Shortened PQ interval in the differential diagnosis of Anderson-Fabry disease: a case report

In this article, we present a case of a patient with a late diagnosis of Fabry disease caused by a pathogenic variant in the GLA gene (p.1287_1288dup), who repeatedly attempted interventional treatment of Wolff-Parkinson-White Syndrome due to characteristic electrocardiographic pattern of ventricular preexcitation and paroxysmal arrhythmias. The proposed pathognomonic signs of the disease will ensure timely diagnosis and the appointment of specific treatment

Клинико-экономический анализ и оценка влияния на бюджет применения имплантируемых кардиовертеров-дефибрилляторов в Российской Федерации

Objective: to evaluate cost-effectiveness and budget impact of using single and dual chamber implantable cardioverter-defibrillators (ICD) adjunctive to the standard drug therapy (DT) compared to the standard DT alone for the primary and secondary prevention of sudden cardiac death (SCD).Material and methods. Original partitioned survival analysis model was developed to assess the cost-effectiveness of using ICD within the modelling horizon of 8 years. The following model outcomes were used: life years and quality-adjusted life years (QALY). Primary prevention model was focused on patients after myocardial infarction with left ventricular ejection fraction (LVEF) ≤30%, whilst secondary prevention model considered cardiac arrest survivors and/or patients diagnosed with ventricular tachycardia or ventricular fibrillation with LVEF ≤35%. The model summarizes treatment effect and costs for ICD and DT specific to the healthcare system of the Russian Federation (RF). The main scenario accounted for ICD implantation cost in accordance with general reimbursement price asserted in the high technology medical care list part 2 (HТMC 2). Additionally, alternative scenario of ICD reimbursement level was developed to account for general tariff split onto singleand dual-chamber ICD implantation reimbursement tariffs which can be financed through high technology medical care list part 1 (HТMC 1). Budget impact analysis compared the costs of using ICD within the current volume of the annual increase in ICD implantations and a threefold increased volume of ICD implantations.Results. By the end of the modelling period, additional 34% of patients survived in the ICD group compared to the DT group. Incremental cost-effectiveness ratio (ICER) per 1 QALY constituted 2.8 and 2.2 million rubles for primary and secondary prevention, respectively. ICER values are slightly above or lower than the willingness-to-pay threshold of 2.5 million rubles per 1 QALY in the RF in the segment of primary and secondary SCD prevention, respectively. Additional HТMC 1 scenario incorporating lower ICD implantation prices resulted in an average ICER drop by 13% compared to HTMC 2. Overall patient population requiring SCD prevention comprised of 7,161 and 3,341 patients in primary and secondary prevention, respectively. Budget impact analysis showed that threefold rise in the ICD implantations rate will require additional 648 million rubles for primary prevention cohort to provide additional 573 patients with ICD, and 230 million rubles for secondary prevention cohort with additional 267 patients covered with ICD. ICD reimbursement price drop within the HТMC 1 scenario will save 133 million rubles and allow to provide additional 143 patients with ICDs for a given budget.Conclusion. ICD is a cost-effective option of secondary prevention of SCD. Additional analysis of ICD reimbursement price drop drives ICER downwards to a considerable extent which in turn increases the accessibility of ICDs to patients. In scenario of ICD implantation financing within HТMC 1, ICD is established to be a cost-effective option for primary and secondary prevention of SCD in the RF.Цель: оценка клинико-экономической эффективности и анализ влияния на бюджет (АВБ) применения одно- и двухкамерных имплантируемых кардиовертеров-дефибрилляторов (ИКД) в сочетании со стандартной лекарственной терапией (ЛТ) по сравнению со стандартной ЛТ для первичной и вторичной профилактики внезапной сердечной смерти (ВСС).Материал и методы. Построена оригинальная модель распределенной выживаемости пациентов с риском ВСС для проведения анализа «затраты–эффективность» с горизонтом моделирования 8 лет. В качестве исходов модели были использованы годы жизни и годы жизни с поправкой на качество (англ. quality-adjusted life year, QALY). Модель первичной профилактики ВСС включала пациентов после инфаркта миокарда с фракцией выброса левого желудочка (ФВЛЖ) 30% и менее, модель вторичной профилактики ВСС – больных после остановки сердца и/или имеющих желудочковую тахикардию или фибрилляцию желудочков с ФВЛЖ 35% и менее. Модель позволяет прогнозировать затраты на лечение и исходы пациентов, использующих ИКД или ЛТ в условиях системы здравоохранения Российской Федерации (РФ). Основной сценарий учитывает стоимость имплантации прибора по единому тарифу для всех типов ИКД в рамках второго перечня высокотехнологичной медицинской помощи (ВМП 2). Дополнительно моделировали сценарий со снижением тарифа на имплантацию ИКД за счет разгруппировки существующего единого тарифа на два: для однои двухкамерных ИКД в отдельности в рамках первого перечня ВМП (ВМП 1). С помощью АВБ сравнивали затраты на использование ИКД в рамках текущего объема ежегодного прироста имплантаций ИКД и повышенного (трехкратного) объема прироста.Результаты. На конец горизонта моделирования дополнительный прирост выживаемости в группе ИКД по сравнению с группой ЛТ составил 34%. Инкрементальный показатель «затраты–эффективность» (англ. incremental cost-effectiveness ratio, ICER) за 1 QALY в основном сценарии составил 2,8 и 2,2 млн руб. в сегментах первичной и вторичной профилактики ВСС соответственно. Полученное значение по первичной профилактике незначительно превышает, а по вторичной профилактике находится ниже референтного значения ICER (порога готовности платить), составляющего в РФ 2,5 млн руб. за 1 QALY. Моделируемое снижение стоимости тарифа на установку ИКД в рамках перечня ВМП 1 улучшает затратную эффективность (снижает ICER) в среднем на 13% от сценария ВМП 2. Суммарная популяция пациентов, нуждающихся в первичной и вторичной профилактике ВСС, составляет около 7161 и 3341 человека соответственно. Моделирование трехкратного прироста числа ИКД по отношению к текущим объемам обеспеченности в АВБ позволяет дополнительно обеспечить 573 пациента в рамках первичной профилактики ВСС, затратив дополнительно 638 млн руб., и 267 пациентов в рамках вторичной профилактики ВСС при размере дополнительных затрат 230 млн руб. Снижение стоимости имплантации ИКД в сценарии ВМП 1 способствует повышению доступности данной технологии за счет высвобождения дополнительных средств в размере 133 млн руб., позволяющих выполнить дополнительные операции по установке приборов ИКД 143 пациентам при первичной и вторичной профилактике ВСС суммарно.Заключение. ИКД является затратно-эффективной технологией в сегменте вторичной профилактики ВСС. Снижение стоимости ИКД в результате разгруппировки тарифа ВМП 2 значительно повышает клинико-экономическую эффективность данной технологии и способствует ее доступности для пациентов. Таким образом, при финансировании имплантаций по разгруппированным тарифам в рамках перечня ВМП 1 ИКД является затратно-эффективной опцией первичной и вторичной профилактики ВСС в РФ

Predictors of sustained isolation of pulmonary vein ostia with cryoballoon ablation using a second-generation cryoballoon Arctic Front Advance

Aim. To determine the electrophysiological and biophysical predictors of sustained isolation of pulmonary vein (PV) ostia, affecting the immediate and longterm results of interventional treatment of atrial fibrillation (AF) using cryoballoon ablation (CBA) with the second-generation cryoballoon Arctic Front Advance.Material and methods. The analysis included 143 PV in 37 patients with a paro-xysmal/persistent form of AF, which, after the primary CBA with the use of the second-generation cryoballoon, carried out additional interventions due to recurrence of AF and/or combined arrhythmia. During the primary CBA, the registration of biophysical and electrophysiological parameters of the procedure was performed. During the ablation in the right PV from the diagnostic electrode installed in the superior vena cava, the ipsilateral phrenic nerve was stimulated (2000 ms, 25 mA). When the phrenic nerve response to stimulation was decreased/disappeared, ablation ceased instantly. At the end of the procedure, isolation control of the PV was performed. A repeat/additional procedure was performed for the recurrence of AF and/or documented combined arrhythmia no earlier than 3 months after the initial ablation. The stability of the LV isolation was assessed using a circular mapping technique. According to the results of mapping, drugs were divided into 2 groups: isolated and with recurrent activity.Results. The frequency of sustained isolation of PV was 67,8%. Recurrent spike activity was recorded in 46 PV (32,2%). Higher frequency of registration of electrical isolation of PV in real time (68% vs 50%, p=0,001), stability of occlusion with a cryoballoon (85,5% vs 69,5%, p=0,024), low values of the minimum cryoballoon temperatures reached (49,2±6,3 vs 44,0±4,9, p<0,0001) and less need for additional applications (8,3% vs 34,7%, p<0,0001) were registered in the group of chronic isolation of PV. Multi-factor analysis of these parameters confirmed the predictor role of the minimum cryoballoon temperature. According to the ROC analysis, the threshold minimum temperature was 45,5° C with a sensitivity of 68% and a specificity of 60,9%.Conclusion. Cryoballoon isolation of PV ostia is an effective and safe method for achieving chronic isolation of PV. The minimum cryoballoon temperature with a threshold value of <-45,5° C is an independent predictor of long-term isolation of PV

THE FIRST EXPERIENCE OF LEFT ATRIAL APPENDAGE OCCLUDING DEVICES IMPLANTATION FOR PREVENTION OF STROKE IN THE STATE RESEARCH CENTRE FOR PREVENTIVE MEDICINE

Some aspects of prevention of thromboembolic events in patients with atrial fibrillation, in particular, the use of implantable left atrial appendage occluding devices are discussed. Along with the literature review on this issue, the results of authors own experience of left atrial appendage occluding devices implantation is presented.</p

THE FIRST EXPERIENCE OF LEFT ATRIAL APPENDAGE OCCLUDING DEVICES IMPLANTATION FOR PREVENTION OF STROKE IN THE STATE RESEARCH CENTRE FOR PREVENTIVE MEDICINE

Some aspects of prevention of thromboembolic events in patients with atrial fibrillation, in particular, the use of implantable left atrial appendage occluding devices are discussed. Along with the literature review on this issue, the results of authors own experience of left atrial appendage occluding devices implantation is presented

A CASE OF THE LEFT ATRIUM APPENDAGE OCCLUSION, SUGGESTIONS ON THE CLINICAL FIELD DEVELOPMENT

Aim. To evaluate the possibility of hybrid procedure that includes single-moment cryoballoon isolation of pulmonary veins (PV) ostia and implantation of occluder into the left atrium appendage (LAA) in patients of high thromboembolic and hemorrhagic risk and prominent symptoms in atrial fibrillation (AF) paroxysms.Material and methods. From 2012 to 2016 y., 72 procedures performed, of LAA occluder implantation for patients with high thromboembolic and hemorrhagic risk (mean age 68±7,4 y. o., 48 females, 24 males, mean rate by CHA2DS2VASc 4,82±1,48 points), of those 3 underwent single-moment cryoballoon isolation of the PV ostia without electrical isolation of LAA. The parameters of the intervention were assessed, and prevalence of intra-operation complications in AF patients that included single-moment catheter cryoballoon isolation of PV ostia and implantation of LAA occluding device, in comparison with isolated LAA occluder implantation.Results. Occluding device implantation was successful in 100% cases. Periprocedural efficacy of PV ostia isolation was 100%. In both groups, intraoperational complications were absent. Mean heparin time and dosage of contrast in the hybrid intervention group were higher: 94,3±6,8 min vs 59,7±19,95 min (p&lt;0,05), 196,8±20,82 mL vs 124,6±30,24 mL (p&lt;0,05), respectively.Conclusion. Performing of the hybrid intervention that includes single-moment catheter isolation of PV ostia and implantation of the occluding device into LAA increases mean procedure duration and contrast load in comparison to isolated LAA occluder implantation, with no operational risk increase

The role of inflammatory theory in the pathogenesis of atrial fibrillation

Based on the data available in the literature, it is obvious that inflammation plays a significant role in the initiation, support and progression of AF. However, it is important that AF by itself is a factor provoking the development of inflammation in the atrial myocardium, which leads to the further progression of the disease according to the principle of “AF produces AF.” The question of the primacy of the development and correlations of process links is still not fully understood: does inflammation leads to fibrillation or does fibrillation leads to inflammation?The authors analyzed the data of the world literature and tried to describe the main concepts of the inflammatory theory of AF and the practical aspects of its application

Relationship between serum inflammatory markers and recurrent atrial fibrillation in patients undergoing pulmonary vein isolation

Aim. To study the baseline level of inflammatory markers (IM) and proteins of serum proteolytic system in patients scheduled for pulmonary vein isolation, and to analyze the potential of IM to predict the arrhythmogenic foci outside the PV sleeves — non-pulmonary vein dependent atrial fibrillation (AF).Material and methods. The study included 100 patients with paroxysmal (n=89) and persistent (n=11) AF who underwent the primary PV cryoballoon ablation with the simultaneous implantation of a loop recorder (Reveal XT, Medtronic). Before the procedure, we analyzed following parameters in all patients: N-terminal pro-brain natriuretic peptide (NTproBNP), matrix metalloproteinases 1, 3, 9, tissue inhibitor of matrix metalloproteinase 1, fibroblast growth factor-2, fatty acid binding protein 3, transforming growth factor p1, tumor necrosis factor a, interleukin 1p.Results. A significant negative predictive value of baseline NTproBNP (hazard ratio, 1,00053, p=0,00935) for recurrent AF (recurrence risk increases by 8,4% with an increase in NTproBNP level by 100 pg/ml) was demonstrated. For the rest of the parameters, there was no significant effect on recurrence risk in the postoperative period.Conclusion. The role of IM in the AF pathogenesis should be studied further, taking into account the recommended sample sizes to assess their predictive ability in relation to preoperative detection of patients with non-pulmonary vein dependent