Rapid progression of a coronary artery aneurysm caused by IgG4-related disease.

BACKGROUND: IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory disorder that can affect almost any organ. IgG4-RD has also been reported in coronary arteries as periarteritis. IgG4-related coronary periarteritis may cause coronary artery aneurysms, and IgG4-related coronary artery aneurysms (IGCAs) are life-threatening. We describe a case of a patient with IGCA that highlights the usefulness and limitations of various IGCA evaluation modalities and provides insight into disease pathophysiology. CASE SUMMARY: A 60-year-old man with IgG4-RD diagnosed 2 years before and with IGCA at the proximal right coronary artery (RCA) on coronary angiography (CAG) 9 months prior to admission to the hospital presented with acute coronary syndrome. Emergent CAG revealed the rapid progression of IGCA at the RCA, an obstruction of the diagonal branch, and stenosis of the left anterior descending artery (LAD) and the high lateral branch (HL). The patient underwent percutaneous coronary intervention for the diagonal branch. The RCA aneurysm was resected and bypassed with a saphenous vein graft (SVG); coronary bypass grafting (left internal mammary artery to LAD and SVG to HL) was performed. Pathological findings showed inflammatory cell infiltration and disruption of the elastic plate. CONCLUSION: IGCAs require careful follow-up with computed tomography scans for early detection of aneurysmal enlargement.

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.

The relationship between TNF-like protein 1A and coronary artery aneurysms in children with Kawasaki disease.

Kawasaki disease (KD) is an acute, systemic vasculitis of unknown etiology that occurs predominantly in infants and children, and the most crucial complication of KD is coronary artery aneurysm (CAA). Tumor necrosis factor (TNF)-like protein 1A (TL1A) is a member of the TNF superfamily, which possesses the ability of maintaining vascular homeostasis and regulating immune responses. This study aimed to examine serum TL1A levels in KD patients, and to investigate the relationship between TL1A and CAAs in children with KD. Blood samples were recruited from 119 KD patients, 35 febrile controls (FCs), and 37 healthy controls (HCs). The KD group was further divided into KD with CAAs (KD-CAAs) and KD non-CAAs (KD-NCAAs) groups. Serum TL1A levels were measured using enzyme-linked immunosorbent assays, and clinical parameters were collected from KD patients. Serum TL1A levels of KD patients in the acute phase of KD were significantly higher than in the FC and HC groups. In particular, serum TL1A levels were substantially increased in the KD-CAA group compared with the KD-NCAA group. Furthermore, TL1A levels in the KD group were positively correlated with the duration of fever and the time point of IVIG and WBC levels, but negatively correlated with levels of RBC, Hb and albumin. TL1A might be involved in KD-associated vasculitis and in the development of CAAs.

A Large Mediastinal Mass in a Young Woman: A Case of Giant Coronary Artery Aneurysm.

We report the case of a 23-year-old female patient with recurrent episodes of chest and back pain. A cardiac mass of uncertain etiology was discovered and she was referred to our multidisciplinary cardiac tumor team. Pathologic analysis from the surgical specimen confirmed this mass was a giant aneurysm of the left anterior descending artery.

A Pediatric Case Report of Epstein-Barr Virus-associated Hemophagocytic Lymphohistiocytosis With Pericardial Effusion and Multiple Coronary Artery Aneurysms.

Pediatric coronary artery aneurysms (CAAs) are mainly detected in Kawasaki disease and in chronic active Epstein-Barr virus (EBV) infection sometimes, and cardiac complications are rare in viral-associated hemophagocytic lymphohistiocytosis (HLH) patients. Here, we report a pediatric case of EBV-associated HLH with pericardial effusion and multiple CAAs, whereas the patient did not fulfill the diagnostic criteria of Kawasaki disease or chronic active EBV. The case indicates that CAAs may occur in EBV-HLH. Specifically, in a patient with a long-term fever and a high EBV DNA copy number, the detection of cardiac complications may help signal the possible occurrence of HLH, and CAAs may affect the prognosis for high risk of cardiac events.

Frequency of risk factors in patients of acute coronary syndrome due to coronary ectasia.

BACKGROUND: Coronary artery ectasia is a relatively common entity characterized by inappropriate dilatation of the coronary vasculature. In some cases of acute coronary syndrome without obstructive coronary lesions, coronary ectasia is the sole cause. The exact mechanism of its development is unknown but evidence suggests a combination of genetic predisposition, common risk factors for coronary artery disease, and abnormal vessel wall metabolism. As there are few data regarding the pattern of coronary risk factors in patients with coronary ectasia, the objective of the study was to determine the frequency and distribution of coronary risk factors in patients with acute coronary syndrome solely due to coronary ectasia. METHODS: The study included 155 patients over a period of 6 months, with coronary angiographic evidence of coronary ectasia as the sole cause of acute coronary syndrome. There were 79 (51%) men and 76 (49%) women with a mean age 51.92 ± 7.83 years; 73 (47.10%) were aged 20-50 years and 82 (52.90%) were 51-80 years of age. The frequencies of coronary risk factors were stratified according to sex and the two age groups. RESULTS: Seventy-one patients (45.80%) had diabetes mellitus, 83 (53.54%) had hypertension, 55 (35.48%) were smokers, 46 (29.68%) had dyslipidemia, and 47 (30.3%) were obese. CONCLUSION: Hypertension is the leading coronary risk factors in patients with acute coronary syndrome solely due to coronary ectasia, followed by diabetes mellitus and smoking.

An unknown mass on the cardiac surface with huge coronary aneurysm and fistula: A case report.

Cardiac mass is rare in the clinic and can be primary or secondary. It is quite rare to find a mass only on the cardiac surface. Today we report a patient with a cardiac mass grown on the cardiac surface and also had a coronary aneurysm combined a coronary fistula, pathology examination showed that the mass was not a tumor but an aneurysm with thrombosis. This is the first time that a primary thrombus discovered on the surface of the heart.

Is KCNH1 mutation related to coronary artery ectasia.

BACKGROUND: Coronary artery ectasia (CAE) is an uncommon finding in patients undergoing coronary angiography and acute myocardial infarction is an extremely uncommon condition in the presence of coronary artery ectasia. To date, 50 gene variants associated with coronary artery disease have been identified, but none appear to be related to coronary artery ectasia. CASE PRESENTATION: This is a rare case of Coronary artery ectasia which is considered to be related to Gene variations in potassium voltage-gated channel subfamily H member 1, KCNH1 (encoding a protein designated ether à go-go, EAG1 or KV10.1). CONCLUSION: Occurrence of Acute myocardial infarction in patient with coronary artery ectasia after diarrhea is a very rare condition and involvement of KCNH1 gene mutation which is described in this case report.

Interleukin-6 is prone to be a candidate biomarker for predicting incomplete and IVIG nonresponsive Kawasaki disease rather than coronary artery aneurysm.

Kawasaki disease (KD) is an acute, systemic vasculitis and occurs mainly in childhood. Interleukin-6 (IL-6) is a pleiotropic cytokine synthesized predominantly by neutrophils and monocytes/macrophages and plays an important role in systemic inflammatory disease. However, a little information is currently available on the relationship of serum IL-6 with conventional inflammatory mediators, clinical classification, IVIG response and coronary artery aneurysm (CAA). 165 Chinese children with KD were enrolled and divided into six subgroups, including complete KD, incomplete KD, IVIG-responsive KD, IVIG-nonresponsive KD, coronary artery noninvolvement KD and coronary artery involvement KD. Blood samples were collected from all subjects within 24-h pre- and 48-h post-IVIG therapy, respectively. Serum IL-6 and conventional inflammatory mediators were detected. (1) Serum IL-6 markedly increased in the acute phase of KD, whereas declined to normal after IVIG therapy; it was positively correlated with C-reactive protein and erythrocyte sedimentation rate. (2) Serum IL-6 was significantly elevated in patients with incomplete KD when compared with their complete counterparts. The area under receiver operating characteristic curve (AUC) value for serum IL-6 in prediction of incomplete KD was 0.596, and the estimated sensitivity and specificity were 77.80% and 54.40% with a cutoff of IL-6 > 13.25 pg/ml, respectively. (3) Serum IL-6 was significantly elevated in patients with IVIG-nonresponsive KD when compared with their IVIG-responsive counterparts; the AUC value for serum IL-6 in prediction of IVIG-nonresponsive KD was 0.580, and the estimated sensitivity and specificity were 60.00% and 66.30% with a cutoff of IL-6 > 26.40 pg/ml, respectively. (4) No significant differences in IL-6 were found between KD patients with and without CAA. IL-6 is prone to be a candidate biomarker for predicting incomplete and IVIG nonresponsive KD rather than CAA.

Histopathological aspects of cardiovascular lesions in Kawasaki disease.

Kawasaki disease (KD) is the commonest vasculitic syndrome. It affects medium-sized arteries, principally the coronary arteries. Histologically, coronary arteritis begins at 6 to 8 days after the onset of KD and the inflammation rapidly involves all layers of the artery. This results in severe damage to the structural components of the artery leading to arterial dilation. The inflammatory infiltrate in KD arteritis is characterized predominantly by infiltration of monocytes and macrophages. Activated neutrophils, monocytes and macrophages are believed to be involved in the initial stage of coronary arteritis. Inflammatory cell infiltration may continue for up to 25 days of disease following which the inflammatory cells gradually decline in number. Inflammatory lesions in the arteries are relatively synchronous as they evolve from an acute to the chronic stage. If a giant aneurysm remains or vessel recanalization occurs after thrombotic occlusion of an aneurysm, the remodeling of vascular structures may continue for a much longer time.