A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly.

Background: Ebstein anomaly (EA) is a rare congenital heart disease characterized by the apical displacement of the tricuspid leaflets, creating an enlarged functional right atrium. Supraventricular arrhythmias (SVA) are common, and catheter ablation remains challenging. SVA is considered a risk factor for sudden cardiac death in this population. Still, there are very few real-life data on the impact of SVA treated invasively or conservatively on a patient's prognosis. We aimed to analyze the incidence of SVA in adults with EA, evaluate the effectiveness of catheter ablation, and analyze the impact of SVA and catheter ablation on survival in this population. Methods and results: 71 pts (median age 53 years; range 24-84 years) with EA were evaluated retrospectively from 1988 to 2020. Forty patients (56.3%) had SVA, and eighteen of them (45.0%) required at least one catheter ablation (35 procedures in total). Indications for ablation were mostly intra-atrial reentrant tachycardia (IART) and atrioventricular reentrant tachycardia (AVRT) (14 pts [77.8% and 9 pts [50.0%], respectively. IART and AVRT coexisted in nine pts. One patient suffered from persistent atrial fibrillation. Procedural effectiveness was reported in 28 (80%) cases; over a longer follow-up (mean 12.6 ± 5.4 years), only eight (44.4%) patients were completely free from SVA after the first ablation. In total, 10 patients (14%) died due to cardiovascular events. There was no difference in survival between patients with or without SVA (p = 0.9) and between ablated and non-ablated EA individuals (p = 0.89). Conclusions: Supraventricular arrhythmia is frequent in adults with Ebstein anomaly. Patients often require more than one catheter ablation but eventually become free from arrhythmias. The imaging parameters assessed by echocardiography or cardiac magnetic resonance do not seem to be associated with ablation outcomes. The impact of supraventricular arrhythmia itself or treatment with radiofrequency ablation is questionable and should be thoroughly investigated in this population.

Complications of cardiac resynchronization therapy implantation: De novo implants versus upgrades.

BACKGROUND: Cardiac resynchronization therapy (CRT) is a well-established treatment of patients with advanced heart failure and electrical dyssynchrony. Implantation of those devices is in some cases associated with intervention on a formerly implanted system. The aim of this analysis was to compare the rate and type of complications of de novo implants and upgrades to CRT-D. METHODS: Retrospective data were collected from medical records, including 326 patients treated with CRT-D between 2015 and 2020. The following data were analyzed: procedure data including complications, demographics, co-morbidities, pharmacotherapy, and laboratory tests. The primary endpoint of the study was all-cause mortality. RESULTS: A total of 326 procedure were included, of which 53% (n = 172) were de novo implants and 47% (n = 154) were upgrades. The groups did not differ in the incidence of complications: in the de novo group: 25.5% (n = 44); in the upgrade group: 30.5% (n = 47), p = 0.78. The incidence of complications was also similar in respect of the following: early (p = 0.98) and late (p = 0.45), infectious (p = 0.38) and non-infectious (p = 0.82), surgical (p = 0.38) and device or lead related (p = 0.6). The most common complication in the upgrade group was pocket hematoma (n = 9, 5.8%) and in the de novo group pneumothorax (n = 8, 4.7%). CONCLUSIONS: Upgrade procedures of are not associated with a higher percentage of complications than de novo implantations of CRT-D. Previously implanted cardiac implantable electronic device should not limit the implantation of CRT-Ds.

Follow-up and characteristics of recipients of cardiac resynchronization therapy with and without a defibrillator.

BACKGROUND: Cardiac resynchronization therapy defibrillator (CRT-D) and pacemaker (CRT-P) are treatment options for patients with advanced heart failure and electrical dyssynchrony. Current guidelines provide only factors favoring, not specific recommendations as to implant CRT-D or CRT-P. This analysis aimed to compare and contrast populations of CRT-D and CRT-P recipients. METHODS: Retrospective data were collected from medical records, including 231 patients treated with either CRT-D or CRT-P between 2015 and 2019. Following data were analyzed demographics, co-morbidities, pharmacotherapy, laboratory tests, and data related to the procedure of implantation. The primary endpoint of the study was all-cause mortality. RESULTS: A total of 231 patients were included (mean age [standard deviation, SD], 64.1 [12.3] years, 76% male), of these, 13.6% (n = 32) with CRT-P and 86.4% (n = 199) with CRT-D. Mean New York Heart Association (NYHA) class did not differ between the groups: 2.23 (0.9) in CRT-P and 2.35 (0.6) in CRT-D group (P = 0.42). Mean left ventricular ejection fraction was lower in patients eligible for CRT-D: 27.1% vs. 38% (P < 0.001). Patients were followed for a median (interquartile range [IQR]) of 29 (13-44) months and survival in the CRT-P group was 84%, in CRT-D - 82% (P = 0.74). Patients in the CRT-P group were older, and more often after atrioventricular node ablation. The CRT-P group had tendency towards higher Charlton Comorbidity Index, reaching a mean of 4.66 (1.5) points vs. 3.96 (1.5) points in CRT-D (P = 0.06). CONCLUSIONS: Populations with CRT-P and CRT-D differ in terms of comorbidities; however, they have similar survival. Further studies are required to identify a group of patients, who derive a benefit from adding a defibrillator.

Predictors of Long-Term Infections After Cardiac Implantable Electronic Device Surgery　- Utility of Novel PADIT and PACE DRAP Scores.

BACKGROUND: Cardiac implantable electronic device-related infections (CDI) are of increasing importance and involve substantial healthcare resources. This study aimed to evaluate potential CDI risk factors and the utility of the novel PADIT and PACE DRAP scores to predict CDI.Methods and Results:The study group included 1,000 consecutive patients undergoing implantable cardioverter-defibrillator (ICD) or cardiac resynchronization therapy (CRT) surgery. Patients' and procedural characteristics were collected. CDI occurrence was assessed during 1-year follow-up. Moreover, if periprocedural significant pocket hematoma (SPH) occurred, the maximal volume was calculated based on ultrasonographic measurements and ABC/2 formula. The overall incidence of CDI was 1.8%. In the multivariable regression analysis independent CDI risk factors were: age >75 years (odds ratio [OR]: 5.93; 95% confidence interval [CI]: 1.77-19.84), system upgrade procedure (OR: 6.46; CI: 1.94-21.44), procedure duration >1 h (OR: 13.96; CI: 4.40-44.25), presence of SPH (OR: 4.95; CI: 1.62-15.13) and reintervention within 1 month (OR: 16.29; CI: 3.14-84.50). The PACE DRAP score had higher discrimination of CDI incidence (area under curve [AUC] 0.72) as compared with the PADIT score (AUC 0.63). CONCLUSIONS: We identified 5 independent risk factors of CDI development. Our study also showed that the PACE DRAP score was better able to identify patients at high risk of CDI than the PADIT score.

PACE DRAP: a simple score for predicting significant bleeding complications after cardiac implantable electronic device surgery.

INTRODUCTION: Currently, no risk score for predicting significant bleeding complications (SBCs) after cardiac implantable electronic device (CIED) surgery is available. OBJECTIVES: We aimed to develop a new scoring system for predicting SBCs aft er CIED surgery. PATIENTS AND METHODS: The incidence of SBCs was 4.5%. Based on multivariable analyses, the following predictors of SBCs were identified: age ≥75 years (odds ratio [OR], 8.10; 95% CI, 3.54-18.54); cardiac resynchronization therapy or implantable cardioverter-defibrillator surgery (OR, 5.96; 95% CI, 2.48-14.32); upgrade procedure (OR, 10.22; 95% CI, 4.05-25.78); uncontrolled arterial hypertension (OR, 4.82; 95% CI, 1.78-13.06); presence of valvular prosthesis (OR, 7.85; 95% CI, 3.15-19.58); current malignancy (OR, 6.11; 95% CI, 1.81-20.66); renal failure (OR, 4.28; 95% CI, 1.86-9.87); and the use of antiplatelet drugs (clopidogrel [OR, 6.69; 95% CI, 2.48-18.04] or ticagrelor [OR, 22.25; 95% CI, 4.56-108.46]). The score was created using the weighted points proportional to the ß regression coefficient of each predictor rounded to the nearest integer, and the acronym PACE DRAP corresponds to the predictor's first letter. The cutoff point for the high risk of SBCs was 6 points with a sensitivity of 88.24% and a specificity of 87.23%. The PACE DRAP showed good predictive ability (area under the curve, 0.95 ; P <0.001). CONCLUSIONS: The PACE DRAP score is useful in identifying patients at high risk for SBCs after CIED surgery.

Double atrial potentials in left-sided accessory pathways are associated with paroxysmal atrial fibrillation.

INTRODUCTION: Muscular connections between the coronary sinus (CS) and left atrium probably impact distribution of electrical activity. Double atrial potentials (DP) may be their presentation. The aim was to investigate the presence of DP in CS recordings during atrioventricular reentrant tachycardia (AVRT) and its contribution to the occurrence of paroxysmal atrial fibrillation (AF). METHODS: A group of 247 patients with accessory pathways (AP) were screened for DP. The patients with DP during AVRT were compared to those without DP. RESULTS: DP during AVRT were found only among the left-sided AP (AP-L). Patients with AP-L were divided into Group 1 (n  =  17) with DP during AVRT and Group 2 (n  =  108) without DP. Patients in Group 1 had higher incidence of AF in history (47.1% vs. 23.1%; P  =  0.0376), AF induced during electrophysiological (EP) study (70.6% vs. 25%; P  =  0.0002). Group 1 had higher heart rate (HR) during AVRT in the EP study (197.2 ± 27 vs. 175.1 ± 26.3 bpm; P  =  0.0019), but HR of clinical AVRT (208.5 ± 30.8 vs. 191.6 ± 27.8 bpm) was not significant different (P  =  ns). Additionally, electrical alternans of QRS amplitude during AVRT in the EP study was more frequent in Group 1 (52.9 vs. 20.4 %; P  =  0.0048). CONCLUSION: Patients with DP and AP-L were more prone to develop AF. The presence of DP was associated with faster AVRT rate. The direction of atrium depolarization during AVRT may be different in the presence of DP and probably plays a role in development of AF in this group of patients.

Asymmetry of the variability of heart rate and conduction time between atria and ventricles.

BACKGROUND: Heart rate asymmetry is caused by an unequal contribution of heart rate decelerations and accelerations to heart rate variability. This study evaluates the asymmetric properties of the variability of the AA, HH, VV, AH and HV intervals. METHODS AND RESULTS: We recorded 50 1-min intracardiac ECG tracings from 10 patients (18-66 years old; 8 females) during a routine electrophysiological study. Standard descriptors of variance asymmetry were calculated for all intervals. Nonparametric tests were used for statistical comparisons. The prolongations of VV (P=0.0297), AH (P=0.0133) and HV (P=0.0004) intervals contributed significantly more than their shortenings to their short-term variance. The proportion of recordings with a larger contribution of prolongations than shortenings was significantly different from random for VV (0.66, P=0.0328), AH (0.68, P=0.0154) HV (0.74, P=0.0009). CONCLUSIONS: In addition to heart rate asymmetry (VV interval), the conduction from the atria, through the atrioventricular node, His-Purkinje system to ventricles shows asymmetric properties in 1-min tracings.

[Unusual epicardial location of ventricular ectopy in left ventricular outflow tract, cured with RF ablation from the great cardiac vein]. / Nietypowa, epikardialna lokalizacja dodatkowych skurczów komorowych z drogi odplywu lewej komory, leczona ablacja RF z obszaru zyly wielkiej serca.

The case report refers to a 54-year-old woman with a drug-refractory premature ventricular contractions (total number of ventricular ectopy: 40,851 beats/24 h) where an ectopy focus was localised in epicardial part of the left ventricular outflow tract. Successful radiofrequency ablation with the open-irrigated-tip catheter was performed at the site of earliest activation in the great cardiac vein.

[Unusual picture of pacemaker leads in patient after Senning procedure]. / Nietypowy uklad elektrod stymulatora u pacjenta po operacji Senninga.

We present a case of a 21-year-old man after Senning operation admitted to our hospital for transvenous implantation of a dual chamber pacemaker. The presence of persistent left superior vena cava enabled us to implant the desired dual chamber pacemaker. It is an extremely unusual situation when two pacemaker leads utilise two different routes to the heart: superior caval vein - atrial baffle - ventricle and persistent left superior caval vein - atrium.

[Mild type of the Ebstein anomaly]. / Lagodna postac anomalii Ebsteina.

Ebstein anomaly is a congenital malformation of the heart that is characterised by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialisation of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. We present a case of a mild type Ebstein anomaly leading to moderate tricuspid valve regurgitation and some degree of right ventricular dysfunction.

[Fascicular tachycardia - a rare case of ventricular tachycardia]. / Czestoskurcz wiazkowy - rzadka forma czestoskurczu komorowego.

It is described a case of an 18 years old woman admitted to the hospital with tachycardia 160/min. Drugs routinely used for supraventricular arrhythmias and cardioversion were ineffective. Patient were transferred to the reference center. Based on careful ECG examination diagnosis was established as fascicular tachycardia. Verapamil given intravenously stopped tachycardia immediately. Location of reentry circuit within left ventricle and differential diagnosis of fascicular tachycardia as well as ECG features are described in discussion.

[Contribution of atrioventricular node inputs on orthodromic atrioventricular reentrant tachycardia cycle length]. / Wplyw wlasciwosci wezla przedsionkowo-komorowego na cykl ortodromowego czestoskurczu przedsionkowo-komorowego.

We describe a 21-year-old woman with a previous history of orthodromic AVRT and overt preexcitation. Electrophysiological study revealed the presence of a left-sided accessory pathway. During an orthodromic AVRT a spontaneous sudden change in heart rate (141 to 202 beats/min) without any change of the QRS morphology was noted due to a decrease in AH interval (from AH=227 ms to AH=100 ms). We explained this phenomenon as the sudden change of the inferior inputs to superior in the AV node.

[Myocarditis--the first symptom of adult Still's disease]. / Zapalenie miesnia sercowego jako pierwszy objaw choroby Stilla u doroslych.

Myocardial involvement during the course of adult Still's disease is very rare. We present a case of a 34-year-old man admitted with fever, tachycardia, weakness and chest discomfort. The electrocardiogram showed non-specific diffuse ST-T changes, troponin level was slightly elevated and echocardiography did not reveal any abnormalities. After ruling out other relevant diseases, the adult Still's disease was diagnosed.

["Benign" atrial arrhythmias exacerbated by physical activity as a cause of cardiomyopathy]. / "Lagodne" arytmie przedsionkowe nasilane wysilkiem przyczyna kardiomiopatii.

We describe a 39-year-old man with premature atrial contractions at rest, in whom tachycardia was exacerbated by exercise into long-lasting atrial tachycardia of 150 beats/min with changeable grade of AV conduction. The feeling of irregular heart beating was the only symptom of arrhythmia and was well tolerated for many years. However, the signs of tachycardia-mediated cardiomyopathy with heart failure developed over several years. Successful RF ablation of focal atrial tachycardia using the CARTO system was performed. After 5 months of follow-up the patient is free from arrhythmia with a significant improvement of the HF.

[Atrioventricular nodal tachycardia with left-sided slow pathway treated with RF ablation]. / Napadowy czestoskurcz wezlowy z lewostronnym szlakiem wolnym.

We present a case of 10-year-old boy with AVNRT. The tachycardia had prolonged RP interval (160 ms) and HA time (135 ms). The application at the rim of the coronary sinus resulted in accelerated junctional rhythm and retrograde block followed by transiently prolonged atrioventricular conduction. The mapping of the mitral annulus transeptally presented no 'slow potentials'. The electrode was then inserted retrogradely through the aorta and slow potentials were recorded on the septal aspect of the left atrium. Two RF applications at this site were successful. The patient was followed for three years without recurrence of arrhythmia.

[Twiddler syndrome: a rare complication of ICD implantation]. / Twiddler syndrome - rzadkie powiklanie wszczepienia kardiowertera-defibrylatora.

Twiddler syndrome is a rare complication after pacemaker or ICD implantation. We present a case of a man who experienced 7 inappropriate ICD shocks because of this complication. Treatment included total system removal and new device implantation on the right side.

[Presyncope caused by non-sustained ventricular tachycardia. Significance of RF ablation]. / Omdlenia z powodu krótkotrwalych napadów czestoskurczu komorowego. Rola ablacji RF.

We describe a case of a 55-year-old man with episodes of presyncope caused by non-sustained ventricular tachycardia (ns-VT). Symptoms of significant weakness started when he was 30-year-old. In the last 2 years there was a substantial increase in frequency of presyncope from 2 per month to 8 per week. He does not have palpitations. Standard ECG, echocardiography and coronary angiography were normal. During an exercise test ns-VT 220/min (5 s, 20 x QRS) with LBBB morphology was documented. Successful RF ablation of ns-VT using the CARTO system was performed. During 4-month follow-up the patient remains free from ventricular arrhythmia.