Anomalous Coronary Artery Variant of Common Origin from Right Coronary Cusp: A Case Report.

Coronary artery anomalies are rare congenital variants of coronary artery anatomy accounting second most common cause of sudden cardiac death in young competitive athletes. A single ostium coronary artery anomalous is an extremely rare variant with an incidence of less than 0.004%. They may present as chest pain, arrhythmia, or sudden death. Recently, advanced imaging techniques such as computed tomography and magnetic resonance imaging coronary angiography are becoming the alternatives investigation for diagnosis. We reported a rare case of 50 years old lady who presented with acute chest pain with normal electrocardiography, echocardiography, and cardiac markers. Coronary Computed tomography angiography revealed anomalous coronary artery anatomy with both right and left coronary artery arising from the large common trunk of the right coronary cusp, left main coronary artery having trans-septal course, there was no flow-limiting coronary artery disease. She was medically managed with a single antiplatelet, beta-blocker, and statin therapy.

The value of plasma fibrillin-1 level in patients with spontaneous coronary artery dissection.

BACKGROUND: Spontaneous coronary artery dissection (SCAD) has emerged as an important etiology of myocardial infarction and sudden death, especially in young women. Early diagnosis is essential for appropriate management. OBJECTIVES: To explore the value of plasma fibrillin-1 (FBN1) levels in patients with SCAD. METHODS: 70 patients with non-atherosclerotic SCAD between January 2014 and September 2018 were age and sex matched with 70 patients with non-SCAD acute coronary syndrome (ACS) and 70 healthy controls. The plasma FBN1 level was measured and compared among three groups. The value of FBN1 for prognosis and treatment decision making was further explored. RESULTS: The plasma FBN1 level of SCAD group (58.44 ± 7.06 ng/mL) was higher than that of non-SCAD ACS group (52.39 ± 6.92 ng/mL, P < 0.001) or healthy controls (50.56 ± 4.48 ng/mL, P < 0.001). Compared with controls, significantly higher percentages of patients with SCAD were found in the highest compared with lowest quartile of FBN1 concentration. The area under the curve (AUC) for plasma FBN1 level to discriminate patients with SCAD from non-SCAD ACS was 0.81 (95% CI 0.74-0.88, P < 0.001). A cut-off value of 54.64 ng/mL was determined to differentiate SCAD from non-SCAD ACS with a sensitivity of 0.77 (95%CI: 0.66-0.86) and specificity of 0.76 (95%CI: 0.64-0.85). After a median follow-up of 28.35 (14.07 ± 44.69) months, 11 (15.7%) cases suffered from major adverse cardiac events (MACE). Higher FBN1 level was detected in patients with MACE (63.71 ± 7.49 vs. 57.45 ± 6.58 ng/mL) (P = 0.006). A cut-point of 58.14 was determined for SCAD patients to identify MACE. At this point, FBN1 might also have potential use for decision making in SCAD patients. CONCLUSION: Plasma FBN1 is a promising biomarker for aiding the diagnosis of SCAD and have potential value in prognosis prediction.

Does Mean Platelet Volume Decrease in the presence of Coronary Artery Fistula?

BACKGROUND: Coronary artery fistula (CAF) is an abnormal connection that links a coronary artery to a cardiac chamber or another major blood vessel. Several studies have shown the association between mean platelet volume (MPV) and cardiovascular diseases. In the literature, there is no previous study about the association between hematologic parameters and congenital CAF. For this reason, we aimed to investigate the association of MPV with CAF. METHODS: 70 patients with normal coronary arteries and 50 with coronary artery fistulas were included. Routine blood and biochemical parameters were measured before the arteriography. Differences between groups for continuous variables were analyzed with t- test or Mann-Whitney test. P values < 0.05 were considered significant. Regression analysis was used to find independent predictors of CAF. RESULTS: Baseline patient demographics, including age and clinical risk factors, were similar between the groups. Compared to the control group, median (IQR) High-density lipoprotein cholesterol (HDL) levels were significantly higher (p=0.04) and MPV levels were significantly lower in the CAF group (8.84 ± 1.71fL vs. 10.43 ± 1.34, p < 0.001). In the multivariate analysis, only MPV was a significant predictor of CAF (p < 0.001, 95% CI for OR: 0.438 (0.306-0.629). A negative correlation was found between MPV and fistulae in Pearson's correlation test (r: -0.454, p < 0.001). An MPV level of < 9,6 fL showed sensitivity, specificity, positive predictive value and negative predictive value of 80%, 68%, 71% and 78% respectively (AUC = 0.766, 95% CI, 0.678-0.854) for the prediction of CAF. CONCLUSION: The present study suggests that MPV may decrease in patients with CAF.

Does Mean Platelet Volume Decrease in the presence of Coronary Artery Fistula? / O Volume Médio Plaquetário Diminui na Presença de Fístula da Artéria Coronária?

Abstract Background: Coronary artery fistula (CAF) is an abnormal connection that links a coronary artery to a cardiac chamber or another major blood vessel. Several studies have shown the association between mean platelet volume (MPV) and cardiovascular diseases. In the literature, there is no previous study about the association between hematologic parameters and congenital CAF. For this reason, we aimed to investigate the association of MPV with CAF. Methods: 70 patients with normal coronary arteries and 50 with coronary artery fistulas were included. Routine blood and biochemical parameters were measured before the arteriography. Differences between groups for continuous variables were analyzed with t- test or Mann-Whitney test. P values < 0.05 were considered significant. Regression analysis was used to find independent predictors of CAF. Results: Baseline patient demographics, including age and clinical risk factors, were similar between the groups. Compared to the control group, median (IQR) High-density lipoprotein cholesterol (HDL) levels were significantly higher (p=0.04) and MPV levels were significantly lower in the CAF group (8.84 ± 1.71fL vs. 10.43 ± 1.34, p < 0.001). In the multivariate analysis, only MPV was a significant predictor of CAF (p < 0.001, 95% CI for OR: 0.438 (0.306-0.629). A negative correlation was found between MPV and fistulae in Pearson's correlation test (r: -0.454, p < 0.001). An MPV level of < 9,6 fL showed sensitivity, specificity, positive predictive value and negative predictive value of 80%, 68%, 71% and 78% respectively (AUC = 0.766, 95% CI, 0.678-0.854) for the prediction of CAF. Conclusion: The present study suggests that MPV may decrease in patients with CAF.

Resumo Fundamento: A fístula da artéria coronária (FAC) é uma conexão anormal que liga a artéria coronária a uma câmara cardíaca ou outro importante vaso sanguíneo. Vários estudos mostraram a associação entre o volume plaquetário médio (VPM) e as doenças cardiovasculares. Na literatura, não há estudo prévio sobre a associação entre os parâmetros hematológicos e a FAC congênita. Por essa razão, nosso objetivo foi investigar a relação do VPM com a FAC. Métodos: Foram incluídos 70 pacientes com artérias coronárias normais e 50 com fístulas de artérias coronárias. Os parâmetros sanguíneos e bioquímicos de rotina foram medidos antes da arteriografia. As diferenças entre os grupos para as variáveis contínuas foram analisadas com o teste t ou teste de Mann-Whitney. Valores de p < 0,05 foram considerados significativos. A análise de regressão foi utilizada para encontrar preditores independentes de FAC. Resultados: Os dados demográficos basais dos pacientes, incluindo idade e fatores de risco clínicos, foram semelhantes entre os grupos. Comparados à mediana do grupo controle (IIQ), os níveis de HDL-colesterol foram significativamente mais altos (p = 0,04) e os níveis de VPM foram significativamente mais baixos no grupo FAC (8,84 ± 1,71fL vs. 10,43 ± 1,34, p < 0,001). Na análise multivariada, apenas o VPM foi um preditor significativo de FAC (p<0,001, IC 95% para OR: 0,438 (0,306-0,629)). Foi encontrada uma correlação negativa entre o VPM e fístulas no teste de correlação de Pearson (r: -0,454, p < 0,001). Um nível de VPM < 9,6 fL apresentou sensibilidade, especificidade, valor preditivo positivo e valor preditivo negativo de 80%, 68%, 71% e 78%, respectivamente (AUC = 0,766, IC 95%, 0,678-0,854) para a previsão de FAC. Conclusão: O presente estudo sugere que o VPM pode diminuir no paciente com FAC.

Eosinophilic inflammation in spontaneous coronary artery dissection: A potential therapeutic target?

Spontaneous coronary artery dissection (SCAD), defined as non-traumatic, non-iatrogenic dissociation of coronary vessel wall resulting from intimal disruption or intramural hemorrhage, represents an important cause of sudden death and myocardial infarction among young or middle-aged women without conventional risk factors for atherosclerosis. On histopathological examination, SCAD is featured by prominent eosinophilic infiltration of the adventitia or periadventitial layer of coronary artery. It has been estimated that approximately 15-30% of SCAD patients experience recurrent episodes of dissection despite medical therapy. Preliminary evidence suggests that injury to the vascular endothelium and myocytes in the arterial wall may be explained by cytotoxic products released from eosinophils in response to inflammatory mediators. In addition, neovascularization of vasa vasorum and dilatation of intimal capillaries may be stimulated by localized eosinophils. Newly formed fragile vasa vasorum may disrupt due to high intraluminal pressure from the interconnected capillary network, leading to the expansion of intramural hemorrhage. It is hypothesized that anti-inflammatory therapy targeting eosinophilic coronary periarteritis would be effective in preventing the recurrence of SCAD by promoting the healing of dissection. The article delineates the biological plausibility, empirical data, and future perspective regarding eosinophilic inflammation as a potential therapeutic target for SCAD.

Familial spontaneous coronary artery dissection: evidence for genetic susceptibility.

IMPORTANCE: Spontaneous coronary artery dissection (SCAD) is a major cause of acute coronary syndrome in young women, especially among those without traditional cardiovascular risk factors. Prior efforts to study SCAD have been hampered by underrecognition and lack of registry-based studies. Risk factors and pathogenesis remain largely undefined, and inheritability has not been reported. OBSERVATIONS: Using novel research methods, patient champions, and social media, the Mayo Clinic SCAD Registry has been able to better characterize this condition, which was previously considered rare. Of 412 patient enrollees, we identified 5 familial cases of SCAD comprising affected mother-daughter, identical twin sister, sister, aunt-niece, and first-cousin pairs, implicating both recessive and dominant modes of inheritance. The mother-daughter pair also reported fatal myocardial infarction in 3 maternal relatives. None of the participants had other potential risk factors for SCAD, including connective tissue disorders or peripartum status. CONCLUSIONS AND RELEVANCE: To our knowledge, this series is the first to identify a familial association in SCAD suggesting a genetic predisposition. Recognition of SCAD as a heritable disorder has implications for at-risk family members and furthers our understanding of the pathogenesis of this complex disease. Whole-exome sequencing provides a unique opportunity to identify the molecular underpinnings of SCAD susceptibility.

Correlation of triglyceride level with acute coronary syndrome.

The study was aimed to find out the correlation of serum triglyceride level with acute coronary syndrome. This cross sectional study was conducted in the department of cardiology, Mymensingh Medical College Hospital, from August 2009 to May 2010. Socio-demographic characteristics, smoking habit, hypertension, serum total cholesterol level, serum HDLc, Serum LDLc, TG level were important variable considered. A total number of 100 respondents consisted of 50 cases (patient) and 50 healthy persons (control). Investigations included ECG, cardiac enzyme (troponin I), FBS and lipid profile. The data were analyzed by computer with the help of SPSS. Chi-square Test, T-test & ANOVA test were used as test of significance. The mean level of TG in acute coronary syndrome (ACS) patients (cases) was 168.2±58.0 mg/dl and in control were 141.2±45.3 mg/dl. So serum TG level is significantly higher in patients with ACS (p=0.01). In multivariate regression analysis, there was a significant association of elevated TG with risk of ACS (relative risk) is the highest, compared with the lowest quarantile = 1.011; 95% confidence interval (CI = 1.002 - 1.020; P for trend = 0.01). The relation of TG level to HDLc was a strong predictor of ACS (RR in the highest) compared with lowest quarantile = 0.02; (95% CI = 0.003 - 0.173; P for trend <0.0001). The study revealed that high level of serum triglyceride is associated with ACS. Categorization of patients with ACS on the basis of TG level may be helpful for risk stratification and management.

Asymmetric dimethylarginine levels in patients with coronary artery ectasia.

BACKGROUND: Endothelial dysfunction might be one of the pathophysiological mechanisms in the development of coronary artery ectasia (CAE) although the exact mechanisms have not yet been demonstrated. Asymmetric dimethylarginine (ADMA), an endogenous competitive inhibitor of nitric oxide synthase, is also related to endothelial and structural dysfunction. AIM: To asses the relationship between CAE and ADMA plasma concentrations. METHODS: Thirty patients with CAE in a mean age of 55.5 +/- 3.6 years and 40 patients with normal coronary arteries in a mean age of 53.3 +/- 11.6 years were studied. The ADMA levels of all patients were analysed by ELISA method. RESULTS: The mean ADMA level in the CAE group was found to be significantly higher than the mean ADMA level in the normal coronary artery group (2.26 +/- 0.47 vs. 1.43 +/- 0.40 micromol/l, p < 0.001). The elevated ADMA level (> 1.80 micromol/l) was present in 83.0% of patients from the CAE group and 25.0% of patients from the normal coronary artery group (p < 0.001). Having an increased ADMA level enhanced the risk of CAE 15-fold. The multiple-adjusted OR of the risk of CAE was 18.71 (95% CI 4.95-70.68) for the higher ADMA level compared to the lower level. CONCLUSION: Asymmetric dimethylarginine level is significantly associated with the presence of coronary artery ectasia. These findings suggest that increased ADMA level may be associated with endothelial dysfunction leading to the development of coronary artery ectasia.

Effects of statin therapy in children complicated with coronary arterial abnormality late after Kawasaki disease: a pilot study.

BACKGROUND: Ongoing low-grade inflammation and endothelial dysfunction persist in patients late after Kawasaki disease (KD). Statins not only reduce cholesterol, but also improve surrogate markers of atherosclerosis and endothelial dysfunction, but their effects for children late after KD complicated with coronary arterial abnormality (CAA) has not been evaluated. METHODS AND RESULTS: The 11 KD children complicated with CAA (mean age 12.9+/-2.5 years, mean interval from episode 10.77+/-3.01 years) and 11 age- and gender-matched healthy controls were studied. The KD group received oral simvastatin 10 mg/day for 3 months. Lipid profiles, high-sensitivity C-reactive protein (hs-CRP) and flow-mediated dilation (FMD) of the brachial artery were performed at baseline in both groups and 3 months later in the KD group. At baseline, the KD group had significantly higher hs-CRP level and decreased FMD than the control group. After 3 months' treatment, the KD group showed a significant reduction in the hs-CRP level and a significant increase in FMD. CONCLUSIONS: In this small study, short-term statin therapy appeared to significantly improve chronic vascular inflammation and endothelial dysfunction with no adverse effects in children complicated by CAA late after KD. However, long-term and randomized studies are still needed to make further conclusions.

Mechanism of myocardial microvessel formation in cyanotic congenital heart disease.

BACKGROUND: Patients with cyanotic congenital heart disease (C-CHD) usually have myocardial thickening and fibrosis, both of which can affect the course of surgical management. Hypoxia and ischemia may stimulate microvessel formation in the myocardium, which may accelerate the myocardial thickening and fibrosis. Whether hyperplasia of microvessels occurs in the myocardium of C-CHD was investigated in this report. METHODS AND RESULTS: The patients were divided into 2 groups; the C-CHD group (n = 22), and the acyanotic congenital heart disease (A-CHD) group (n = 24). The microvessels and vascular endothelial growth factor (VEGF) mRNA of the myocardium were detected by immunohistochemical staining assay and real-time quantitative reverse transcriptase polymeric chain reaction, respectively. The serum VEGF levels were measured by using enzyme-linked immunosorbent assay. The results were that: (1) the number of microvessels in the myocardium were more in the C-CHD group than in A-CHD group; (2) the serum VEGF levels in the C-CHD group vs the A-CHD group were higher in the preoperative period (p < 0.001), but there was no difference after operation; and (3) VEGF protein and the expression of VEGF mRNA in the myocardium were higher in the C-CHD group than in the A-CHD group (p < 0.01). CONCLUSIONS: Myocardial microvessels formed in the myocardium of patients with C-CHD, possibly mediated by increasing VEGF levels (for this group of patients).