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.
METHODS: 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.

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BACKGROUND: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects infants and young children but is extremely rare in neonates, especially afebrile KD. We present a case of KD without fever in a neonate and review the literature on KD in neonates.
METHODS: A newborn female was hospitalized because her peripheral blood leukocytes increased for half a day. The admission diagnosis was considered neonatal sepsis and bacterial meningitis. She had no fever since the admission, but a rash appeared on her face by the 7th day. On day 11 after admission, there was a desquamation on the distal extremities. On day 15 after admission, ultrasound showed non-suppurative cervical lymphadenopathy. Echocardiogram revealed coronary artery aneurysms in both sides. Finally, the patient was diagnosed with incomplete KD (IKD). The follow-up echocardiogram showed that the internal diameter of both coronary arteries returned to normal three months after birth.
CONCLUSIONS: Fever, rash, and distal extremity desquamation during the recovery phase are the most common symptoms of IKD. When newborns present with clinical manifestations such as rash, distal extremity desquamation and cervical lymph adenitis and with an increased peripheral blood leukocyte count and progressive increase in platelets simultaneously, the medical staff should be highly alert to the possibility of KD even without fever. The echocardiogram needs to be performed promptly. The incidence of coronary artery lesions is significantly higher if neonatal KD patients miss timely diagnosis and treatment.

BACKGROUND: Coronary artery aneurysms (CAAs), coronary arteriovenous malformations (CAVMs), and spontaneous coronary artery dissections (SCADs) are rare clinical entities, and much is unknown about their natural history, prognosis, and management.
METHODS: A systematic search of MEDLINE, Embase, and Cochrane Library databases was performed in March 2023 to identify published papers related to CAAs, CAVMs, and SCADs.
RESULTS: CAAs are found in 0.3% to 12% of patients undergoing angiography and are often associated with coronary atherosclerosis. They are usually asymptomatic but can be complicated by thrombosis in up to 4.8% of patients and rarely by rupture (0.2%). CAAs can be managed medically, percutaneously with stents or coil embolization, and surgically. The most common surgical procedure is ligation of the aneurysm, followed by coronary artery bypass grafting. The incidence of CAVMs is 0.1% to 0.2% in patients undergoing angiography, and they are most likely associated with congenital abnormal development of the coronary vessels. The diagnosis of CAVMs is usually incidental. Surgical or percutaneous intervention is indicated for patients with large CAVMs, which carry a potential risk of myocardial infarction. SCADs represent 1% to 4% of all acute coronary syndromes and typically affect young women. SCADs are strongly correlated with pregnancy, suggesting the role of sex hormones in their pathogenesis. Conservative management of SCAD is preferred for stable patients without signs of ischemia as spontaneous resolution is frequently reported. Unstable patients should undergo revascularization either percutaneously or with coronary artery bypass grafting.
CONCLUSIONS: Further evidence regarding the management of these rare diseases is needed and can ideally be derived from multicenter collaborations.

Coronary periarteritis is a dangerous manifestation of IgG4-related disease, because it forms coronary artery aneurysms, which may cause sudden cardiac death. We report the case of a 78-year-old woman with IgG4-related coronary periarteritis and a coronary aneurysm, which showed progressive enlargement despite maintenance therapy for Type 1 autoimmune pancreatitis. This case was unique, in that coronary periarteritis was the only active lesion that recurred. Low-dose glucocorticoids suppressed the progression of periarterial lesions but led to rapid thinning of the aneurysmal wall and an increase in the size of mural thrombi, which pose a risk of myocardial infarction. Our systematic literature review including 98 cases of 86 articles was performed to examine its treatment strategies and complications. Among the cases in which the effect of immunosuppressive therapy could be followed radiologically, 33 of 37 (89.1%) cases showed improvement in wall thickening/periarterial soft tissue, while 6 of 13 (46.2%) showed worsening increase in the outer diameter of the coronary aneurysms. We propose a draft treatment algorithm and suggest that immunosuppressive therapy for IgG4-related coronary periarteritis with coronary aneurysms should be conducted only after the therapeutic benefit has been determined to outweigh the risks. Because coronary periarteritis can occur without other organ involvement, as in our case, all cases of IgG4-related disease require careful monitoring of coronary artery lesions.

Various factors (e.g., infections) have been postulated to trigger Kawasaki disease (KD) in genetically predisposed individuals. Whether neoplasms can trigger KD is largely unknown due to paucity of data. Herein, we provide a detailed account of KD occurring in temporal proximity (within 6 months) to neoplasms (\'neoplasm-KD\'). Patients with \'neoplasm-KD\' diagnosed/treated at our center from January 1994 to May 2021 were included. Additionally, we performed a systematic literature review (as per PRISMA 2020 guidelines) utilizing PubMed, Web of Science and Scopus databases to retrieve details of all patients with \'neoplasm-KD\' reported till June 2021. Patients with multisystem inflammatory syndrome in children were excluded. As all reports pertained to case description(s), risk of bias assessment was not performed. The details of patients with \'neoplasm-KD\' were analyzed using SPSS software. Primary and secondary outcomes were occurrence of coronary artery abnormalities (CAAs) and clinical characteristics of \'neoplasm-KD\', respectively. A total of 25 patients (data from 18 reports) were included in the \'neoplasm-KD\' dataset. The most frequently diagnosed neoplasm was acute lymphoblastic leukemia followed by neuroblastoma and acute myeloblastic leukemia. Overall, CAAs were noted in 48% of patients. Interval between diagnoses of KD and neoplasm was shorter in patients with CAAs as compared to patients with normal coronary arteries (p-value = 0.03). Besides providing a comprehensive description of \'neoplasm-KD\', this study raises a possibility that neoplasms might trigger KD. Also, \'neoplasm-KD\' may be associated with a higher risk of development of CAAs. However, the small size of \'neoplasm-KD\' dataset precludes definitive conclusions regarding this association. Funding: nil. Registration: PROSPERO (CRD42021270458).
This study is the first exhaustive description of cancers and Kawasaki disease (KD) occurring in close temporal proximity. Nearly half of these patients develop coronary artery abnormalities. In KD, persistent lymphadenopathy, enlargement of liver/spleen and development of low blood cell counts should trigger evaluation for cancer. Our study also raises a possibility that cancers might occasionally trigger KD.

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.
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.
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.

BACKGROUND: Kawasaki disease (KD) is a medium vessel vasculitis, of unknown etiology, typically presenting in children younger than 5 years of age. Prolonged fever (at least five days) is a major clinical criterion of KD, while cardiac involvement may occur in up to 25% of patients, generally in the second week of the disease.
METHODS: We describe the case of KD developing in a 3-month infant, with an early occurrence of coronary artery aneurysm after only 3 days of fever, complicated by thrombosis, requiring aggressive treatments.
CONCLUSIONS: Time of development of cardiac complications can be different in young infants with KD and both diagnostic criteria and treatment indications should be individualized in this class of age.

Acute clinical manifestations of COVID-19 are generally less severe in childhood, however a proportion of them can develop a severe systemic hyperinflammatory syndrome after SARS-CoV-2 infection, known as the multisystem inflammatory syndrome (multisystem inflammatory syndrome in children, MIS-C). Cardiovascular manifestations in MIS-C are frequent (34-82%), including myocardial dysfunction, coronary artery dilation or aneurysms, arrhythmias, conduction abnormalities, pericarditis and valvulitis. The most affected cases can develop cardiogenic shock needing intensive care unit admission, inotropic support and sometimes even mechanical circulatory support. The elevation of myocardial necrosis markers, the frequently transient left ventricular systolic dysfunction and the presence of changes on magnetic resonance imaging, support the hypothesis of an immune-mediated post-viral pathogenesis similar to myocarditis. Although MIS-C shows excellent short-term survival, further studies are needed to demonstrate complete reversibility of residual subclinical heart damage.

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.