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Background: In patients with HCM at high risk of SCD, an ICD should be considered as a standard of care. Current risk approximation algorithms recommended by ESC 2014 criteria indicate that SCD risk is not stable. The aim of the study was to investigate how the calculated SCD risk in HCM patients with an ICD implanted in the past changed over time. Methods: We analyzed 64 HCM patients with ICD for primary prevention, referred for ICD re-implantation, and 32 HCM patients referred for a first-time ICD placement during the same period. The 5-year-SCD risk was assessed for suitable patients using the recommended ESC calculator. Results: The first-time group had a higher 5-year-SCD risk than those referred for ICD re-implantation: 7.50 (IQR 5.98–10.46) vs. 4.88 (IQR 3.42–7.25), p < 0.05. Out of the patients with an initial calculated risk below 4%, the risk increased in 22% of cases, reaching the 4–6% range. In 78% of patients, the risk remained stable and low. In 31% of patients with an initial calculated SCD risk ≥ 6%, the risk decreased over time to below 6%, and in 14% of the cases, below 4%. Conclusions: SCD risk in HCM patients is usually stable or gets lower. Our data suggest it is important to re-evaluate the risk profile for patients with HCM when ICD re-implantation is considered.

Background: Cardiac muscle hypercontractility is a key pathophysiological abnormality in hypertrophic cardiomyopathy, and a major determinant of dynamic left ventricular outflow tract (LVOT) obstruction. Available pharmacological options for hypertrophic cardiomyopathy are inadequate or poorly tolerated and are not disease-specific. We aimed to assess the efficacy and safety of mavacamten, a first-in-class cardiac myosin inhibitor, in symptomatic obstructive hypertrophic cardiomyopathy. Methods: In this phase 3, randomised, double-blind, placebo-controlled trial (EXPLORER-HCM) in 68 clinical cardiovascular centres in 13 countries, patients with hypertrophic cardiomyopathy with an LVOT gradient of 50 mm Hg or greater and New York Heart Association (NYHA) class II–III symptoms were assigned (1:1) to receive mavacamten (starting at 5 mg) or placebo for 30 weeks. Visits for assessment of patient status occurred every 2–4 weeks. Serial evaluations included echocardiogram, electrocardiogram, and blood collection for laboratory tests and mavacamten plasma concentration. The primary endpoint was a 1·5 mL/kg per min or greater increase in peak oxygen consumption (pVO2) and at least one NYHA class reduction or a 3·0 mL/kg per min or greater pVO2 increase without NYHA class worsening. Secondary endpoints assessed changes in post-exercise LVOT gradient, pVO2, NYHA class, Kansas City Cardiomyopathy Questionnaire-Clinical Summary Score (KCCQ-CSS), and Hypertrophic Cardiomyopathy Symptom Questionnaire Shortness-of-Breath subscore (HCMSQ-SoB). This study is registered with ClinicalTrials.gov, NCT03470545. Findings: Between May 30, 2018, and July 12, 2019, 429 adults were assessed for eligibility, of whom 251 (59%) were enrolled and randomly assigned to mavacamten (n=123 [49%]) or placebo (n=128 [51%]). 45 (37%) of 123 patients on mavacamten versus 22 (17%) of 128 on placebo met the primary endpoint (difference +19·4%, 95% CI 8·7 to 30·1; p=0·0005). Patients on mavacamten had greater reductions than those on placebo in post-exercise LVOT gradient (−36 mm Hg, 95% CI −43·2 to −28·1; p2 (+1·4 mL/kg per min, 0·6 to 2·1; p=0·0006), and improved symptom scores (KCCQ-CSS +9·1, 5·5 to 12·7; HCMSQ-SoB −1·8, −2·4 to −1·2; p

The assessment of defibrillation energy requirement (DER) is a standard practice during cardioverter-defibrillator (ICD) implantation. It is recommended to assure that the energy at least 10 J below the maximal energy deliverable by the implanted device successfully converts the induced ventricular fibrillation (VF). The cardiac resynchronisation therapy with defibrillator (CRT-D) recipients are at increased risk of developing serious complications due to repeated VF induction.To define the prevalence of high DER among CRT-D recipients and to determine the factors which allow to obtain defibrillation safety margin.We examined all patients who underwent CRT-D implantation between June 2006 and June 2009 in our institution. The verification of the DER required at least one termination of the induced VF with the energy at least 10 J below the maximal energy deliverable by the implanted device.The CRT-D was implanted in 65 patients. The first defibrillation test was successful in 57 (88%) patients. In the remaining 8 patients (12%), the defibrillation test was unsuccessful. These patients required system revision: reprogramming shocking polarity (2), reversing polarity and adjusting waveform (3), lead repositioning (1) and adding a subcutaneous lead (2). The use of high output devices (maximal energy30 J) and dual-coil leads was associated with a significantly (p0.05) lower rate of high DER, although high DER occurred in one patient implanted with the high output device. There was a correlation between the probability of successful defibrillation and renal function. It was less likely to obtain successful defibrillation safety margin in patients with creatinine175 micromol/L. During the follow up, ventricular tachyarrhythmia detected in the VF detection zone occurred in 13 (20%) patients, including two patients, who required system modification during implantation. In both cases, VF was terminated by the first defibrillation with the maximal energy of the implanted devices.High DER occurred in a significant number of CRT-D recipients. There is a correlation between high DER and impaired renal function. The use of high output devices significantly decreases the number of patients who required system modification in order to obtain an adequate defibrillation safety margin.

Double counting of ventricular beats is not only one of the cause of an inappropriate detection of ventricular arrhythmias but a reason of lost of resynchronisation therapy. Sensing disturbances often creates the need for reprogramming of the device or additional pharmacotherapy and procedures. We present a case of 76-year-old man with CRT-D and inappropriate detection and intervention due to ventricular bigeminy. Fortunately, change of device program, potassium and magnesium supplementation was successful, without necessity of RF ablation of the ventricular ectopic beats.

We present the improved method of recognition of sustained ventricular tachycardia (SVT) based on new filtering technique (FIR), extended signal-averaged electrocardiography (SAECG) description by 9 parameters and the application of support vector machine (SVM) classifier.The dataset consisted of 376 patients (100 patients with sustained ventricular tachycardia after myocardial infarction (MI) labelled as class SVT+, 176 patients without sustained ventricular tachycardia after MI and 77 healthy persons, 50% of data were left for validation. The analysis of SAECG was performed by 2 types of filtration: low pass four-pole IIR Butterworth filter and FIR filter with Kaiser window. We calculated 3 commonly used SAECG parameters: hfQRS (ms), RMS40 (microV), LAS40 microV(ms) and 6 new parameters: LAS25 microV(ms) - duration of the low amplitude25 microV signals at the end of QRS complex; RMS QRS(microV) - root mean square voltage of the filtered QRS complex; pRMS(microV) - root mean square voltage of the first 40 ms of filtered QRS complex; pLAS(ms) - duration of the low amplitude40 microV signals in front of QRS complex; RMS t1(microV) - root mean square voltage of the last 10 ms the filtered QRS complex; RMS t2(microV) - root mean square voltage of the last 20 ms the filtered QRS complex. For the recognition of SVT+ class patients we used the SVM with the Gaussian kernel.The results confirmed good generalization of obtained models. The recognition score (calculated as correct classification/total number of patients) of SVT+patients on data set containing 3 standard parameters (Butterworth filter) is 92.55%. The same score was obtained for data set containing 9 parameters (Butterworth filter). The best score (95.21%) was obtained for data set based on 9 parameters and FIR filter.Our approach improved risk stratification up to 95% based on SAECG due to the application of FIR filter, 6 new parameters and efficient statistical classifier, the support vector machine.

Coexistence of the goitre, proptosis and palpitations was observed in XIX century for the first time. Sinus tachyarytmias and atrial fibrillation are typical cardiac symptoms of hyperthyroidism. Atrial fibrillation occurs more often in patients with toxic goiter than in young patients with Grave's disease. These findings suggest that causes of atrial fibrillation might be multifactorial in the elderly. The aims of our study were to evaluate correlations between the parameters of atrial signal averaged ECG (SAECG) and the serum concentration of thyroid free hormones. 25 patient with untreated Grave's disease (G-B) (age 29,6 +/- 9,0 y.o.) and 26 control patients (age 29,3 +/- 6,9 y.o.) were enrolled to our study. None of them had history of atrial fibrillation what was confirmed by 24-hour ECG Holter monitoring. The serum fT3, fT4, TSH were determined in the venous blood by the immunoenzymatic method. Atrial SAECG recording with filtration by zero phase Butterworth filter (45-150 Hz) was done in all subjects. The duration of atrial vector magnitude (hfP) and root meat square of terminal 20ms of atrial vector magnitude (RMS20) were analysed. There were no significant differences in values of SAECG parameters (hfP, RMS20) between investigated groups. The positive correlation between hfP and serum fT3 concentration in group G-B was observed (Spearman's correlation coefficient R = 0.462, p0.02). No significant correlations were found between RMS20 and serum fT3 in G-B group and between hfP or RMS 20 and serum fT3 in group K. These findings suggest that occurrence of atrial fibrillation in patients with Grave's disease depends not only on hyperthyroidism but on serum concentration of fT3 also.

Renal function assessment is an important element of management and therapeutic decision-making in patients with chronic heart failure (CHF).To evaluate the prognostic value of renal dysfunction in patients with CHF in 12-month follow-up.639 consecutive patients hospitalised in our department from 1 July 2002 to 31 December 2003 with diagnosis of CHF (NYHA II-IV), based on medical records, were initially enrolled in the study. Patients underwent one-year follow-up. Finally, 498 patients, aged 22-98 years (mean age 69+/-12 years) in whom creatinine concentration was measured and creatinine clearance was estimated at admission with the Cockroft-Gault quotation and with long-term follow-up results obtained, were enrolled in the study. Patients were divided into two groups according to the creatinine level: Group I without renal dysfunction (creatinine level1.4 mg/dl), and Group II--with renal dysfunction (creatinine level1.4 mg/dl).Patients with renal dysfunction were significantly older and more likely to be male and in NYHA class III-IV (p0.001). Analysis of pharmacotherapy for CHF revealed that patients with renal impairment significantly less frequently received beta-blockers (67% vs 81%, p0.005), angiotensin-converting enzyme inhibitors (68% vs 82%, p0.005) and combined treatment of beta-blocker and angiotensin-converting enzyme inhibitor (56% vs 71%, p0.05), whereas loop diuretics were more frequently prescribed in this group (80% vs 70%, p0.05). In patients with renal dysfunction, there was a significantly higher mortality rate at 30 days (32% vs 14%, p0.001) as well as at 12 months (45% vs 20%, p0.001). The incidence of re-hospitalisation for cardiovascular reasons (CHF worsening, myocardial infarction, stroke) was significantly higher in patients with renal dysfunction (70% vs 55%, p0.005). Multivariate analysis of all factors affecting one-year mortality demonstrated that renal dysfunction is a strong and independent risk factor for death in patients with CHF (RR=2.13, 95% CI: 1.31-3.45; p0.05) and it increases the risk of re-hospitalisation (RR=1.53, 95% CI: 1.01-2.14; p0.05).Renal dysfunction is an independent prognostic factor in patients with CHF, which allows identification of a high-risk group and administration of optimal therapy, which in turn can result in a reduction of mortality.