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EURASIAN ASSOCIATION OF CARDIOLOGY (EAC) GUIDELINES FOR THE PREVENTION AND TREATMENT OF CARDIOVASCULAR DISEASES IN PATIENTS WITH DIABETES AND PREDIABETES (2021) 欧亚心脏病学会(eac)糖尿病和前驱糖尿病患者心血管疾病预防和治疗指南(2021)
Pub Date : 2021-05-29 DOI: 10.38109/2225-1685-2021-2-6-61
I. Chazova, M. Shestakova, Y. Zhernakova, N. Blinova, T. Markova, N. Mazurina, M. Ezhov, S. Tereshchenko, I. Zhirov, A. L. Komarov, O. Mironova, Y. Yuricheva, O. Y. Suhareva, O. Kislyak, A. Mkrtumyan, V. Podzolkov, V. Azizov, P. A. Zelveyan, E. A. Grigorenko, Z. Y. Rahimov, A. Sarybaev, S. D. Kasymova, A. Narzullaeva
The EAC Guidelines represent the views of the EAC, and were produced after careful consideration of the scientific and medical knowledge, and the evidence available at the time of their publication. The EAC is not responsible in the event of any contradiction, discrepancy, and/or ambiguity between the EAC Guidelines and any other official recommendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of healthcare or therapeutic strategies. Health professionals are encouraged to take the EAC Guidelines fully into account when exercising their clinical judgment, as well as in the determination and the implementation of preventive, diagnostic, or therapeutic medical strategies; however, the EAC Guidelines do not override, in any way whatsoever, the individual responsibility of health professionals to make appropriate and accurate decisions in consideration of each patient’s health condition and in consultation with that patient and, where appropriate and/or necessary, the patient’s caregiver. Nor do the EAC Guidelines exempt health professionals from taking into full and careful consideration the relevant official updated recommendations or guidelines issued by the competent public health authorities, in order to manage each patient’s case in light of the scientifically accepted data pursuant to their respective ethical and professional obligations. It is also the health professional’s responsibility to verify the applicable rules and regulations relating to drugs and medical devices at the time of prescription.
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引用次数: 3
ARRHYTHMIC PHENOTYPE OF NON-COMPACTION CARDIOMYOPATHY 非压实性心肌病的心律失常表型
Pub Date : 2021-05-29 DOI: 10.38109/2225-1685-2021-2-62-69
S. Komissarova, N. Chakova, N. M. Rinejskaya, T. Dolmatovich, S. Niyazova
Сведения об авторах: Комиссарова Светлана Михайловна, ведущий научный сотрудник лаборатории хронической сердечной недостаточности РНПЦ «Кардиология», доктор мед. наук, доцент, ORCID: https://orcid.org/0000-0001-9917-5932 Чакова Наталья Николаевна, ведущий научный сотрудник лаборатории генетики животных ГНУ «Институт генетики и цитологии НАН Беларуси», ORCID: https://orcid.org/0000-0003-4721-9109 Долматович Татьяна Владимировна, ведущий научный сотрудник лаборатории генетики животных ГНУ «Институт генетики и цитологии НАН Беларуси», ORCID: https://orcid.org/0000-0001-7562-131X Ниязова Светлана Сергеевна, младший научный сотрудник лаборатории генетики животных ГНУ «Институт генетики и цитологии НАН Беларуси», ORCID: https://orcid.org/0000-0002-3566-7644 *Автор, ответственный за переписку: Ринейская Надежда Михайловна, младший научный сотрудник лаборатории хронической сердечной недостаточности РНПЦ «Кардиология», 220036, Минск, ул. Р.Люксембург 110Б, РНПЦ “Кардиология”, тел.+375(29)340-87-88, e-mail: nadya.rin@ gmail.com, ORCID: https://orcid.org/0000-0002-1986-1367 ГЕНЕТИЧЕСКИЕ ПРИЧИНЫ АРИТМИЧЕСКОГО ФЕНОТИПА НЕКОМПАКТНОЙ КАРДИОМИОПАТИИ
作者:斯维特拉娜·米哈伊洛夫娜,慢性心力衰竭实验室的首席研究员,医学博士。科学,副教授,ORCID:ORCID是动物遗传学家南白俄罗斯遗传学和细胞学研究所的初级研究员。r .卢森堡110b, rprc“心脏病学”,尸体+375(29)340-87-88,电子邮件:nadya。不规则心肌病的遗传原因
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引用次数: 1
METABOLOMIC APPROACHES IN STUDYING OF CARDIOVASCULAR DISEASES 代谢组学在心血管疾病研究中的应用
Pub Date : 2021-02-28 DOI: 10.38109/2225-1685-2021-1-106-117
A. A. Аbdullaev, G. J. Аbdullaeva, Kh. F. Usupova
Modern scientific approaches give clinicians an advantage in using additional tools and methods for assisting in clinical assessment and expanding their capabilities for classifying patients according to risk factors for cardiovascular complications. Biomarkers are a simple tool that allows to identify and classify people with different risk degree, quickly and accurately diagnose the condition of the disease, effectively predict and control treatment. Therefore, the identification and study of biomarkers is a serious and promising approach to understanding and treating cardiovascular diseases (CVD). Among a wide range of biomarkers, the genetic and metabolic markers is of high importance. Cardio-metabolomics is a new direction in cardiovascular science that allows researchers to study changes in metabolome and metabolic networks in diseases of the cardiovascular system in order to better understand their pathophysiological mechanism. Thus, the study of metabolome can provide important information about the pathogenesis of CVDs, as well as offer the possibility of identifying new CVD biomarkers.
现代科学方法使临床医生在使用额外的工具和方法来协助临床评估和扩大他们根据心血管并发症危险因素对患者进行分类的能力方面具有优势。生物标志物是一种简单的工具,可以识别和分类不同风险程度的人,快速准确地诊断疾病的状况,有效地预测和控制治疗。因此,识别和研究生物标志物是了解和治疗心血管疾病的重要途径。在众多的生物标志物中,遗传和代谢标志物具有重要意义。心血管代谢组学是心血管科学的一个新方向,它使研究人员能够研究心血管系统疾病中代谢组学和代谢网络的变化,以便更好地了解其病理生理机制。因此,代谢组学的研究可以为CVD的发病机制提供重要信息,并为发现新的CVD生物标志物提供可能。
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引用次数: 1
EURASIAN ASSOCIATION OF CARDIOLOGY (EAC) GUIDELINES FOR THE DIAGNOSIS AND TREATMENT OF CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION (2020) 欧亚心脏病学会慢性血栓栓塞性肺动脉高压诊断和治疗指南(2020)
Pub Date : 2021-02-28 DOI: 10.38109/2225-1685-2021-1-6-43
I. Chazova, T. Martynyuk, Z. Valieva, V. Azizov, R. Akchurin, A. Ansheles, O. Vasiltseva, T. Veselova, A. Galyavich, S. Gorbachevsky, N. Danilov, A. Edemskiy, P. A. Zelveyan, I. Lazareva, Y. Matchin, K. Mershin, M. Mukarov, S. Nakonechnikov, M. Saidova, A. Sarybaev, Sergienko Vb, O. Stukalova, E. Filippov, A. Chernyavsky, M. A. Chernyavsky, S. Shalaev, A. A. Shmalts
Disclaimer The EAC Guidelines represent the views of the EAC, and were produced after careful consideration of the scientific and medical knowledge, and the evidence available at the time of their publication. The EAC is not responsible in the event of any contradiction, discrepancy, and/or ambiguity between the EAC Guidelines and any other official recommendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of healthcare or therapeutic strategies. Health professionals are encouraged to take the EAC Guidelines fully into account when exercising their clinical judgment, as well as in the determination and the implementation of preventive, diagnostic, or therapeutic medical strategies; however, the EAC Guidelines do not override, in any way whatsoever, the individual responsibility of health professionals to make appropriate and accurate decisions in consideration of each patient’s health condition and in consultation with that patient and, where appropriate and/or necessary, the patient’s caregiver. Nor do the EAC Guidelines exempt health professionals from taking into full and careful consideration the relevant official updated recommendations or guidelines issued by the competent public health authorities, in order to manage each patient’s case in light of the scientifically accepted data pursuant to their respective ethical and professional obligations. It is also the health professional’s responsibility to verify the applicable rules and regulations relating to drugs and medical devices at the time of prescription.
EAC指南代表了EAC的观点,是在仔细考虑了科学和医学知识以及出版时可用的证据后编制的。如果EAC指南与相关公共卫生当局发布的任何其他官方建议或指南之间存在任何矛盾、差异和/或歧义,特别是在良好使用医疗保健或治疗策略方面,EAC概不负责。鼓励卫生专业人员在进行临床判断时,以及在确定和实施预防、诊断或治疗性医疗策略时,充分考虑《EAC指南》;然而,《EAC指南》并没有以任何方式推翻卫生专业人员的个人责任,即考虑到每个病人的健康状况,并与病人以及在适当和/或必要时与病人的照顾者协商,作出适当和准确的决定。《EAC准则》也不能免除卫生专业人员充分和仔细考虑主管公共卫生当局发布的有关官方最新建议或准则,以便根据科学接受的数据根据各自的道德和专业义务管理每个病人的病例。卫生专业人员也有责任在开处方时核实与药品和医疗器械有关的适用规则和条例。
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引用次数: 16
CARDIOLOGICAL ASPECTS OF THE PERIOPERATIVE MANAGEMENT OF PATIENTS WITH CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION FOR PULMONARY THROMBOENDARTERECTOMY 慢性血栓栓塞性肺动脉高压患者肺血栓动脉内膜切除术围手术期管理的心脏学方面
Pub Date : 2021-01-01 DOI: 10.38109/2225-1685-2020-3-94-104
D. D. Tsyrenov, R. Akchurin, K. Mershin, E. A. Tabakyan, E. Vlasova, V. V. Gazizоv, S. Kurbanov, I. Starostin
Aim. To analyze the factors associated with a poor outcome of pulmonary thromboendarterectomy (PTE) and complications of the hospital postoperative period; on the basis of this analysis to optimize preoperative preparation and therapeutic support of the operation. Materials and methods. The study included 47 patients with operable CTEPH, who underwent PTE in the Department of cardiovascular surgery of the national medical research center of cardiology from 2010 to 2018. Patients were observed during the intrahospital period, all were evaluated for clinical, instrumental, hemodynamic, and laboratory parameters. Diagnosis and treatment of complications, assessment of the relationship of factors associated with the development of these pathological conditions were carried out. Results. A comprehensive assessment of the parameters revealed that age over 50 years, the presence of proven antiphospholipid syndrome (AFS) were independently associated with a higher frequency of adverse surgical outcomes and in-hospital complications. Older age and a history of smoking were independently associated with a greater likelihood of developing reperfusion pulmonary edema. The probability of developing transient neurological complications is independently associated with a long duration of deep hypothermic circulatory arrest (DHCA), an increased level of D-dimer. A greater age and longer duration of ventilation are independently associated with the likelihood of developing acute kidney injury (AKI). A higher level of antithrombin III and the presence of AFS were independently associated with the likelihood of developing prolonged ventilation. Conclusion. When selecting candidates for surgery, in addition to the generally accepted clinical and instrumental parameters, it is necessary to take into account a history of Smoking, an increase in d-dimer, and the presence of AFS. Patients with this pathology need a more thorough risk assessment, correction of target levels of activated partial thromboplastin time (aPTT), activated clotting time (ACT) due to their falsely inflated indicators, and further development of standards for perioperative support. The main principle of cardiological support of the operation is the earliest possible diagnosis of all known perioperative complications and the rapid start of their treatment, which ensures the stabilization of the patient’s condition in 85% of cases in the hospital period. In the postoperative period, an early transition from ventilator to independent breathing is indicated for the prevention of associated complications, including AKI.
的目标。分析肺血栓动脉内膜切除术(PTE)预后不良及术后并发症的相关因素;在此基础上优化手术的术前准备和治疗支持。材料和方法。本研究纳入了2010年至2018年在国家心脏病医学研究中心心血管外科接受PTE治疗的47例可手术CTEPH患者。在住院期间对患者进行观察,对所有患者的临床、仪器、血流动力学和实验室参数进行评估。进行并发症的诊断和治疗,评估与这些病理状况发展相关的因素的关系。结果。对这些参数的综合评估显示,年龄超过50岁、已证实存在抗磷脂综合征(AFS)与较高频率的不良手术结果和院内并发症独立相关。年龄较大和吸烟史与发生再灌注肺水肿的可能性较大独立相关。发生短暂性神经系统并发症的可能性与深度低温循环骤停(DHCA)持续时间长、d -二聚体水平升高独立相关。较大的年龄和较长的通气时间与发生急性肾损伤(AKI)的可能性独立相关。较高水平的抗凝血酶III和AFS的存在与发展为延长通气的可能性独立相关。结论。在选择手术候选人时,除了普遍接受的临床和仪器参数外,还需要考虑吸烟史、d-二聚体的增加和AFS的存在。有这种病理的患者需要进行更彻底的风险评估,校正活化部分凝血酶活时间(aPTT)和活化凝血时间(ACT)的目标水平,因为他们的指标被错误地夸大了,并进一步制定围手术期支持的标准。手术的心脏学支持的主要原则是尽可能早地诊断所有已知的围手术期并发症并迅速开始治疗,这确保85%的病例在住院期间病情稳定。术后应尽早从呼吸机过渡到独立呼吸,以预防相关并发症,包括AKI。
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引用次数: 0
INACTIVATION OF RENIN-ANGIOTENSIN-ALDOSTERONE SYSTEM. WHICH CLASS OF ANTIHYPERTENSIVE MEDICINE PRODUCTS TO PREFER? 肾素-血管紧张素-醛固酮系统失活。选择哪一类降压药产品?
Pub Date : 2020-12-01 DOI: 10.38109/2225-1685-2020-4-64-78
СИСТЕМЫ. Какой Класс, Препаратов Предпочесть, S. V. Stolov
Сведения об авторе: Столов Сергей Валентинович, д.м.н., заведующий кафедрой терапии, МСЭ и реабилитации No1 ФГБУ ДПО «Санкт-Петербургский институт усовершенствования врачей-экспертов» Минтруда России, Санкт-Петербург; профессор кафедры терапии и ревматологии им. Э.Э. Эйхвальда ФГБУ ВО «Северо-Западный государственный медицинский университет», e-mail: sv100lov@gmail.com; sergey.stolov@szgmu.ru, ORCID: 00000002-3431-1224 ИНАКТИВАЦИЯ РЕНИН-АНГИОТЕНЗИН-АЛЬДОСТЕРОНОВОЙ СИСТЕМЫ. КАКОЙ КЛАСС ПРЕПАРАТОВ ПРЕДПОЧЕСТЬ?
关于作者的资料:谢尔盖·瓦伦蒂诺维奇,d.m.,圣彼得堡劳工部“圣彼得堡医学专家改进研究所”心理治疗、中小企业和康复部主任;他是治疗和风湿病学系的教授。ehwalda fgbu在西北州立医学院,电子邮件:sv100lov@gmail.com;sergey.stolov@szgmu.ru, ORCID: renin -血管生成- aldosteran系统的失活。你想要哪种药物?
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引用次数: 2
ORTHOSTATIC HYPOTENSIVE REACTIONS IN ELDERLY AND SENILE PATIENTS WITH ARTERIAL HYPERTENSION 老年及老年高血压患者的体位性低血压反应
Pub Date : 2020-12-01 DOI: 10.38109/2225-1685-2020-4-52-63
I. Atyunina, E. Oshchepkova, A. Rogoza
Сведения об авторах: *Автор, ответственный за связь с редакцией: Атюнина Ирина Валентиновна, мл. науч. сотрудник лаборатории мониторинга снижения смертности от ССЗ научно-организационного отдела ФГБУ «НМИЦ кардиологии» Минздрава России, atyunina_i@mail.ru, 121552, Москва, ул. 3-я Черепковская, д. 15 а; ORCID: 0000-0002-7771-172X Ощепкова Елена Владимировна, д-р мед. наук, профессор, главный научный сотрудник отдела гипертонии ФГБУ «НМИЦ кардиологии» Минздрава России; ORCID: 0000-0003-4534-9890 Рогоза Анатолий Николаевич, д-р биол. наук, профессор, руководитель отдела новых методов диагностики, ФГБУ «НМИЦ кардиологии» Минздрава России; ORCID: 0000-0002-0543-3089 ОРТОСТАТИЧЕСКИЕ ГИПОТЕНЗИВНЫЕ РЕАКЦИИ У БОЛЬНЫХ ПОЖИЛОГО И СТАРЧЕСКОГО ВОЗРАСТА С АРТЕРИАЛЬНОЙ ГИПЕРТОНИЕЙ
作者:*负责编辑的作者:小阿图宁·伊琳娜·瓦伦丁诺夫娜。学会。俄罗斯卫生部科学组织科技部(atyunina_i@mail.ru)第3个骷髅头,第15个a ?ORCID: 0000-0002-7771科学,教授,俄罗斯卫生部高血压部门首席研究员;ORCID: 0000-0003-4534-9890罗戈萨·阿纳托利·尼古拉耶维奇,生物博士。科学,教授,新诊断方法部门主管,俄罗斯卫生部心脏病学委员会;ORCID: 10 -0002-0543-3089动脉高血压患者的直立低血压反应
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引用次数: 0
MARKERS OF RISK FACTORS FOR CARDIOVASCULAR DISEASE 心血管疾病危险因素的标记
Pub Date : 2020-09-30 DOI: 10.38109/2225-1685-2020-3-78-84
N. Bashirov
Objective: To study the role of risk factors for cardiovascular diseases to optimize their pathogenetic diagnosis.Material and research methods. At the initial stage of clinical trials, all participants (n = 200) were questioned with age-sex characteristics and the main risk factors for cardiovascular diseases (alcohol abuse, smoking, obesity, stress). Biochemical and clinical studies were carried out in two groups of patients, 15 people in each group (the first main group - in addition to bad habits, there is an obesity factor, the second control group - bad habits are detected, but in the absence of obesity) as part of preventive medical examinations.The results of research. When studying psychosocial risk factors for the development of diseases of the cardiovascular system, a higher prevalence rate was noted for men in depression indicators - 26.3% of cases, and in the group of female patients the results were almost 2.5 times lower and amounted to only 15.0%. in both groups of healthy individuals, a low level of the frequency of occurrence of overweight and obesity was observed, compared with the main group, which were diagnosed in 28.6% of cases in the male half of the subjects, and among the representatives of the opposite sex, the indicator was 24.0%. The proportion of obese women in the main and control groups was higher than men by almost 1.5 times. Against the background of obesity and the presence of signs of non-alcoholic fatty degeneration of the liver, in comparison with patients with normal body weight, certain violations were detected in the form of a higher level in the blood of the liver enzyme ALAT - 35.2 ± 1.57 U / L and 21.3 ± 0 95 U / L, respectively, in the first main group and in the second control group.Conclusions. Thus, it was found that the cause of cardiovascular diseases, in particular, cardiac automatism disorders, stroke, myocardial infarction due to metabolic and psychoemotional disorders are overweight and obesity, which worsen the prognosis of CVD, creating the prerequisites for the development of complications.
目的:探讨心血管疾病危险因素的作用,优化其病理诊断。材料和研究方法。在临床试验的初始阶段,所有参与者(n = 200)都被询问了年龄-性别特征和心血管疾病的主要危险因素(酗酒、吸烟、肥胖、压力)。在两组患者中进行了生化和临床研究,每组15人(第一主要组-除了坏习惯外,还有肥胖因素,第二对照组-检测到坏习惯,但没有肥胖)作为预防性医学检查的一部分。研究的结果。在研究心血管系统疾病发展的社会心理风险因素时,在抑郁症指标中,男性的患病率较高,为26.3%,而在女性患者组中,结果几乎低2.5倍,仅为15.0%。在两组健康个体中,超重和肥胖的发生频率都较低,与主要组相比,男性一半受试者中有28.6%的病例被诊断为超重和肥胖,而在异性代表中,这一指标为24.0%。在主要和对照组中,肥胖女性的比例几乎是男性的1.5倍。在肥胖和存在肝脏非酒精性脂肪变性迹象的背景下,与体重正常的患者相比,在第一主要组和第二对照组中,肝脏酶ALAT的血液水平较高,分别为35.2±1.57 U / L和21.3±0.95 U / L。因此,我们发现心血管疾病,特别是心脏自动性疾病、中风、代谢和心理情绪障碍引起的心肌梗死的病因是超重和肥胖,这使CVD的预后恶化,为并发症的发生创造了先决条件。
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引用次数: 1
CONTRAST-INDUCED NEPHROPATHY IN PATIENTS WITH STABLE CORONARY ARTERY DISEASE AND 1-YEAR PROGNOSIS 稳定冠状动脉疾病患者造影剂肾病及1年预后
Pub Date : 2020-09-30 DOI: 10.38109/2225-1685-2020-3-100-105
O. Mironov, O. Sivakova, V. Fomin
Aim.The aim of our study was to assess the prevalence of contrast-induced nephropathy (CIN) in patients with chronic coronary artery disease (CAD) and its 1-year prognostic significance.Materials and methods.462 patients with chronic CAD and indications to the interventions with intraarterial contrast media administration were included in the study. We conducted a prospective open cohort study (ClinicalTrials.gov NCT04014153). The primary endpoint was the development of CIN. The secondary endpoints were total mortality, cardiovascular mortality, myocardial infarction, stroke, gastrointestinal bleeding, acute decompensation of heart failure, coronary artery bypass grafting, repeat percutaneous coronary intervention.Results.28 patients (6%) developed CIN. The rate of CIN in female patients was twice higher, than in males (9,29% vs. 4,66%). There was a trend towards less cases of CIN in patients without obesity (5,88% vs. 6,22%). CIN developed more frequently in patients with anemia (8,9% и 5,7%, р=0,3649, ОR 1,633,95% CI 0,6507-4,239). There was a trend to higher incidence of CIN in people with hyperuricemia (8% vs. 5,95%, р=0,6575, ОR 1,375,95% CI 0,3055-5,808). The rate of CIN in patients with diabetes mellitus was 2% higher, then without one. People, who suffered from myocardial infarction after 1 year of follow up, had the highest rate of CIN (26,7%), as well as patients with other major cardiovascular complications (18,1%). The rate of repeat percutaneous coronary interventions was 9,5%, the rate of acute decompensation of heart failure was 7%.Conclusion.The prevalence of CIN in patients with chronic CAD was 6%. After 1 year of follow up the rate of CIN was higher in patients who had myocardial infarction, repeat percutaneous coronary interventions and acute decompensation of heart failure.
的目标。本研究的目的是评估慢性冠状动脉疾病(CAD)患者造影剂肾病(CIN)的患病率及其1年预后意义。材料和方法。本研究纳入462例慢性CAD患者及动脉内造影剂干预适应症。我们进行了一项前瞻性开放队列研究(ClinicalTrials.gov NCT04014153)。主要终点是CIN的发展。次要终点为总死亡率、心血管死亡率、心肌梗死、脑卒中、胃肠道出血、心力衰竭急性失代偿、冠状动脉旁路移植术、重复经皮冠状动脉介入治疗。28例(6%)发生CIN。女性患者的CIN发生率是男性的2倍(9.29% vs. 4.66%)。非肥胖患者的CIN病例有减少的趋势(5.88% vs. 6.22%)。贫血患者发生CIN的频率更高(8.9% vs 5.7%, r =0,3649, ОR 1,633,95% CI 0,6507-4,239)。高尿酸血症患者的CIN发生率有升高的趋势(8% vs. 5,95%, r =0,6575, ОR 1,375,95% CI 0,3055-5,808)。糖尿病患者CIN发生率比无糖尿病患者高2%。随访1年后发生心肌梗死的患者CIN发生率最高(26.7%),其他主要心血管并发症患者CIN发生率最高(18.1%)。经皮冠状动脉介入治疗的重复率为9.5%,急性心力衰竭失代偿率为7%。慢性CAD患者中CIN的发生率为6%。随访1年后,心肌梗死、多次经皮冠状动脉介入治疗和心力衰竭急性失代偿的患者CIN发生率较高。
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引用次数: 0
CLINICAL CASE OF NON-COMPACTION CARDIOMYOPATHY IN A PATIENT WITH PKP2 GENE MUTATION pkp2基因突变致非压实性心肌病1例
Pub Date : 2020-09-30 DOI: 10.38109/2225-1685-2020-3-106-111
S. Komissarova, N. Rineiskaya, N. Chakova, S. Niyazova, T. Dolmatovich
The features of the clinical course of non-compaction cardiomyopathy, its prognosis and even its diagnosis are still the subject of discussion. The variety of phenotypes of this disease and genetic heterogeneity dictates the need for detailed clinical and molecular genetic examination of patients with this pathology. The article presents a clinical observation of a patient with a dilated phenotype of left ventricular non-compaction, progression of chronic heart failure, as well as the presence of ventricular rhythm and conduction disorders that required the implantation of cardiac resynchronization therapydefibrillator (CRT-D). The patient was found to have a missense mutation c. 1892A>G (p.Tyr631Cys, rs1060501183) in PKP2 gene in a heterozygous state. The issues of differential diagnostics with arrhythmogenic right ventricular cardiomyopathy and treatment strategies for the disease were discussed.
非压实性心肌病的临床病程特点、预后甚至诊断仍有争议。这种疾病表型的多样性和遗传异质性决定了需要对患有这种病理的患者进行详细的临床和分子遗传学检查。本文介绍了一例左心室不压实扩张型患者的临床观察,慢性心力衰竭的进展,以及心室节律和传导障碍的存在,需要植入心脏再同步治疗除颤器(CRT-D)。患者在杂合状态下发现PKP2基因c. 1892A>G (p.Tyr631Cys, rs1060501183)错义突变。本文讨论了致心律失常性右室心肌病的鉴别诊断和治疗策略。
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引用次数: 0
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Eurasian heart journal
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