A case of Behçet syndrome presenting with acute coronary syndrome.

Cardiac involvement (CI) is rare in Behçet syndrome (BS), but the important point is that CI may be the first manifestation of the disease. The presence of CI worsens the prognosis of BS, so early diagnosis and early initiation of immunosuppressive treatment (IST) are vital. Coronary aneurysm may develop spontaneously in these patients, or any vascular intervention may cause aneurysm with a pathergy-like reaction. The risk of restenosis is high after percutaneous coronary intervention or coronary artery bypass surgery applied without IST. Therefore, it should be kept in mind that IST constitutes the main step of treatment. Herein, we present a young male diagnosed with BS after acute coronary syndrome caused by coronary artery aneurysms and thrombosis.

Coronary artery bypass grafting in adolescent with myocardial infarction complicating undiagnosed Kawasaki disease.

Although Kawasaki disease is often self-limiting, significant cardiovascular sequelae may occur in the acute or late stage. The most common late complication is persistent coronary artery aneurysm, which can lead to myocardial ischaemia and even myocardial infarction. We report a case of coronary artery bypass grafting in a 16-year-old boy with a history of undiagnosed Kawasaki disease. Increased awareness of Kawasaki disease, especially among children between the ages of 6 months and 5 years, can increase early treatment and prevent serious complications that may occur in the future.

Pediatric Thromboprophylaxis of Large Coronary Artery Aneurysm Using Rivaroxaban.

Giant or large coronary artery aneurysms (CAA) are rare in children, most often secondary to Kawasaki disease, and anticoagulation is recommended to prevent thromboembolism. There are no published pediatric reports on the use of a direct oral anticoagulant for this indication. We describe the anticoagulation management of an 8-year-old boy with a dilated right CAA secondary to Kawasaki disease that has remained stable on rivaroxaban and aspirin, following bleeding complications on enoxaparin and challenges on warfarin. The use of rivaroxaban appears to be safe and effective in the prevention of thrombosis in a pediatric patient with CAA.

French national diagnostic and care protocol for Kawasaki disease.

Kawasaki disease (KD) is an acute vasculitis with a particular tropism for the coronary arteries. KD mainly affects male children between 6 months and 5 years of age. The diagnosis is clinical, based on the international American Heart Association criteria. It should be systematically considered in children with a fever, either of 5 days or more, or of 3 days if all other criteria are present. It is important to note that most children present with marked irritability and may have digestive signs. Although the biological inflammatory response is not specific, it is of great value for the diagnosis. Because of the difficulty of recognising incomplete or atypical forms of KD, and the need for urgent treatment, the child should be referred to a paediatric hospital as soon as the diagnosis is suspected. In the event of signs of heart failure (pallor, tachycardia, polypnea, sweating, hepatomegaly, unstable blood pressure), medical transfer to an intensive care unit (ICU) is essential. The standard treatment is an infusion of IVIG combined with aspirin (before 10 days of fever, and for a minimum of 6 weeks), which reduces the risk of coronary aneurysms. In case of coronary involvement, antiplatelet therapy can be maintained for life. In case of a giant aneurysm, anticoagulant treatment is added to the antiplatelet agent. The prognosis of KD is generally good and most children recover without sequelae. The prognosis in children with initial coronary involvement depends on the progression of the cardiac anomalies, which are monitored during careful specialised cardiological follow-up.

Coronary Artery Aneurysms: Comprehensive Review and a Case Report of a Left Main Coronary Artery Aneurysm.

Coronary artery aneurysms (CAAs) are rare anatomical disorders of the coronary arteries. Atherosclerosis and Kawasaki disease are the principal causes of CCAs, while other causes including genetic factors, inflammatory arterial diseases, connective tissue disorders, endothelial damage after cocaine use, iatrogenic complications after interventions and infections, are also common among patients with CAAs. Although there is a variety of noninvasive methods including echocardiography, computed tomography, and magnetic resonance imaging, coronary angiography remains the gold standard diagnostic method. There is still no consensus about the most appropriate therapeutic strategy. Medical therapy including antiplatelets, anticoagulants, statins and ACEs are preferred either in patients with atherosclerosis, inflammatory status and stable CAAs, while percutaneous or surgery interventions are usually applied in patients with acute coronary syndrome due to a CAA culprit, obstructive coronary artery disease or large saccular aneurysms at a high risk of rupturing.

Growing Coronary Aneurysm Secondary to Coronary Fistula Despite Decreased Pulmonary Blood Flow/Systemic Blood Flow Ratio in a Child: A Case Report.

Coronary aneurysm secondary to coronary fistula is a rare condition, with no existing report on its pathological examination. We report the case of a patient diagnosed with a right coronary artery fistula with coronary aneurysm during the fetal period. During follow-up after delivery, the aneurysm became larger, even though the shunt size decreased. We were afraid the aneurysm would rupture and therefore, planned elective catheter embolization. At the age of 4 years, the patient underwent surgery, which involved closing the fistula and making the lumen of the aneurysm smaller. However, the surgery was not catheter embolization as planned because segment 3 branched off from the largest aneurysm where we planned to embolize. Pathologically, the structure of the coronary artery differed from that of a healthy one, with thickened intima and media, fewer scattered smooth muscle cells, widely distributed elastic fibers, and mucoid degeneration in the media. The structure of the coronary artery suggested that the vessel wall was weak and that the aneurysm would rupture if not treated. Postoperative coronary angiography showed that segment 2 was obstructed, while the collaterals from the left coronary artery perfused the area. We could have treated the fistula with a catheter as scheduled.

Coronavirus disease 2019-related Kawasaki syndrome: a case report.

BACKGROUND: Coronavirus disease 2019 has changed the pattern of some diseases in the world, especially in pediatrics. Despite data suggesting that the pediatric population is less affected by coronavirus disease-19 infection, new concerns have been raised owing to reported cases with hyperinflammatory conditions such as Kawasaki disease. CASE PRESENTATION: We report herein the case of a pediatric patient diagnosed and treated for classic Kawasaki disease in the setting of confirmed coronavirus disease 2019 infection. She was an 8-year-old, previously healthy, and fully immunized Iranian girl who initially presented to the pediatric emergency department with 5 days of intermittent fever, followed by abdominal pain, nausea, and vomiting. She was admitted for fever and abdominal pain to the surgery service of Akbar Hospital with suspected appendicitis. CONCLUSIONS: This case report may serve as a useful reference to other clinicians caring for pediatric patients affected by coronavirus disease 2019 infection. Standard therapeutic interventions for Kawasaki disease must be performed to prevent critical coronary aneurysm-related complications in the coronavirus disease 2019 era.

An autopsy case of hyperimmunoglobulin E syndrome with coronary fibrinoid necrotizing arteritis and an aneurysm.

We report a case of hyperimmunoglobulin (Ig) E syndrome (HIES) with a coronary artery aneurysm (CAA) in a 25-year-old Japanese man. He died suddenly due to chronic heart failure associated with HIES. We noted a CAA at the trunk of the left coronary artery and granulomatous and fibrinoid necrotizing arteritis of the middle portion of the left anterior descending during the autopsy. We speculate herein on the relationship between the aneurysm and arteritis. These findings facilitate a better understanding of the pathogenesis underlying HIES.

Coronary Revascularization in Patients With Cardiac Sequelae of Kawasaki Disease at a Single Center.

Background: Kawasaki disease (KD) is a self-limited vasculitis that mainly affects infants and preschool-age children, characterized by coronary aneurysms and/or stenoses that may lead to the occurrence of ischemia, heart attack, and sudden death. This study aimed to evaluate the clinical and surgical results of pediatric patients with KD and cardiac sequelae who underwent coronary artery bypass grafting. Methods: A retrospective study was carried out. Patients with a diagnosis of KD and cardiac sequelae who underwent coronary artery bypass grafting from January 1, 2004, through March 31, 2021, were included: preoperative characteristics and conditions. Results: Ten patients were included, with a mean age of 6.4 ± 3.7 years; 80% males. Seven (70%) had the compromise of 2 coronary arteries, the most affected being the left anterior descendent artery (36.9%) with aneurysm and the right coronary artery (36.8%) with stenosis. 40% had preoperative moderate-to-severe ventricular dysfunction. The graft most frequently used was the right internal thoracic artery (47.6%), and the most frequent coronary bypass target vessel was the left anterior descending artery (38.1%). There was no early mortality or deaths during follow-up; 90% remained in functional class I. Among the cohort as a group, there was an overall improvement in postoperative ventricular function (P = .03). This persisted, being unchanged at subsequent follow-up (P = .95). Conclusions: Coronary artery bypass grafting is an appropriate treatment option for children with cardiac sequelae of KD, with excellent surgical results. Furthermore, this surgical procedure avoids the deterioration of left ventricular function in pediatric age.

[Transient Left Ventricular Dysfunction after Giant Coronary Aneurysm Resection and Repair of Coronary Arteriovenous Fistula with Coronary Artery Bypass Grafting:Report of a Case].

Coronary artery aneurysm with coronary arteriovenous fistula is a relatively rare clinical setting. We report a surgical case of a 69-year-old male with a giant coronary artery aneurysm, finding coronary arteriovenous fistula on computed tomography (CT). We performed complete aneurysm excision and coronary artery bypass grafting with the left internal thoracic artery to the posterolateral branch. The fistula was located between the giant aneurysm on the circumflex artery and the coronary vein close to the coronary sinus, closed with aneurysm sac. The postoperative CT found no residual aneurysm and fistula. However, the great cardiac vein was thrombosed, and the impeded venous flow by the thrombus seemed to reduce the left ventricular ejection fraction (LVEF). Four months after the operation, the LVEF improved to the preoperative level.

Etiology, clinical presentation, and management of left main coronary artery aneurysms.

BACKGROUND AND AIM OF THE STUDY: The widespread use of noninvasive/invasive coronary imaging increased the probability of recognition of coronary aneurysms. Left main coronary aneurysms (LMCA), though rare, are potentially life-threatening but in the absence of controlled studies, guidelines do not provide any specific recommendation for their management. We, therefore, aimed to investigate the epidemiology, clinical presentation, therapeutic strategies, and prognostic implication of LMCA. METHODS: A systematic review of the literature was performed to retrieve all the reported cases of LMCA as of December 2021, which were summarized and classified according to their etiology, clinical presentation, and therapeutic management. RESULTS: Out of 1997 works retrieved, 180 studies were analyzed, describing 209 LMCA cases (aged 51 ± 19 years, 68% males). Atherosclerosis was the most common etiology (40%), followed by inflammatory (12%), congenital (9%), or degenerative (6%) conditions. Stable angina (43%) and acute coronary syndromes (32%) were more often the first clinical manifestations, while 29 (14%) LMCA were incidental findings. Most cases were treated surgically (53%), while percutaneous intervention was rarely adopted (7%). Data about antithrombotic therapies were scarce and heterogeneous. Finally, when longitudinal data were reported (n = 81), LMCA resulted associated with a severe prognosis, with a 15% mortality over an 8-month median follow-up. CONCLUSIONS: LMCA are most frequently, but not exclusively, caused by advanced atherosclerosis. Irrespective of their etiology and clinical presentation, LMCA may be associated with high short-term mortality. In absence of controlled studies, a careful evaluation of each case is warranted to optimize therapeutic strategies.

Histological studies shed new light on the initiation and characteristics of calcification of coronary artery aneurysms in Kawasaki disease.

BACKGROUND: Calcification of coronary artery aneurysms (CAAs) is common in the remote phase of Kawasaki disease (KD), but the detailed features of its development remain unclear. This study aimed to elucidate the histological characteristics of calcification in KD CAAs. MATERIALS AND METHODS: The study materials consisted of 24 coronary artery branches with aneurysms that were obtained from 14 Japanese patients who died during the period from 40 days to 3 years after the onset of KD. We first examined the CAAs for the presence of thrombi and calcification. When calcifications were observed, we determined their location and shape, and investigated the time-course of the changes based on the time-interval from KD onset until death. Then we measured the area of each calcification and examined for correlations between the calcified area, and (1) the disease duration, and (2) the aneurysm diameter. RESULTS: Calcification was observed in 14 of 24 CAAs (in 7 of 13 LCA and 7 of 11 RCA). Thrombi were also seen in 13 of 14 CAAs with calcification. Calcification showed two localizations: in the organized portion of the thrombus (seen in 12 CAAs) and deep in the thickened tunica of the intima (3 CAAs). The earliest observation of calcification was in an infant who died on the 49th disease day: it was a small, band-shaped calcified lesion in granulation tissue that had formed at the boundary between the thrombus and the blood vessel wall. As the duration of KD increased, the calcified lesion increased in size, and nodular shapes were formed. Moreover, the calcified area tended to increase as the diameter of the aneurysm increased. CONCLUSION: Histologically, CAA calcification starts early in the remote phase of KD, and it is closely related to organization of thrombi.

Multiple Giant Coronary Artery Aneurysms in a Pediatric Patient with Granulomatosis with Polyangiitis.

Granulomatosis with polyangiitis (GPA) is characterized by necrotizing vasculitis of small and medium sized vessels and is rarely present in the pediatric population. Cardiac manifestations in pediatric patients with GPA are extremely uncommon, with only two known reported cases associated with coronary artery aneurysms (Rehani and Nelson in Pediatrics 147:e20200932, 2021, https://doi.org/10.1542/peds.2020-0932 ;Aghaei Moghadam et al. in Case Rep Cardiol 2020:3417910, 2020, https://doi.org/10.1155/2020/3417910 ). We report a case of a 14-year-old male who presented with a 1 month history of fatigue and shortness of breath. He ultimately was found to have multiple giant coronary aneurysms in both the left and right coronaries including a giant aneurysm in the posterior descending; this has not been previously reported. The case highlights the need for complete multi-modality imaging of the coronary arteries in patients with GPA.

Silent Kawasaki Disease Affecting Multiple Coronary Arteries in a 39-Year-Old Egyptian Woman.

Kawasaki disease, an acute febrile illness, can cause vasculitis in the coronary arteries. It is the chief acquired cause of myocardial infarction and sudden cardiac death in infants, children, and young adults in developed countries. We report a case of chronic, silent Kawasaki disease complicated by multivessel thrombosis in a 39-year-old Egyptian woman. The patient presented with progressive, unstable angina but was otherwise asymptomatic and at negligible risk of ischemic heart disease. Coronary angiograms showed critical arterial stenosis with multiple aneurysms. During revascularization surgery, the patient's harvested left internal mammary artery was found to have occlusive lesions and aneurysmal areas that made it unfit for bypass grafting, and subsequent histopathologic examination revealed features characteristic of chronic Kawasaki disease-associated systemic vasculitis. We think that this is only the second report of Kawasaki disease in the Arabian Mediterranean region. In addition to the patient's case, we discuss the epidemiology and management of Kawasaki disease, in hopes of increasing clinicians' awareness.

Coronary Artery Aneurysms in ST-Elevation Myocardial Infarction (From a United States Based National Cohort).

This study aimed to study group differences in patients presenting with ST-elevation myocardial infarction (STEMI) based on the presence or absence of associated coronary artery aneurysms (CAA). The cause-and-effect relationship between CAAs and STEMI is largely unknown. The Nationwide Readmission database was used to identify and study group differences of patients with STEMI and with and without CAA from 2014 to 2018. The primary outcome in the 2 groups was mortality. Secondary outcomes in the 2 groups included differences in clinical outcomes, cardiovascular interventions performed, and prevalence of coronary artery dissection. The total number of patients with STEMI included was 1,038,299. In this sample, 1,543 (0.15%) had CAA. Compared with those without CAA, patients with CAAs and STEMI were younger (62.6 vs 65.4), more likely to be male (78 vs 66%), and had a higher prevalence of a history of Kawasaki disease (2.5 vs 0.01%). A difference exists in the prevalence of coronary dissection in patients with STEMI with and without CAA (73% vs 1%). Patients with CAA were more often treated with coronary artery bypass grafting (13.1 vs 5.6%), thrombectomy (16.5 vs 6%), and bare-metal stent implantation (8 vs 4.4). Patients in the CAA STEMI group had lower all-cause mortality (6.3 vs 11.7%). In conclusion, there are important differences in patients with STEMI with and without CAA, which include, but are not limited to, factors such as patient profile, the risk for coronary dissection, treatment, outcomes, and mortality.

[Successful Surgical Repair of Left Coronary Ostial Aneurysm after Modified Bentall Procedure Using the Carrel Patch Technique].

A 38-year-old woman underwent aortic root surgery using the Carrel patch technique at the age of 14 years for annuloaortic ectasia of 59 mm. Although there were no clinical findings of Marfan syndrome or bicuspid aortic valve, the pathological findings of the aortic aneurysmal wall showed degeneration of the media. After 24 years, contrast-enhanced computed tomography (CT) showed an enlargement of the left coronary ostial aneurysm of 17 mm with saccular formation. Re-coronary reconstruction with the Piehler technique using an 8 mm Dacron graft was performed. The post-operative course was uneventful, and post-operative CT showed no pseudoaneurysm or stenosis at the anastomosis sites. The Carrel patch coronary ostial reconstruction has been shown to reduce coronary anastomotic pseudoaneurysms and to improve aortic root surgical outcomes. However, coronary ostial aneurysm is a true aneurysm and one of the later complications after the modified Bentall procedure using the Carrel patch technique. Although it is common in Marfan syndrome, the consensus on diagnosis, operative indication, and surgical procedure have not yet been established. Not only in Marfan syndrome, but also after coronary artery reconstruction using the Carrel patch technique, longer-term follow-up is necessary to take care for aneurysmal formation at coronary ostium.