Severe Mitral Valve Regurgitation due to Significant Coronary Artery Stenosis in a 14-month-old boy with Williams-Beuren Syndrome

2019 ◽  
Vol 232 (01) ◽  
pp. 40-42
Author(s):  
Andreas Hornung ◽  
Ludger Sieverding ◽  
Christian Schlensak ◽  
Michael Hofbeck

IntroductionWilliams-Beuren syndrome (WBS) is characterized by a combination of cardiovascular malformations, typical dysmorphic stigmata, a moderate developmental delay and behavioral abnormalities. According to the literature the incidence is within a range from 1:20 000 to 1:7 500 (Yildiz O et al., World J Pediatr Congenit Heart Surg 2015; 6: 311–316). Microdeletion of chromosome region 7q11.23 is pathognomonic, affecting the elastin gene (von Beust G et al., Klin Padiatr 2000; 212: 299–307). Due to reduced elastin content elasticity in the media of the arterial vessel wall is reduced, resulting in a vasculopathy affecting preferentially the medium size and large arteries. As a characteristic feature supravalvular aortic and pulmonary artery stenosis is very common in patients with WBS. The supravalvular aortic stenosis may also affect the origin of the coronary arteries resulting in an increased risk of myocardial ischemia (Collins RT 2nd, Circulation 2013; 127: 2125–2134). We report the case of a little boy with ischemic mitral regurgitation due to stenosis of the left main coronary artery.

2020 ◽  
pp. 1-7
Author(s):  
Ching-I Wu ◽  
Chia-Lun Wu ◽  
Feng-Chieh Su ◽  
Shun-Wen Lin ◽  
Wen-Yi Huang

<b><i>Background:</i></b> The coincidence of coronary artery disease (CAD) and carotid artery stenosis (CAS) was observed. However, the association between pre-existing CAD and ischemic stroke (IS) outcome in patients with high-grade CAS remains unclear. We aimed to investigate the association between pre-existing CAD and outcomes of acute IS patients with high-grade CAS. <b><i>Methods:</i></b> From January 1, 2007, to April 30, 2012, we enrolled 372 acute IS patients with high-grade CAS and prospectively observed them for 5 years. Demographic features, vascular risk factors, comorbidities, and outcomes were compared between patients with and without pre-existing CAD. <b><i>Results:</i></b> Among 372 individuals, 75 (20.2%) patients had pre-existing CAD and 297 (79.8%) patients did not have pre-existing CAD. The prevalence rates of hypertension, congestive heart failure, chronic kidney disease, and gout in patients with pre-existing CAD were significantly higher than in those without pre-existing CAD (<i>p</i> = 0.017, <i>p</i> &#x3c; 0.001, <i>p</i> = 0.002, and <i>p</i> &#x3c; 0.001, respectively). The multivariate Cox proportional hazards model revealed that pre-existing CAD was a significant risk factor for a 5-year all-cause mortality in acute IS patients with high-grade CAS (hazard ratio = 2.26; 95% confidence interval = 1.35–3.79; <i>p</i> = 0.002). <b><i>Conclusion:</i></b> Pre-existing CAD was associated with an increased risk of 5-year mortality in acute IS patients with high-grade CAS. Intensive treatment for the pre-existing CAD may reduce long-term mortality in acute IS patients with high-grade CAS.


2021 ◽  
Vol 10 (15) ◽  
pp. 3341
Author(s):  
Hyun-Jin Kim ◽  
Min-Ho Lee ◽  
Sang-Ho Jo ◽  
Won-Woo Seo ◽  
Hack-Lyoung Kim ◽  
...  

Vasospastic angina (VA) is a functional disease of the coronary artery and occurs in an angiographically normal coronary artery. However, it may also occur with coronary artery stenosis. We investigated the effect of coronary artery stenosis on clinical outcomes in VA patients. Study data were obtained from a prospective multicenter registry that included patients who had symptoms of VA. Patients were classified into two groups according to presence of significant coronary artery stenosis. Among 1920 patients with VA, 189 patients were classified in the “significant stenosis” group. The one-year composite clinical events rate was significantly higher in the significant stenosis group than in the “no significant stenosis” group (5.8% vs. 1.4%, respectively; p < 0.001). Additionally, the prevalence of ACS was significantly greater in the "significant stenosis" group (4.8% vs. 0.9%, respectively; p < 0.001). After propensity score matching, the adverse effects of significant stenosis remained. In addition, significant stenosis was independently associated with a 6.67-fold increased risk of ACS in VA patients. In conclusion, significant coronary artery stenosis can increase the adverse clinical outcomes in VA patients at long-term follow-up. Clinicians should manage traditional risk factors associated with atherosclerosis and control vasospasm as well as reduce the burden of atherosclerosis.


2000 ◽  
Vol 83 (06) ◽  
pp. 822-825 ◽  
Author(s):  
Antoine Da Costa ◽  
Stéphane Munier ◽  
Bernard Mercier ◽  
Brigitte Tardy ◽  
Claude Ferec ◽  
...  

SummaryFactor V Leiden is associated with an increased risk of venous thrombosis and myocardial infarction in young women, but not in men in this latter case. The aim of this study was to evaluate the prevalence of this mutation in patients with myocardial infarction but normal coronary angiography.We compared 3 groups of patients: one group consisted of 107 patients with premature myocardial infarction but no significant coronary artery stenosis; another group of 244 patients with myocardial infarction and significant coronary artery stenosis; a third group of 400 healthy controls.Factor V Leiden was found in 13 patients (12.1%) who had a myocardial infarction without significant coronary artery stenosis, 11 patients (4.5%) who had a myocardial infarction with significant coronary artery stenosis (p = 0.01) and in 20 controls (5%) (p = 0.01). Odds ratio associated with factor V Leiden were respectively 2.93 (CI95 : 1.18-7.31) and 2.63 (CI95 : 1.19-5.78) when we compared myocardial infarction patients without significant coronary artery stenosis to controls or to patients with significant coronary artery stenosis.In myocardial infarction patients without significant coronary artery stenosis, prevalence of factor V Leiden is significantly higher than in controls. This new finding supports the hypothesis that thrombosis plays a key role in this selected situation.


2020 ◽  
Author(s):  
Miha Tibaut ◽  
Sara Mankoč Ramuš ◽  
Daniel Petrovic

Abstract Background: We aimed to examine the role of the rs6060566 polymorphism of the reactive oxygen species modulator 1 (ROMO1) gene in the development of myocardial infarction (MI) in Caucasians with type 2 diabetes (T2DM).Methods: A total of 1072 subjects with T2DM were enrolled in this cross-sectional case-control study: 335 subjects with MI and 737 subjects without clinical signs of coronary artery disease (CAD). The genetic analysis of the rs6060566 polymorphism was performed in all subjects. To assess the degree of coronary artery obstruction, a subpopulation of 128 subjects with T2DM underwent coronary computed tomography angiography. Next, endarterectomy samples were obtained during myocardial revascularization from diffusely diseased coronary arteries in 40 cases, which were analysed for ROMO1 expression according to their genotype.Results: There were no statistically significant associations between different genotypes or alleles of the rs6060566 polymorphism and MI in subjects with T2DM. The carriers of the C allele of the ROMO1 rs6060566 had a threefold increased likelihood of having 50%-75% coronary artery stenosis (Adjusted OR= 3.27, 95% CI 1.16 – 9.20). Subjects with two affected coronary arteries had a 3.72 fold higher prevalence of MI (OR= 3.72, 95% CI 1.27 – 10.84). With CAD in LMCA or LAD, MI prevalence was about 3.5-fold higher (p=0.07 for LMCA and p=0.01 for LAD). Furthermore, the carriers of the rs6060566 C allele showed higher number of positive cells for ROMO1 expression in endarterectomy samples of coronary arteries.Conclusions: According to our study, the rs6060566 polymorphism of the ROMO1 gene is not a risk factor for MI in Caucasians with T2DM. However, we found that subjects carrying the C allele were at a 3.27-fold increased risk of developing severe CAD compared with those who had non-obstructive CAD. Moreover, C allele carriers showed a statistically higher number of cells positive for ROMO1 compared with T allele carriers in coronary endarterectomy samples.


2020 ◽  
Author(s):  
Pham Viet Thai ◽  
Hoang Anh Tien ◽  
Huynh Van Minh ◽  
Paul Valensi

Abstract Background: Triglyceride Glucose (TyG) index has been associated with an increased risk in cardiovascular events. Silent coronary disease is common in patients with type 2 diabetes. In Vietnam, a low-middle income country, the burden of cardiovascular disease is growing simultaneously with the epidemiologic transition. Our aim was to assess the prevalence of coronary stenoses (CS) in patients with type 2 diabetes and no history or symptom of cardiovascular disease and to investigate the association between TyG index and cardiovascular risk factors and both the presence and severity of CS. Futhermore, we assessed the value of TyG index in predicting subclinical CS. Methods: This was a cross-sectional observational study. We recruited 166 patients at Ninh Thuan General Hospital, Vietnam. TyG index and HOMA-IR were calculated, and a coronary computed tomography angiography (CCTA) was performed.Results: The population was classified according to tertiles of TyG index. The highest TyG values were associated with higher BMI, waist circumference, total cholesterol, LDL-cholesterol, triglycerides, plasma glucose, HbA1c levels and HOMA-IR, lower HDL-cholesterol, a higher incidence of metabolic syndrome and less frequent physical activity (p<0.05 to <0.0001). TyG index correlated with logHOMA-IR (p<0.0001). CS ≥50% were present in 60 participants and 32 had coronary artery stenosis ≥70%. TyG index and HOMA-IR were significantly higher in patients with CS ≥70%. The number of narrowed coronary arteries and the degree of stenosis were associated with higher TyG index levels (p=0.04 and <0.005 respectively). A TyG index ≥ 10 was significantly associated with an increased risk of multiple coronary artery disease and of more severe CS. After adjusting for confounding factors, including logHOMA-IR, these risks remained mostly significant. A TyG index threshold at 10 resulted in 57% sensitivity and 75% specificity for predicting the presence of CS ≥ 70%. In subgroup analysis TyG index ≥ 10 was associated with an increased risk in CS ≥ 70% in patients treated with statin or antiplatelet therapy.Conclusion: More than one third of asymptomatic patients with type 2 diabetes had significant CS on CCTA. TyG index may be considered as a marker for insulin resistance and increased TyG index could identify patients with high risk of coronary artery stenoses and is associated with the number and the severity of artery stenoses.


2020 ◽  
Author(s):  
Miha Tibaut ◽  
Sara Mankoč Ramuš ◽  
Daniel Petrovic

Abstract Background: We aimed to examine the role of the rs6060566 polymorphism of the reactive oxygen species modulator 1 (ROMO1) gene in the development of myocardial infarction (MI) in Caucasians with type 2 diabetes (T2DM).Methods: A total of 1072 subjects with T2DM were enrolled in this cross-sectional case-control study: 335 subjects with MI and 737 subjects without clinical signs of coronary artery disease (CAD). The genetic analysis of the rs6060566 polymorphism was performed in all subjects. To assess the degree of coronary artery obstruction, a subpopulation of 128 subjects with T2DM underwent coronary computed tomography angiography. Next, endarterectomy samples were obtained during myocardial revascularization from diffusely diseased coronary arteries in 40 cases, which were analysed for ROMO1 expression according to their genotype.Results: There were no statistically significant associations between different genotypes or alleles of the rs6060566 polymorphism and MI in subjects with T2DM. The carriers of the C allele of the ROMO1 rs6060566 had a threefold increased likelihood of having 50%-75% coronary artery stenosis (AOR= 3.27, 95% CI 1.16 – 9.20). Subjects with two affected coronary arteries had a 3.72 fold higher prevalence of MI (OR= 3.72, 95% CI 1.27 – 10.84). With CAD in LMCA or LAD, MI prevalence was about 3.5-fold higher (p=0.07 for LMCA and p=0.01 for LAD). Furthermore, the carriers of the rs6060566 C allele showed higher number of positive cells for ROMO1 expression in endarterectomy samples of coronary arteries.Conclusions: According to our study, the rs6060566 polymorphism of the ROMO1 gene is not a risk factor for MI in Caucasians with T2DM. However, we found that subjects carrying the C allele were at a 3.27-fold increased risk of developing severe CAD compared with those who had non-obstructive CAD. Moreover, C allele carriers showed a statistically higher number of cells positive for ROMO1 compared with T allele carriers in coronary endarterectomy samples.


2020 ◽  
Vol 19 (1) ◽  
Author(s):  
Pham Viet Thai ◽  
Hoang Anh Tien ◽  
Huynh Van Minh ◽  
Paul Valensi

Abstract Background Triglyceride Glucose (TyG) index has been associated with an increased risk in cardiovascular events. Silent coronary disease is common in patients with type 2 diabetes. In Vietnam, a low-middle income country, the burden of cardiovascular disease is growing simultaneously with the epidemiologic transition. Our aim was to assess the prevalence of coronary stenoses (CS) in patients with type 2 diabetes and no history or symptom of cardiovascular disease and to investigate the association between TyG index and cardiovascular risk factors and both the presence and severity of CS. Futhermore, we assessed the value of TyG index in predicting subclinical CS. Methods This was a cross-sectional observational study. We recruited 166 patients at Ninh Thuan General Hospital, Vietnam. TyG index and HOMA-IR were calculated, and a coronary computed tomography angiography (CCTA) was performed. Results The population was classified according to tertiles of TyG index. The highest TyG values were associated with higher BMI, waist circumference, total cholesterol, LDL-cholesterol, triglycerides, plasma glucose, HbA1c levels and HOMA-IR, lower HDL-cholesterol, a higher incidence of metabolic syndrome and less frequent physical activity (p < 0.05 to < 0.0001). TyG index correlated with logHOMA-IR (p < 0.0001). CS ≥ 50% were present in 60 participants and 32 had coronary artery stenosis ≥ 70%. TyG index and HOMA-IR were significantly higher in patients with CS ≥ 70%. The number of narrowed coronary arteries and the degree of stenosis were associated with higher TyG index levels (p = 0.04 and < 0.005 respectively). A TyG index ≥ 10 was significantly associated with an increased risk of multiple coronary artery disease and of more severe CS. After adjusting for confounding factors, including logHOMA-IR, these risks remained mostly significant. A TyG index threshold at 10 resulted in 57% sensitivity and 75% specificity for predicting the presence of CS ≥ 70%. In subgroup analysis TyG index ≥ 10 was associated with an increased risk in CS ≥ 70% in patients treated with statin or antiplatelet therapy. Conclusion More than one third of asymptomatic patients with type 2 diabetes had significant CS on CCTA. TyG index may be considered as a marker for insulin resistance and increased TyG index could identify patients with high risk of coronary artery stenoses and is associated with the number and the severity of artery stenoses.


2019 ◽  
Vol 7 ◽  
pp. 2050313X1984195 ◽  
Author(s):  
Barry S Weinstock ◽  
Yitzhak Daniel Haim

Patients with Takayasu’s arteritis have pulmonary artery involvement more commonly than previously appreciated. The majority of these lesions are in segmental or sub-segmental arteries, but there are some patients who have severe stenosis of the main pulmonary arteries. Interventional treatment of these patients is technically feasible, but there are only limited case reports describing such intervention. Balloon angioplasty and stenting in these lesions often require high pressure inflations to achieve adequate results and thus has increased risk due to the possibility of pulmonary artery rupture. The recently approved Viabahn BX balloon-expandable covered stent may be an optimal device for main pulmonary artery stenosis as it is relatively low profile, can be over-expanded to large vessel diameter without compromise or disruption of the polytetrafluoroethylene covering and virtually eliminates the risk of catastrophic pulmonary artery rupture. We report here the first known use of this novel stent for treatment of severe pulmonary artery stenosis.


Sign in / Sign up

Export Citation Format

Share Document