[Cardiac channelopathies in the context of hereditary arrhythmia syndromes]. / Kardiale Kanalopathien im Kontext hereditärer Arrhythmiesyndrome.

Genetic arrhythmia disorders are rare diseases; however, they are a common cause of sudden cardiac death in children, adolescents, and young adults. In principle, a distinction can be made between channelopathies and cardiomyopathies in the context of genetic diseases. This paper focuses on the channelopathies long and short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT). Early diagnosis of these diseases is essential, as drug therapy, behavioral measures, and if necessary, implantation of a cardioverter defibrillator can significantly improve the prognosis and quality of life of patients. This paper highlights the pathophysiological and genetic basis of these channelopathies, describes their clinical manifestations, and comments on the principles of diagnosis, risk stratification and therapy.

Activated rate-response is associated with increased mortality risk in cardiac device carriers with acute heart failure.

AIMS: This study investigated whether an activated R-mode in patients carrying a cardiac implantable electronic device (CIED) is associated with worse prognosis during and after an episode of acutely decompensated heart failure (AHF). METHODS: Six hundred and twenty-three patients participating in an ongoing prospective cohort study that phenotypes and follows patients admitted for AHF were studied. We compared CIED carriers with activated R-mode stimulation (CIED-R) to CIED carriers not in R-mode (CIED-0) and patients without CIEDs (no-CIED). The independent impact of R-mode activation on 12-month all-cause death was examined using uni- and multivariable Cox proportional hazards regression taking into account potential confounders, and hazard ratios (HR) with their 95% confidence intervals (CI) were reported. RESULTS: Mean heart rate on admission was lower in CIED-R (n = 37, 16% women) vs. CIED-0 (n = 64, 23% women) or no-CIED (n = 511, 43% women): 70 bpm vs. 80 bpm or 82 bpm; both p<0.001. In-hospital mortality was similar across groups, but age- and sex-adjusted all-cause 12-month mortality risk was differentially affected by R-mode activation; CIED-R vs. CIED-0: HR 2.44, 95%CI 1.25-4.74; CIED-R vs. no-CIED: HR 2.61, 95%CI 1.59-4.29. These effects persisted after multivariable adjustment for potential confounders. Within CIED-R, mortality risk was similar in patients with pacemakers vs. ICDs and in subgroups with left ventricular ejection fraction (LVEF) <50% vs. ≥50%. CONCLUSION: In patients admitted with AHF, R-mode stimulation was associated with a significantly increased 12-month mortality risk. Our findings shed new light on "admission heart rate" as a potentially treatable target in AHF. Our data are compatible with the concept that chronotropic incompetence contributes to an adverse outcome in these patients and may not be adequately treated through accelerometer-based R-mode stimulation.

Focal atrial tachycardia mimicking catecholaminergic polymorphic ventricular tachycardia: a case report.

Background: In childhood and adolescence, cardiac arrhythmias are often benign in the absence of congenital heart defects. Nevertheless, life-threatening inherited arrhythmogenic syndromes can become clinically manifest in early childhood. As early symptoms may be similar in both conditions, thorough workup is fundamental to avoid delayed diagnosis and misdiagnosis. Case summary: We present the case of a 26-year-old Caucasian female patient who presented with recurrent non-sustained polymorphic wide complex tachycardia. Structural heart disease was excluded by echocardiography as well as cardiac magnetic resonance imaging. Due to wide complex extrasystoles and couplets with alternating QRS axis occurring at low levels of physical exertion, catecholaminergic polymorphic ventricular tachycardia (CPVT) was suspected and further investigated. Epinephrine testing in combination with an electrophysiological (EP) study with placement of a coronary sinus catheter and subsequent programmed stimulation ruled out CPVT and unmasked wide complex tachycardia as varying aberrant conduction of focal atrial tachycardia (FAT). 3D-navigated mapping of FAT revealed a direct parahisian origin. Due to significantly increased risk of atrio-ventricular (AV) block during ablation, the patient refused ablation and preferred medical antiarrhythmic therapy. Discussion: Given the consequences of both, delayed diagnosis and misdiagnosis of CPVT, thorough workup is fundamental. In case of doubt regarding potential aberrant AV conduction in the context of wide complex tachycardia, an invasive EP study may easily and safely prove or rule out aberrancy.

Ventricular arrhythmia burden in patients with implantable cardioverter defibrillator and remote patient monitoring during different time intervals of the COVID-19 pandemic.

PURPOSE: The current study investigated whether the changes in patient care in times of the COVID-19 pandemic, especially the reduction of in-person visits, would result in a deterioration of the arrhythmic and clinical condition of patients with an implantable cardioverter defibrillator (ICD) and remote patient monitoring. METHODS: Data were obtained from a local ICD registry. 140 patients who received ICD implantation at our department and had remote patient monitoring were included. The number of patients with ventricular arrhythmias, appropriate ICD therapy, the number of visits to our outpatient clinic and hospitalization due to acute coronary syndrome, stroke or heart failure were compared during three time intervals of the COVID-19 pandemic (first (LD1) and second (LD2) national lockdown in Germany and the time after the first lockdown (postLD1)) and a time interval 1 year before the pandemic began (preCOV). Each time interval was 49 days long. RESULTS: Patients had significantly fewer visits to our outpatient clinic during LD1 (n = 13), postLD1 (n = 22) and LD2 (n = 23) compared to the time interval before the pandemic (n = 43, each p ≤ 0.05). The number of patients with sustained ventricular arrhythmias, appropriate ICD therapy and clinical events showed no significant difference during the time intervals of the COVID-19 pandemic and the time interval 1 year prior. CONCLUSIONS: The lockdown measures necessary to reduce the risk of infection during the COVID-19 pandemic, led to a reduction of in-person patient visits, but did not result in a deterioration of the arrhythmic and clinical condition of ICD patients with remote patient monitoring.

Impact of coronavirus disease (COVID-19) pandemic on physical activity of patients with cardiac implantable electronic devices-A remote monitoring study.

OBJECTIVES: The study aims to investigate the impact of COVID-19 pandemic on physical activity and frequency of implantable cardioverter-defibrillator (ICD) therapies of patients with cardiac implantable electronic devices. METHODS AND RESULTS: Physical activity, heart rate and ICD-therapies were assessed via routine remote monitoring over two years. We focussed on a 338-day period during COVID-19 pandemic that was divided in 6 time-intervals defined by public health interventions and compared to the previous regular year. Paired nonparametric longitudinal analysis was performed to detect differences between time-intervals. To model effects of age, sex and time we applied a nonparametric ANOVA-type-statistic. 147 patients with cardiac implantable electronic devices were analysed. Longitudinal analysis of physical activity in 2019 and 2020 showed a specific weekly and seasonal pattern. Physical activity was reduced during the pandemic (mean daily physical activity 2019: 12.4% vs. 2020: 11.5%; p<0.0001) with the strongest reductions (fold changes 0.885/0.889, p<0.0001/p<0.0001) during the two lockdown-periods. In older patients (>70 years), physical activity was decreased in every time-interval of the year 2020. In time-intervals of eased restrictions, physical activity of younger patients (≤70 years) was not different compared to 2019. No variation in mean heart rate, arrhythmia-burden and count of ICD-therapies was found. CONCLUSION: Physical activity shows fluctuations dependent on days of the week and time of the year. During the pandemic, physical activity was reduced in patients with cardiac implantable electronic devices with the strongest reductions during lockdown-periods. Younger patients resumed former levels of physical activity in times of eased restrictions while older patients remained less active. Thus, activation of the elderly population is important to prevent long-term health impairments due to the pandemic.

[Catheter ablation for atrial fibrillation - standard of care and future perspectives]. / Katheterablation von Vorhofflimmern ­ aktuelle Versorgungsstandards und Zukunftstrends.

Atrial fibrillation is the most frequent cardiac arrhythmia in adults. For symptomatic atrial fibrillation, catheter ablation has proven to be an effective and safe treatment that is superior to antiarrhythmic drug treatment regarding quality of life and maintenance of normal sinus rhythm. Moreover, there is increasing evidence that early rhythm control and catheter ablation can be of prognostic benefit. Especially heart failure patients with impaired left ventricular systolic function seem to benefit from rhythm control therapy by catheter ablation. Furthermore, technological innovations such as novel single-shot devices, contact force mapping, the concept of high-power-short-duration (HPSD) ablation and implementation of electroporation (pulsed field ablation) offer the prospect of further improving ablation efficiency and safety. This review provides an overview of current standards of care as well as future trends in atrial fibrillation catheter ablation techniques.

Atrioventricular 2:1-conduction via an accessory pathway during left atrial flutter unmasking WPW syndrome: a case report.

Background: Implantable cardioverter defibrillators (ICDs) are most effective in treating sudden cardiac death. However, accurate diagnostic workup of broad complex tachycardia is crucial to ensure correct indication for ICD treatment and to avoid unnecessary invasive treatment and device-associated morbidity. Case summary: We present a case of atypical atrial flutter with 2:1 atrioventricular (AV) conduction via a left-posterior accessory pathway (AP), leading to the diagnosis of Wolff-Parkinson-White (WPW) syndrome. Upon admission, the 72-year-old patient showed a regular broad complex tachycardia with superior axis and positive concordance in precordial leads, suggestive of either ventricular tachycardia (VT), antidromic AV re-entrant tachycardia (AVRT), or supraventricular tachycardia with antegrade conduction via a left-posterior AP. Interrogation of the two-chamber ICD, which was very likely implanted unjustified in a peripheral clinic before, revealed atrial flutter with 2:1 AV conduction. Remarkably, after the restoration of sinus rhythm, no classic echocardiogram (ECG) criteria for preexcitation syndrome were detected. An invasive electrophysiological study proved the diagnosis of a bidirectionally conducting, left-posterior AP, which was successfully ablated. Discussion: Differential diagnosis of broad complex tachycardia with superior axis and positive concordance of chest leads consists of i) VT with a left ventricular exit at the posterior mitral annulus, ii) antidromic AVRT involving a left-posterior AP, and iii) supraventricular tachycardia predominantly conducted via a left-posterior AP. The absence of classic ECG criteria for preexcitation syndrome does not rule out AP sufficiently, highlighting the importance of minimal surface-ECG preexcitation criteria. In the case of detection of minimal surface-ECG preexcitation criteria, administration of adenosine rules out or proves the existence of an AP noninvasively and cost-effectively.

Profound repolarization abnormalities after cylindrical battery ingestion: a case report.

BACKGROUND: Cardiac arrhythmias are a serious complication in patients admitted due to intoxication in suicidal attempts. Upon admission, detailed information about the specific kind of intoxication are frequently missing. The differential diagnoses of electrocardiogram (ECG) changes such as elevation of T-waves, prolongation of the QT-interval or elevation of ST-segments in this special subgroup of patients comprise drug-induced electrolyte disorders or direct toxic effects on cardiac excitation and repolarization. CASE SUMMARY: In this clinical report of a 27-year-old male patient, we present a case of unusual ECG alterations mimicking ST-elevation, high amplitude, biphasic T-waves and prolongation of QT-interval. These changes of surface ECG were induced by ingestion of cylindrical batteries in a suicidal attempt and immediately normalized after removal of batteries by esophagogastroduodenoscopy. DISCUSSION: There is limited literature describing changes in surface ECG in patients having ingested cylindrical batteries. We propose two hypotheses for the occurrence of these changes after ingestion of cylindrical batteries: (i) Cardiac movement within the perturbation field induced by the batteries causes electrical changes on a time scale of the heart rate which are above the threshold of the high pass filter. (ii) The batteries' electrotonic potential affects the membrane currents of cardiac myocytes, not inducing an action potential but generating repolarization abnormalities. Individual factors, such as body constitution and localization of the batteries within the stomach, determine the interindividual characteristics of repolarization abnormalities.