scholarly journals Sedation in a patient with William Syndrome without supravalvular aortic stenosis and normal QT: Challenge still remains

2020 ◽  
Vol 24 (3) ◽  
Author(s):  
Roma Sharma ◽  
Habib Md Reazaul Karim ◽  
Omer Mohammed Mujahid ◽  
Rahul Kanjilal

William Syndrome (WS) is a rare genetic condition related to deletion of elastin gene resulting in distinctive facies, cardiovascular diseases, learning difficulties, developmental delay, unique personality characteristics, endocrine involvement, etc.1 Although successful anaesthesia is reported, literature review indicate significant major adverse cardiac events (MACE) including sudden death, as frequent.2 So anaesthetic management of these patients has proved to be challenging. The cardiovascular abnormality, corrected QT, and cardio-depressant actions of anaesthetic drugs need critical considerations.

2018 ◽  
Vol 25 (17) ◽  
pp. 1852-1860 ◽  
Author(s):  
Hayato Tada ◽  
Masa-aki Kawashiri ◽  
Akihiro Nomura ◽  
Kenichi Yoshimura ◽  
Hiroshi Itoh ◽  
...  

Aims Low-density lipoprotein cholesterol predicts cardiovascular events in patients with diabetes. However, it is uncertain whether serum triglycerides level is also associated with an occurrence of future cardiovascular events in diabetic patients. We aimed to test whether serum triglycerides is associated with first cardiovascular events in diabetic patients. Methods and results We recruited 5042 participants with diabetes mellitus from the standard versus intEnsive statin therapy for hypercholesteroleMic Patients with diAbetic retinopaTHY (EMPATHY), multicenter, prospective, randomized, open-label, blinded-endpoint study. Median follow-up was three years. We evaluated an association of serum triglycerides with first cardiovascular events in cox-regression hazard models adjusted by age, sex, hypertension, current smoking, low-density lipoprotein cholesterol, and body mass index. Cardiovascular events were defined as (a) major adverse cardiac events including myocardial infarction, stroke, or cardiac death; and (b) cardiovascular diseases including myocardial infarction, unstable angina, ischemic stroke, or large artery disease or peripheral arterial disease. Serum triglycerides were associated with major adverse cardiac events (adjusted hazard ratio: 1.021 per 10 mg/dl; 95% confidence interval: 1.007–1.035; p = 0.0025) and cardiovascular diseases (adjusted hazard ratio: 1.023 per 10 mg/dl; 95% confidence interval: 1.013–1.034; p = 0.0000077). Comparing the top quintile (>185 mg/dl) with the bottom quintile (<79 mg/dl), the adjusted hazard ratio increased 1.89 (95% confidence interval: 1.03–2.80, p = 0.04) for major adverse cardiac events, and 1.90 (95% confidence interval: 1.18–3.07, p = 0.007) for cardiovascular diseases. There were no overall interactions of triglycerides and treatment assignment (standard/intensive statins) on both outcomes ( p-trend = 0.33 for major adverse cardiac events, p-trend = 0.62 for cardiovascular diseases). Conclusions Serum triglycerides were associated with first cardiovascular events among high-risk diabetes patients with hypercholesterolemia and retinopathy.


2013 ◽  
Vol 10 (2) ◽  
pp. 100-102
Author(s):  
GS Shrestha ◽  
D Aryal

Dermatomyositis is an idiopathic inflammatory myopathy with involvement of muscle, skin and other organs. Valvular heart disease increases the risk of perioperative adverse cardiac events. Only a little information is available about the anaesthetic management of a patient with dermatomyositis and valvular heart disease. Here we considered combined spinal-epidural technique for total abdominal hysterectomy, minimizing the risk of delayed recovery from muscle relaxants, aspiration pneumonitis, arrhythmias and cardiac failure. Kathmandu University Medical Journal | Vol.10 | No. 2 | Issue 38 | Apr – June 2012 | Page 100-102 DOI: http://dx.doi.org/10.3126/kumj.v10i2.7355


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
L Piatek ◽  
L Zandecki ◽  
J Kurzawski ◽  
A Janion-Sadowska ◽  
M Zabojszcz ◽  
...  

Abstract Background Both unstable angina (UA) and non-ST-segment elevation myocardial infarction (NSTEMI) are still classified together in non-ST-elevation acute coronary syndromes despite the fact they substantially differ in both clinical profile and prognosis. Purpose The aim of the present study was to evaluate contemporary clinical characteristics and outcomes of UA patients after percutaneous coronary intervention (PCI) in comparison with stable angina (SCAD) and myocardial infarction (NSTEMI as well as STEMI) in Swietokrzyskie District of Poland in years 2014–2017. Methods A total of 7'187 patients after PCI from ORPKI Registry (38% with diagnosis of UA) were included into the analysis. Impact of clinical presentation (UA, SCAD, NSTEMI, STEMI) on 3-year outcomes were determined. Results UA patients were older that SCAD but younger than NSTEMI individuals. Diabetes and hypertension were more often encountered into UA group than in NSTEMI but less often than in SCAD cases. In UA group the percentage of previous myocardial infarction (MI), PCI or coronary artery bypass grafting (CABG) was the highest among all analyzed groups. In 3-year observation the risk of death as well as myocardial infarction (MI) and major adverse cardiac events (MACE) in unstable angina after PCI was higher than in stable angina but considerably lower than in NSTEMI group. Multivariate analysis confirmed that prognosis in NSTEMI was substantially worse in comparison with UA (RR 1.365, 95% CI: 1.126–1.655, p=0.0015). On the contrary there were no difference in mortality risk between UA and SCAD patients (RR 1.189, 95% CI: 0.932–1.518, p=0.1620). Parallel results were observed in respect of MI and MACE. Independ predictors of death were: age, kidney disease, hypertension, diabetes, previous stroke or previous PCI. Multivariate logistic regression analyse Clinical presentation Death Myocardial infarction MACE RR 95% CI p-value RR 95% CI p-value RR 95% CI p-value NSTEMI/UA 1.365 1.126–1.655 0.0015 1.822 1.076–3.055 0.0260 1.514 1.267–1.807 <0.0001 NSTEMI/SCAD 1.624 1.251–2.109 0.0003 1.882 0.982–3.789 0.0568 1.604 1.275–2.094 <0.0001 UA/SCAD 1.189 0.932–1.518 0.1620 1.033 0.557–2.034 0.9219 1.060 0.855–1.323 0.6023 MACE, major adverse cardiac events; NSTEMI, non-ST-segment elevation myocardial infarction; UA, unstable angina; SCAD, stable angina. Conclusion Unstable angina accounted for 38% of all cases and was the most common diagnosis in patients that underwent PCI in that time. 3-year prognosis in UA was considerable better in comparison with NSTEMI. On contrary there was no difference in outcomes (death, MI, MACE) between UA and SCAD patients.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yahya Dadjo ◽  
Maryam Moshkani Farahani ◽  
Reza Nowshad ◽  
Mohsen Sadeghi Ghahrodi ◽  
Alireza Moaref ◽  
...  

Abstract Background Rheumatic heart disease (RHD) is still a concerning issue in developing countries. Among delayed RHD presentations, rheumatic mitral valve stenosis (MS) remains a prevalent finding. Percutaneous transvenous mitral commissurotomy (PTMC) is the intervention of choice for severe mitral stenosis (MS). We aimed to assess the mid-term outcome of PTMC in patients with immediate success. Methods In this retrospective cohort study, out of 220 patients who had undergone successful PTMC between 2006 and 2018, the clinical course of 186 patients could be successfully followed. Cardiac-related death, undergoing a second PTMC or mitral valve replacement (MVR) were considered adverse cardiac events for the purpose of this study. In order to find significant factors related to adverse cardiac outcomes, peri-procedural data for the studied patients were collected.The patients were also contacted to find out their current clinical status and whether they had continued secondary antibiotic prophylaxis regimen or not. Those who had not suffered from the adverse cardiac events were additionally asked to undergo echocardiographic imaging, in order to assess the prevalence of mitral valve restenosis, defined as mitral valve area (MVA) < 1.5 cm2 and loss of ≥ 50% of initial area gain. Results During the mean follow-up time of 5.69 ± 3.24 years, 31 patients (16.6% of patients) had suffered from adverse cardiac events. Atrial fibrillation rhythm (p = 0.003, HR = 3.659), Wilkins echocardiographic score > 8 (p = 0.028, HR = 2.320) and higher pre-procedural systolic pulmonary arterial pressure (p = 0.021, HR = 1.031) were three independent predictors of adverse events and immediate post-PTMC mitral valve area (IMVA) ≥ 2 cm2 (p < 0.001, HR = 0.06) was the significant predictor of event-free outcome. Additionally, follow-up echocardiographic imaging detected mitral restenosis in 44 patients (23.6% of all patients). The only statistically significant protective factor against restenosis was again IMVA ≥ 2 cm2 (p = 0.001, OR = 0.240). Conclusion The mid-term results of PTMC are multifactorial and may be influenced by heterogeneous peri-procedural determinants. IMVA had a great impact on the long-term success of this procedure. Continuing secondary antibiotic prophylaxis was not a protective factor against adverse cardiac events in this study. (clinicaltrial.gov registration: NCT04112108).


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