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Addressing comprehensive complexities a striking familial hypercholesterolemia case study 解决综合复杂问题 一个引人注目的家族性高胆固醇血症案例研究
Pub Date : 2024-04-20 DOI: 10.1186/s43044-024-00483-5
Shazia Rasheed, Ghulam Kubra, Lubna Baqai, Muhammad Liaquat Raza, Fariha Hassan, Syed Ghazi Abbas Rizvi
Premature aortic involvement and comprehensive management strategies in familial hypercholesterolemia familial hypercholesterolemia (FH), a rare autosomal dominant genetic disorder, poses significant challenges due to its propensity for elevated low-density lipoprotein cholesterol, premature coronary heart disease, and vascular atherosclerosis. Unraveling Cardiovascular Complexities: A Striking Familial Hypercholesterolemia. This case study delves into a remarkable instance of FH in a 16-year-old female who presented with chest pain and worsening dyspnea. Diagnostic evaluation revealed distinct electrocardiographic changes, elevated troponin levels, and profound dyslipidemia. Remarkable findings on transthoracic echocardiography, computed tomography angiography, and catheterization prompted multidisciplinary interventions and demonstrated remarkable enhancements in ventricular function, mitral regurgitation, and aortic stenosis. The case study underscores the urgency of comprehensive management strategies in confronting the myriad challenges of FH, emphasizing the value of early intervention, innovative therapies, and rigorous imaging modalities for unraveling the intricate cardiovascular manifestations of this condition.
家族性高胆固醇血症过早累及主动脉和综合管理策略 家族性高胆固醇血症(FH)是一种罕见的常染色体显性遗传疾病,由于容易导致低密度脂蛋白胆固醇升高、过早发生冠心病和血管动脉粥样硬化,因此面临着巨大的挑战。揭开心血管疾病的复杂面纱:一个惊人的家族性高胆固醇血症。本病例研究深入探讨了一名 16 岁女性的家族性高胆固醇血症,她因胸痛和呼吸困难恶化而就诊。诊断评估发现了明显的心电图改变、肌钙蛋白水平升高和严重的血脂异常。经胸超声心动图、计算机断层扫描血管造影和导管检查的显著发现促使患者接受多学科干预,并显示心室功能、二尖瓣反流和主动脉狭窄明显改善。该病例研究强调了综合管理策略在应对 FH 的无数挑战方面的紧迫性,强调了早期干预、创新疗法和严格的成像模式在揭示这种疾病错综复杂的心血管表现方面的价值。
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引用次数: 0
Unusual coexistence of restrictive heart disease and Kallmann syndrome: a case report 限制性心脏病与卡尔曼综合征并存的罕见病例报告
Pub Date : 2024-04-18 DOI: 10.1186/s43044-024-00479-1
Ghali Bennani, Soukaina Zahri, Mohamed Khaldi, Ghali Benouna, Abdenasser Drighil, Rachida Habbal
Kallmann–Morsier syndrome is a rare disease characterized by the association of congenital gonadotropic deficiency and anosmia or hyposmia. The cardiac manifestations associated with this syndrome are little known. Through this case, we will characterize the cardiac involvement of this disease in the light of what is already described in the literature. We report the case of a young patient who presented with a picture of cardiac decompensation revealing restrictive heart disease. In her exploration, she was found to have primary amenorrhea, leading to the diagnosis of Kallmann syndrome. Medical treatment was optimized for the management of her cardiac decompensation as well as hormonal replacement treatment for her delayed puberty and growth. Cardiac manifestations in Kallmann–Morsier syndrome are few reported in the literature, and restrictive heart disease is uncommon with no cases report till now. This association suggests a possible common genetic origin that should be explored in the future.
卡尔曼-莫西埃综合征是一种罕见的疾病,其特点是伴有先天性促性腺激素缺乏和无睾症或睾丸发育不全。与该综合征相关的心脏表现鲜为人知。通过本病例,我们将根据文献中已有的描述来描述这种疾病累及心脏的特点。我们报告了一例年轻患者的病例,她出现心脏失代偿症状,显示为局限性心脏病。在对她进行检查时,发现她有原发性闭经,因此诊断为卡尔曼综合征。为了治疗她的心脏失代偿以及青春期发育延迟,医生对她进行了优化的药物治疗和激素替代治疗。Kallmann-Morsier 综合征的心脏表现在文献中鲜有报道,限制性心脏病也不常见,至今尚无病例报道。这种关联提示可能存在共同的遗传起源,今后应对此进行研究。
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引用次数: 0
Efficacy of single high-dose statin prior to percutaneous coronary intervention in acute coronary syndrome: a systematic review and meta-analysis 急性冠状动脉综合征经皮冠状动脉介入治疗前单次大剂量他汀类药物的疗效:系统综述和荟萃分析
Pub Date : 2024-04-17 DOI: 10.1186/s43044-024-00481-7
Bryan Gervais de Liyis, Gusti Ngurah Prana Jagannatha, Anastasya Maria Kosasih, I. Kadek Susila Surya Darma, I. Made Junior Rina Artha
The impacts of single high-dose statin preloading in patients undergoing percutaneous coronary intervention (PCI) have not been fully examined. This study aims to evaluate post-procedure impacts of single high-dose statin pretreatment with acute coronary syndrome (ACS). The meta-analysis reviewed Cochrane, PubMed, and Medline databases for studies comparing single high-dose atorvastatin or rosuvastatin to placebo in ACS patients undergoing PCI. The primary endpoints included major adverse cardiovascular events (MACE), myocardial infarction (MI), all-cause mortality, and target vessel revascularization (TVR) at three months. Secondary endpoints examined were the TIMI flow grade 3 and left ventricular ejection fraction (LVEF). Comprehensive analysis was conducted on fifteen RCTs, encompassing a total of 6,207 patients (3090 vs 3117 patients). The pooled results demonstrated that a single high-dose of statin administered prior to PCI led to a significant decrease in the incidence of MACE at three months post-PCI compared to the control group (OR 0.50, 95%CI 0.35–0.71, p = 0.0001). The occurrence of MI (OR 0.57, 95%CI 0.42–0.77, p = 0.0002), all-cause mortality (OR 0.56, 95%CI 0.39–0.81, p = 0.0002), and TVR (OR 0.56, 95%CI 0.35–0.92, p = 0.02) was significantly lower in the statin single high-dose group compared to the control group. No significant effects on TIMI flow grade 3 (OR 1.20, 95%CI 0.94–1.53, p = 0.14) or left ventricular ejection fraction (OR 2.19, 95%CI − 0.97 to 5.34, p = 0.17) were observed. Subgroup analysis demonstrated reduced incidence of MACE with a single dose of 80 mg atorvastatin (OR 0.66, 95%CI 0.54–0.81, p < 0.0001) and 40 mg rosuvastatin (OR 0.19, 95%CI 0.07–0.54, p = 0.002). Single high-dose statin before PCI in patients with ACS significantly reduces MACE, MI, all-cause mortality, and TVR three months post-PCI.
单次大剂量他汀类药物预处理对接受经皮冠状动脉介入治疗(PCI)患者的影响尚未得到充分研究。本研究旨在评估单次大剂量他汀预处理对急性冠脉综合征(ACS)患者术后的影响。荟萃分析回顾了 Cochrane、PubMed 和 Medline 数据库中对接受 PCI 的 ACS 患者进行的单次大剂量阿托伐他汀或罗苏伐他汀与安慰剂的比较研究。主要终点包括主要不良心血管事件(MACE)、心肌梗死(MI)、全因死亡率和三个月后靶血管血运重建(TVR)。次要终点是 TIMI 血流 3 级和左心室射血分数 (LVEF)。对 15 项 RCT 进行了综合分析,共涉及 6207 名患者(3090 对 3117 名患者)。汇总结果显示,与对照组相比,在PCI术前服用单次大剂量他汀类药物可显著降低PCI术后三个月的MACE发生率(OR 0.50,95%CI 0.35-0.71,P = 0.0001)。与对照组相比,他汀类药物单次高剂量组的 MI(OR 0.57,95%CI 0.42-0.77,p = 0.0002)、全因死亡率(OR 0.56,95%CI 0.39-0.81,p = 0.0002)和 TVR(OR 0.56,95%CI 0.35-0.92,p = 0.02)发生率显著降低。对 TIMI 血流 3 级(OR 1.20,95%CI 0.94-1.53,p = 0.14)或左室射血分数(OR 2.19,95%CI - 0.97 至 5.34,p = 0.17)无明显影响。亚组分析显示,单次服用80毫克阿托伐他汀(OR 0.66,95%CI 0.54-0.81,p < 0.0001)和40毫克罗伐他汀(OR 0.19,95%CI 0.07-0.54,p = 0.002)可降低MACE发生率。ACS患者在PCI术前服用单次大剂量他汀类药物可显著降低PCI术后三个月的MACE、MI、全因死亡率和TVR。
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引用次数: 0
Efficacy and safety of cangrelor as compared to ticagrelor in patients with ST-elevated myocardial infarction (STEMI): a systematic review and meta-analysis 坎格雷罗与替卡格雷罗相比对ST段抬高型心肌梗死(STEMI)患者的疗效和安全性:系统综述和荟萃分析
Pub Date : 2024-04-16 DOI: 10.1186/s43044-024-00480-8
Subhro Chakraborty, Debalina Sarkar, Shambo Samrat Samajdar, Pallab Biswas, Debasish Mohapatra, Saptarshi Halder, Mohammad Yunus
This systematic review and meta-analysis aimed to compare the efficacy and safety of cangrelor as compared to ticagrelor in patients with ST-elevated myocardial infarction (STEMI) who underwent percutaneous intervention. PubMed, Embase, Scopus, Web of Science, Cochrane CENTRAL, and ClinicalTrials.gov databases were searched for relevant head-on-comparison or swapping studies. The primary outcome was the rate of high platelet reactivity (HPR) at specific time intervals after stopping cangrelor infusion during the first 24 h. Secondary outcomes were the risks of thrombosis, all-cause mortality and bleeding. Pooled odds ratios (ORs) were calculated using random-effects models. A total of 1018 studies were screened and eight were included in the analysis. There were four head-on-comparison studies and four swapping studies. There was no significant difference in the proportion of patients achieving a high platelet reactivity in swapping studies [OR, 0.71 (95% CI 0.04, 13.87), p = 0.82, i2 = 88%]. In head-on-comparison studies, PRU from Fig. 2B shows there was no significant reduction in high platelet reactivity [mean difference – 77.83 (95% CI − 238.84, 83.18), p < 0.001, i2 = 100%]. PRU results from (Fig. 2C) show a mean difference of 7.38 (95% CI − 29.74, 44.51), p < 0.001, i2 = 97%. There was no significant difference in the risks of thrombosis [OR, 0.91 (95% CI 0.20, 4.13), p = 0.81, i2 = 0%], all-cause mortality [OR, 3.52 (95% CI 0.44, 27.91), p = 0.24, i2 = 26%] and bleeding [OR, 0.89 (95% CI 0.37, 2.17), p = 0.93, i2 = 0%] between the two groups as revealed in the head-on-comparison studies. The efficacy and safety profiles of cangrelor and ticagrelor were similar in patients with STEMI.
本系统综述和荟萃分析旨在比较坎格雷与替卡格雷在接受经皮介入治疗的ST段抬高型心肌梗死(STEMI)患者中的疗效和安全性。在 PubMed、Embase、Scopus、Web of Science、Cochrane CENTRAL 和 ClinicalTrials.gov 数据库中检索了相关的正面比较或互换研究。主要结果是在停止输注坎格雷罗后的头 24 小时内特定时间间隔内的血小板高反应率(HPR)。采用随机效应模型计算汇总的几率比(OR)。共筛选出 1018 项研究,其中 8 项纳入分析。其中有四项正面比较研究和四项交换研究。在交换研究中,达到高血小板反应性的患者比例没有明显差异[OR,0.71(95% CI 0.04,13.87),P = 0.82,i2 = 88%]。在正面比较研究中,图 2B 的 PRU 显示高血小板反应性没有显著降低[平均差 - 77.83 (95% CI - 238.84, 83.18),p < 0.001,i2 = 100%]。PRU 结果(图 2C)显示平均差异为 7.38(95% CI - 29.74,44.51),P < 0.001,i2 = 97%。在血栓形成风险[OR,0.91 (95% CI 0.20, 4.13),p = 0.81,i2 = 0%]、全因死亡率[OR,3.52 (95% CI 0.44, 27.91),p = 0.24,i2 = 26%]和出血风险[OR,0.89 (95% CI 0.37, 2.17),p = 0.93,i2 = 0%]方面,两组之间没有明显差异,这一点在正面比较研究中也有所体现。坎格雷乐和替卡格雷乐对 STEMI 患者的疗效和安全性相似。
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引用次数: 0
Cost–utility analysis of using high-intensity statin among post-hospitalized acute coronary syndrome patients 住院后急性冠状动脉综合征患者使用高强度他汀类药物的成本效用分析
Pub Date : 2024-04-14 DOI: 10.1186/s43044-024-00478-2
Pramitha Esha Nirmala Dewi, Montarat Thavorncharoensap, Bangunawati Rahajeng
Post-hospitalized acute coronary syndrome (ACS) patients in Indonesia National Insurance does not pay for the use of high-intensity statin (HIS) for secondary prevention after ACS hospitalization. Moreover, a cost–utility analysis needs to be conducted to evaluate the cost-effectiveness of prescribing HIS and low-to-moderate-intensity statin (LMIS) per quality-adjusted life year (QALY). This study aimed to estimate the cost–utility of long-term HIS treatment in post-hospitalized ACS patients in Indonesia compared to current practice. This study compared the economic outcomes of long-term HIS and LMIS in Indonesian post-hospitalized ACS patients. A lifetime Markov model predicted ACS-related events, costs, and QALY from a payer perspective. A systematic review estimated treatment-specific event probabilities, post-event survival, health-related quality of life, and Indonesia medical-care expenses from published sources. This study conducted probabilistic sensitivity analysis (PSA) using 1000 independent Monte Carlo simulations and a series of one-way deterministic sensitivity analyses utilizing a tornado diagram. The economic evaluation model proved that intensive HIS treatment can increase per-patient QALYs and care expenditures compared to LMIS. The use of HIS among post-hospitalized ACS patients had ICER 31.843.492 IDR per QALY gained, below the Indonesia willingness-to-pay (WTP) for terminal disease and life-saving treatment. From the Indonesia payer perspective, using HIS for post-hospitalized ACS patients in Indonesia is cost-effective at 31.843.492 IDR per QALY gained.
在印度尼西亚,急性冠状动脉综合征(ACS)患者住院后使用高强度他汀类药物(HIS)进行二级预防的费用不属于国家保险支付范围。此外,还需要进行成本效用分析,以评估处方高强度他汀和中低强度他汀(LMIS)每质量调整生命年(QALY)的成本效益。本研究旨在估算印度尼西亚住院后 ACS 患者接受长期 HIS 治疗的成本效用,并与现行做法进行比较。本研究比较了印尼住院后 ACS 患者长期 HIS 和 LMIS 的经济效益。一个终身马尔可夫模型从支付方的角度预测了 ACS 相关事件、成本和 QALY。一项系统性综述从已发表的资料中估算了治疗特异性事件概率、事件后存活率、健康相关生活质量和印尼医疗费用。本研究利用 1000 次独立蒙特卡罗模拟进行了概率敏感性分析(PSA),并利用龙卷风图进行了一系列单向确定性敏感性分析。经济评估模型证明,与 LMIS 相比,强化 HIS 治疗可增加每位患者的 QALYs 和护理支出。在住院后的 ACS 患者中使用 HIS,每 QALY 收益的 ICER 为 31.843.492 印度卢比,低于印尼对绝症和救生治疗的支付意愿(WTP)。从印尼支付方的角度来看,印尼住院后 ACS 患者使用 HIS 的成本效益为每 QALY 收益 31.843.492 IDR。
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引用次数: 0
Surgically repaired tetralogy of Fallot in the 7th decade: a late presentation of severe pulmonic regurgitation 法洛四联症手术修复后的第七个十年:严重肺动脉反流的晚期表现
Pub Date : 2024-04-14 DOI: 10.1186/s43044-024-00477-3
Kyle Varkoly, Akarsh Parekh, Melissa Ianitelli, Mostafa Hamada, Alexandra Lucas, Thomas Forbes
Surgically repaired tetralogy of Fallot (TOF) is a congenital heart disease with a cumulative survival rate of 72% in the 4th decade of life in longitudinal single-cohort studies. Debate surrounds conservative versus surgical management in adults with TOF once pulmonary regurgitation occurs. A 73-year-old male with surgically corrected TOF presented with heart failure symptoms. He underwent ToF repair with a classic right Blalock–Taussig shunt at 2 years of age with transannular patching at 18 years of age. Echocardiography revealed elevated right ventricular systolic pressures, severe right ventricular dilatation, and pulmonary regurgitation. Our patient’s new-onset right-sided heart failure was managed medically with diuresis. He received a new pulmonic valve via percutaneous approach on a later planned hospitalization with resolution of symptoms and improved tricuspid regurgitation. It is a class I recommendation for pulmonic valve intervention once greater than moderate PR occurs; however, medical optimization should take place first. Following adequate RV load optimization, our patient underwent successful transcatheter pulmonic valve implantation with resolution of symptoms and cessation of diuretic.
经手术修复的法洛氏四联症(TOF)是一种先天性心脏病,在单队列纵向研究中,其第 4 个十年的累积存活率为 72%。一旦发生肺动脉反流,成人法洛氏四联症患者的保守治疗与手术治疗之间存在争议。一名 73 岁的男性 TOF 患者经手术矫正后出现心衰症状。他在 2 岁时接受了经典的右侧 Blalock-Taussig 分流术进行 TOF 修复,并在 18 岁时接受了经环修补术。超声心动图显示右心室收缩压升高、右心室严重扩张和肺动脉反流。我们对患者新发的右侧心力衰竭进行了药物治疗和利尿。在后来的一次住院计划中,他通过经皮途径接受了新的肺动脉瓣,症状得到缓解,三尖瓣反流也得到改善。一旦出现中度以上的 PR,I 级建议对其进行肺动脉瓣介入治疗;但应首先进行医疗优化。在充分优化 RV 负荷后,我们的患者成功接受了经导管肺动脉瓣植入术,症状得到缓解,并停用了利尿剂。
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引用次数: 0
Left ventricular diastolic function and cardiotoxic chemotherapy 左心室舒张功能与心脏毒性化疗
Pub Date : 2024-04-12 DOI: 10.1186/s43044-024-00476-4
Haider Rashid, Aamir Rashid, Asif Mattoo, Faisal R. Guru, Syed Mehvish, Shahood Ajaz Kakroo, Ajaz Ahmad Lone, Khursheed Aslam, Imran Hafeez, Hilal Rather
Left ventricular ejection fraction falls when the myocardium has already lost a significant portion of its functional capacity. There are conflicting data on whether diastolic dysfunction precedes systolic dysfunction after cardiotoxic chemotherapy. We aimed to study systolic and diastolic dysfunction after cardiotoxic chemotherapy and whether diastolic dysfunction can predict subsequent risk of systolic dysfunction. It was an observational prospective cohort study, and patients receiving cardiotoxic chemotherapy were included. Baseline, demographic, and clinical details were recorded. Echocardiographic measurements of left ventricular systolic function, global longitudinal strain, and diastolic function were noted at baseline, three months, and 6 months. We included eighty patients. The mean age of the patients was 54.92 ± 7.6 years, predominantly females (80%). The mean left ventricular ejection fraction fell from 64.92 ± 1.96 to 60.97 ± 4.94 at 6 months. Low ejection fraction was seen in 8 (10%) patients at 6 months. The mean global longitudinal strain (GLS) at baseline was − 18.81 ± 0.797 and fell to − 17.65 ± 2.057 at 6 months, with 12 (15%) patients having low GLS (< − 18). Grade 1 diastolic dysfunction was seen in 22 (27.5%) patients, and grade 2 diastolic dysfunction was seen in 3 (3.8%) patients at 6 months. There was a significant decrease in E/A ratio (inflow early diastolic velocity/Inflow late diastolic velocity), mitral tissue Doppler velocity, and an increase in isovolumic relaxation time, mitral valve deceleration time, and E/e′ (inflow early diastolic velocity/tissue Doppler mitral annular velocity), at three months and 6 months. Ejection fraction at 6 months was significantly and negatively correlated with diastolic dysfunction at three months (r = − 0.595, p = 0.02). Cardiotoxic chemotherapy is associated with early diastolic dysfunction. Early diastolic dysfunction predicts subsequent left ventricular systolic dysfunction.
当心肌已丧失大部分功能时,左心室射血分数就会下降。关于心脏毒性化疗后舒张功能障碍是否先于收缩功能障碍,目前的数据相互矛盾。我们旨在研究心脏毒性化疗后的收缩和舒张功能障碍,以及舒张功能障碍是否能预测随后出现收缩功能障碍的风险。这是一项前瞻性队列观察研究,研究对象包括接受心脏毒性化疗的患者。研究人员记录了基线、人口统计学和临床细节。分别在基线、三个月和六个月时对左心室收缩功能、整体纵向应变和舒张功能进行超声心动图测量。我们共纳入了 80 名患者。患者的平均年龄为(54.92 ± 7.6)岁,主要为女性(80%)。平均左心室射血分数从 64.92 ± 1.96 降至 6 个月时的 60.97 ± 4.94。6个月时,8名患者(10%)的射血分数偏低。基线时的平均整体纵向应变(GLS)为-18.81±0.797,6个月时降至-17.65±2.057,其中12名患者(15%)的GLS较低(<-18)。22 名患者(27.5%)出现一级舒张功能障碍,3 名患者(3.8%)在 6 个月时出现二级舒张功能障碍。三个月和六个月时,E/A 比值(血流舒张早期速度/血流舒张晚期速度)和二尖瓣组织多普勒速度明显下降,等容舒张时间、二尖瓣减速时间和 E/e′(血流舒张早期速度/二尖瓣组织多普勒二尖瓣环速度)增加。6 个月时的射血分数与 3 个月时的舒张功能障碍呈显著负相关(r = - 0.595,p = 0.02)。心脏毒性化疗与早期舒张功能障碍有关。早期舒张功能障碍可预测随后的左心室收缩功能障碍。
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引用次数: 0
The effect of significant weight loss after bariatric surgery on echocardiographic indices: an observational study focusing on left ventricular deformation by 2D speckle echocardiography and right ventricular size 减肥手术后体重明显减轻对超声心动图指标的影响:一项观察性研究,重点是二维斑点超声心动图显示的左心室变形和右心室大小
Pub Date : 2024-04-08 DOI: 10.1186/s43044-024-00474-6
Saeed Safari, Mozhgan Parsaee, Mohammad Moradi, Mahdi Hakiminejad, Parisa Koohsari, Farnoosh Larti
Obesity is a known risk factor for atherosclerosis and cardiac disease. This study evaluated the effect of significant weight loss following bariatric surgery on myocardial deformation indices and right ventricular size (RV). This was a prospective cohort study. Morbid obese patients scheduled for bariatric surgery from July 2017 to February 2018 at Firoozgar Hospital were included in our study and referred for transthoracic echocardiography at Rajaie Cardiovascular Medical and Research Center. Thirty-four patients entered the study. The absolute value of global longitudinal strain (GLS) at baseline, 3, and 6 months after surgery was 17.42 ± 2.94%, 18.24 ± 3.09%, and 19.52 ± 2.78%, respectively, with a statistically significant difference from baseline to after six months (P value < 0.001). The absolute value of global circumferential strain (GCS) at baseline, 3, and 6 months after surgery was 20.14 ± 4.22%, 23.32 ± 4.66%, and 24.53 ± 4.52%, respectively, with statistically significant changes (P value < 0.001) from baseline to three months and from baseline to six months and no significant difference from three months to six months. A significant decrease was reported in mechanical dispersion of circumferential strain (38.05 ± 23.81–23.37 ± 20.86 ms, P value = 0.006) 6 months after surgery. Right ventricular size three- and six-month post-surgery showed a significant decrease relative to baseline echocardiography. Bariatric surgery could enhance cardiac function, as proven by 2D speckle echocardiography. Changes in RV size may be related to weight loss and should be considered when assessing patients who have undergone bariatric surgery.
众所周知,肥胖是动脉粥样硬化和心脏病的危险因素。本研究评估了减肥手术后体重大幅下降对心肌变形指数和右心室大小(RV)的影响。这是一项前瞻性队列研究。2017年7月至2018年2月期间,计划在菲罗兹加医院接受减肥手术的病态肥胖患者被纳入我们的研究,并转诊至拉杰心血管医疗和研究中心接受经胸超声心动图检查。34 名患者参与了研究。基线、术后 3 个月和 6 个月时的整体纵向应变(GLS)绝对值分别为 17.42 ± 2.94%、18.24 ± 3.09% 和 19.52 ± 2.78%,从基线到 6 个月后的差异具有统计学意义(P 值 < 0.001)。术后基线、3个月和6个月时的全周应变(GCS)绝对值分别为(20.14 ± 4.22%)、(23.32 ± 4.66%)和(24.53 ± 4.52%),从基线到3个月以及从基线到6个月的变化均有统计学意义(P值< 0.001),而从3个月到6个月则无明显差异。术后 6 个月,周向应变的机械分散性(38.05 ± 23.81-23.37 ± 20.86 ms,P 值 = 0.006)明显下降。与基线超声心动图相比,手术后三个月和六个月的右心室大小明显缩小。二维斑点超声心动图证实,减肥手术可增强心脏功能。RV 大小的变化可能与体重减轻有关,因此在评估接受减肥手术的患者时应加以考虑。
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引用次数: 0
The outcome of genetic and non-genetic pediatric cardiomyopathies 遗传性和非遗传性小儿心肌病的治疗结果
Pub Date : 2024-04-03 DOI: 10.1186/s43044-024-00473-7
Ali AlAlakhfash, Luciano Agati, Giuseppe Mazzesi, Dalia Elhobi, Abdullah Alqwaiee, Khalid Alhory, Abdulrahman Almesned, Zuhair Alhasnan, Abdullah Alwadai
Pediatric cardiomyopathies (CMP) can be familial or idiopathic with increasing detection of genetic mutations. The study is a retrospective single-center review of cardiomyopathy patients from January 2011 to May 2020. Results of the genetic study, as well as the outcome, were reported. Patients were divided according to the type of CMP, age of presentation, and EF at presentation. Univariate and multivariate analysis and ROC and survival curves were done. We reported 229 patients under 14 years of age with a diagnosis of cardiomyopathy, most commonly DCM (160 patients (70%)) followed by HCM (26.2%). 52% presented at 6 months of age or less and 119 (52%) required ICU admission at presentation. The genetic and or metabolic disorder was confirmed in 21.4% of patients, most commonly VLCAD defect (16, 7%) and ELAC2 gene defect (10, 4.4%). During the disease course, 88 patients (38.4%) died (48 with DCM, 39 with HCM, and 1 with RCM). An EF of 20% or less at presentation and presentation at 6 months of age or less carries a risk for mortality in patients with DCM and HCM, respectively (RR 3.88 and 2.06 and OR of 11.09 and 4.35, respectively). Death was more common among HCM patients especially patients with positive genetic abnormality compared with patients with DCM. The mortality for CMP in children reaches up to 40%, (30% in DCM and 65% in HCM patients). Mortality was higher in those with HCM, DCM with EF of 20% or less, and HCM presented at 6 months of age or less. Whole-exome and/or whole-genome sequencing is advised for all patients of CMP and at-risk family members.
小儿心肌病(CMP)可以是家族性或特发性的,基因突变的检出率越来越高。该研究是对2011年1月至2020年5月期间的心肌病患者进行的单中心回顾性研究。报告了基因研究的结果以及治疗效果。根据 CMP 的类型、发病年龄和发病时的 EF 值对患者进行了分类。进行了单变量和多变量分析,并绘制了 ROC 曲线和生存曲线。我们报告了 229 名 14 岁以下确诊为心肌病的患者,其中最常见的是 DCM(160 名患者(70%)),其次是 HCM(26.2%)。52%的患者在 6 个月或更小的时候发病,119 人(52%)在发病时需要入住重症监护室。21.4%的患者被证实患有遗传或代谢性疾病,最常见的是VLCAD缺陷(16,7%)和ELAC2基因缺陷(10,4.4%)。在病程中,88 名患者(38.4%)死亡(48 名 DCM 患者、39 名 HCM 患者和 1 名 RCM 患者)。DCM 和 HCM 患者发病时 EF 值为 20% 或更低,以及发病时年龄为 6 个月或更低,都有死亡风险(RR 分别为 3.88 和 2.06,OR 分别为 11.09 和 4.35)。与 DCM 患者相比,HCM 患者尤其是基因异常呈阳性的患者更容易死亡。儿童 CMP 死亡率高达 40%(DCM 患者为 30%,HCM 患者为 65%)。在 HCM、EF 值为 20% 或更低的 DCM 和 6 个月或更小的 HCM 患者中,死亡率更高。建议对所有 CMP 患者和高危家庭成员进行全外显子组和/或全基因组测序。
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引用次数: 0
The value of neutrophil-to-lymphocyte ratio in predicting severity of coronary involvement and long-term outcome of percutaneous coronary intervention in patients with acute coronary syndrome: a systematic review and meta-analysis 中性粒细胞与淋巴细胞比值在预测急性冠状动脉综合征患者冠状动脉受累严重程度和经皮冠状动脉介入治疗长期疗效方面的价值:系统综述与荟萃分析
Pub Date : 2024-03-28 DOI: 10.1186/s43044-024-00469-3
Farzad Shahsanaei, Shahin Abbaszadeh, Soudabeh Behrooj, Nima Rahimi Petrudi, Bahareh Ramezani
The value of counting inflammatory cells and especially their counting ratio in predicting adverse clinical outcomes in patients with acute coronary syndrome (ACS) undergoing revascularization has been shown, but the results of studies have been very diverse and paradoxical. The aim of the current study was to systematically review the studies that investigated the role of increased neutrophil-to-lymphocyte ratio (NLR) in predicting long-term clinical outcomes in patients with acute coronary syndrome (ACS) undergoing percutaneous coronary intervention (PCI). Data abstraction was independently performed by both un-blinded reviewers on deeply assessing Medline, Web of Knowledge, Google Scholar, Scopus, and Cochrane Central Register of Controlled Trials and using the relevant keywords. The risk of bias for each study was assessed using the criteria outlined in the Cochrane Handbook for Systematic Reviews of Interventions and the QUADAS-2 tool. Statistical analysis was performed using the Stata software. Overall, 14 articles published between 2010 and 2021 were eligible for the final analysis. A total of 20,846 ACS patients undergoing PCI were included. Higher values of NLR were associated with higher numbers of involved coronaries (RR: 1.175, 95%CI 1.021–1.353, P = 0.024). Increasing the value of NLR was associated with a 3.4 times increase in long-term death (RR: 3.424, 95%CI 2.325–5.025, P = 0.001). Similarly, higher values of NLR were significantly associated with a higher likelihood of long-term MACE (RR: 2.604, 95%CI 1.736–3.906, P = 0.001). NLR has a high value in predicting both the severity of coronary artery involvement and long-term adverse clinical outcomes following the PCI procedure.
炎症细胞计数,尤其是其计数比值在预测接受血管再通手术的急性冠状动脉综合征(ACS)患者的不良临床结局方面的价值已经得到证实,但研究结果却五花八门,自相矛盾。本研究旨在系统回顾有关中性粒细胞与淋巴细胞比值(NLR)升高在预测接受经皮冠状动脉介入治疗(PCI)的急性冠状动脉综合征(ACS)患者长期临床结局中的作用的研究。数据摘要由两位非盲审稿人独立完成,他们深入评估了 Medline、Web of Knowledge、Google Scholar、Scopus 和 Cochrane Central Register of Controlled Trials,并使用了相关关键词。每项研究的偏倚风险均按照《科克伦干预措施系统综述手册》中列出的标准和 QUADAS-2 工具进行评估。统计分析使用 Stata 软件进行。共有 14 篇发表于 2010 年至 2021 年的文章符合最终分析的条件。共纳入了20846名接受PCI治疗的ACS患者。NLR值越高,受累冠状动脉的数量越多(RR:1.175,95%CI 1.021-1.353,P = 0.024)。NLR 值的增加与长期死亡人数增加 3.4 倍有关(RR:3.424,95%CI 2.325-5.025,P = 0.001)。同样,NLR 值越高,发生长期 MACE 的可能性越大(RR:2.604,95%CI 1.736-3.906,P = 0.001)。NLR 在预测冠状动脉受累的严重程度和 PCI 术后的长期不良临床结果方面具有很高的价值。
{"title":"The value of neutrophil-to-lymphocyte ratio in predicting severity of coronary involvement and long-term outcome of percutaneous coronary intervention in patients with acute coronary syndrome: a systematic review and meta-analysis","authors":"Farzad Shahsanaei, Shahin Abbaszadeh, Soudabeh Behrooj, Nima Rahimi Petrudi, Bahareh Ramezani","doi":"10.1186/s43044-024-00469-3","DOIUrl":"https://doi.org/10.1186/s43044-024-00469-3","url":null,"abstract":"The value of counting inflammatory cells and especially their counting ratio in predicting adverse clinical outcomes in patients with acute coronary syndrome (ACS) undergoing revascularization has been shown, but the results of studies have been very diverse and paradoxical. The aim of the current study was to systematically review the studies that investigated the role of increased neutrophil-to-lymphocyte ratio (NLR) in predicting long-term clinical outcomes in patients with acute coronary syndrome (ACS) undergoing percutaneous coronary intervention (PCI). Data abstraction was independently performed by both un-blinded reviewers on deeply assessing Medline, Web of Knowledge, Google Scholar, Scopus, and Cochrane Central Register of Controlled Trials and using the relevant keywords. The risk of bias for each study was assessed using the criteria outlined in the Cochrane Handbook for Systematic Reviews of Interventions and the QUADAS-2 tool. Statistical analysis was performed using the Stata software. Overall, 14 articles published between 2010 and 2021 were eligible for the final analysis. A total of 20,846 ACS patients undergoing PCI were included. Higher values of NLR were associated with higher numbers of involved coronaries (RR: 1.175, 95%CI 1.021–1.353, P = 0.024). Increasing the value of NLR was associated with a 3.4 times increase in long-term death (RR: 3.424, 95%CI 2.325–5.025, P = 0.001). Similarly, higher values of NLR were significantly associated with a higher likelihood of long-term MACE (RR: 2.604, 95%CI 1.736–3.906, P = 0.001). NLR has a high value in predicting both the severity of coronary artery involvement and long-term adverse clinical outcomes following the PCI procedure.","PeriodicalId":22462,"journal":{"name":"The Egyptian Heart Journal","volume":"52 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140316080","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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The Egyptian Heart Journal
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