Coronary artery aneurysms (CAA) are defined as a dilation of a coronary vessel greater than 1.5 times the diameter of a local reference vessel. While CAAs tend to be incidental findings on imaging, they result in complications, such as thrombosis, embolization, ischemia, arrhythmias, and heart failure. Among symptomatic cases, chest pain has been the most common manifestation of CAAs. This necessitates an understanding of CAAs as a cause of acute coronary syndrome (ACS) presentation. However, due to the unclear pathophysiology of CAAs and their variable presentation complicated by similar ACS conditions, there is no clear strategy for CAA management. In this article, we will discuss the contribution of CAAs to ACS presentations and review the current management options for CAAs.