Long QT Syndrome and WPW Syndrome: A Very Rare Association between Two Causes of Sudden Cardiac Death in a Young Patient.

Long QT syndrome (LQT) and WPW syndrome are causes of sudden cardiac death (SCD) in the young, and their association has been rarely reported. A 26-year-old woman presented with recurrent syncope. Her ECG showed a short PR interval, wide QRS (150 ms) due to a delta wave, and QT prolongation (QT 580 ms, QTc 648 ms). ECG monitoring documented recurrent salvos of a self-terminating wide QRS tachycardia, generally slightly polymorphic, sometimes with "torsade des pointes" (TdP) appearance, which were linked to the syncopal/presyncope episodes. Electrophysiologic monitoring diagnosed a right para-hisian accessory pathway with a very short ERP (240 ms baseline, <200 ms after isoproterenol). The pathway was ablated successfully. Despite QRS narrowing (80 ms), QT prolongation persisted after ablation (QT 620 ms, QTc 654 ms), with short runs of TdP, despite beta-blocker treatment, which was increased to the maximal dosage. A dual-chamber implantable cardioverter defibrillator (ICD) was implanted. To our knowledge, this is the first case report of an association between LQT and WPW syndrome in which both conditions are associated with an increased risk of SCD.

Predictors for Super-Responders in Cardiac Resynchronization Therapy.

BACKGROUND: Prediction of cardiac resynchronization therapy (CRT) response, particularly a super-response, is of great importance. STUDY QUESTION: The aim of our study was to assess the predictors for super-responders in CRT. STUDY DESIGN: We conducted a retrospective, observational study, which finally included 622 patients with heart failure treated with CRT between January 2008 and May 2020 who had a minimal follow-up of 6 months after CRT. MEASURES AND OUTCOMES: A total of 192 super-responders, defined by a left ventricular ejection fraction (LVEF) of at least 45%, and/or minimum 15% increase in LVEF and an improvement of the New York Heart Association functional class by at least 2 degrees at the last follow-up, and the rest of 430 patients who did not fulfill the super-responder criteria. RESULTS: The highest rate of super-responders (41.91%, n = 171) was at patients with left ventricle-only pacing with optimal fusion (OPT) compared with patients with biventricular (BiV) pacing (9.81%, n = 21, P < 0.000). In the OPT group, univariable analysis showed that nonischemic cardiomyopathy, a smaller degree of mitral regurgitation, and better left ventricle function at enrollment were predictors for super-response compared with the BiV group where a narrower QRS after implantation, nonischemic cardiomyopathy, and a better baseline LVEF were predictors for super-responders. In the multivariable analysis, both narrower QRS after implantation and nonischemic cardiomyopathy were independent predictors for super-response in the BiV group compared with OPT where nonischemic cardiomyopathy remained the only independent predictor for super-response. CONCLUSIONS: In this retrospective study, OPT CRT programing was an additional predictor of super-response to CRT besides nonischemic cardiomyopathy.

Transvenous Lead Extraction of Cardiac Implantable Electronic Devices: Reimplant Strategy and Outcomes in a Single-Center Experience.

Cardiac implantable electronic devices are now widely used worldwide and the numbers are increasing exponentially. Subsequently, long-term complications have increased. Transvenous lead-extraction (TLE) is the gold standard for removing infected devices, treating systemic device-related infections including endocarditis, and removing devices for other non-infectious complications. Most patients still require device therapy after TLE for several indications, including lifesaving defibrillation or pacing in pacemaker-dependent patients. The decision to reimplant is challenging, particularly when the primary cause for device removal includes device-related infections and patients frequently are pacemaker dependent. We aim to present our strategy for reimplanting after performing TLE in 88 consecutive patients. We performed transvenous removal of 150 pacemaker and defibrillator leads, of which 74% for local or systemic infection. We report a 99.3% clinical success after TLE. Out of 88 patients who had undergone TLE and after reanalysing the device indication, 67 patients (76%) still had indication for device removal, but three of them refused to undergo reimplant and 58 were re-implanted. Of the re-implanted patients, 58.6% were implanted on the contra-lateral side, 38% on the ipsilateral side and two patients who had been previously implanted with ICD were reimplanted with a subcutaneous implantable cardioverter defibrillator (S-ICD). Up to 34.4% of patients were re-implanted during the same TLE procedure, which was performed for device up-grade in patients with venous occlusion, 24% were implanted during the same hospital admission for TLE but not during TLE, and 41.3% were discharged and reimplanted afterwards. With this strategy for reimplantation, we report no re-infections and no device-removal-related arrhythmic major events or deaths during one-year follow-up. In conclusion, most patients still require device therapy after TLE. In patients with previous infection of the device, the reimplantation strategy should be carefully analyzed to prevent infection relapse and limit the consequences of the absence of the device.

Non-revascularized chronic total occlusions impact on substrate and post-ablation results in drug-refractory electrical storm.

Background and aims: There is limited data concerning the effect of non-revascularized chronic total occlusions (NR-CTOs) after VT ablation. This study sought to evaluate the impact of NR-CTOs after ablation for electrical storm (ES). Methods: Post-hoc retrospective analysis of data regarding 64 consecutive post-myocardial infarction patients (out of which 12 patients with NR-CTOs and 52 without NR-CTOs) undergoing substrate ablation for ES with an available median follow-up of 37.53 (7.25-64.65) months. Ablation result was assessed by inducibility of sustained monomorphic VT (SMVT) during final programmed ventricular stimulation (PVS). The primary endpoints were all-cause mortality and VT/VF recurrences after ablation, respectively, stratified by the presence of NR-CTOs. The secondary endpoint was to assess the predictive effect of NR-CTOs on all-cause mortality and VT/VF recurrences in relation to other relevant prognostic factors. Results: At baseline, the presence of NR-CTOs was associated with higher bipolar BZ-to-total scar ratio (72.4% ± 17.9% vs. 52% ± 37.7%, p = 0.022) and more failure to eliminate the clinical VT (25% (3) vs. 0% (0), p < 0.001). During follow-up, overall all-cause mortality and recurrences were more frequent in the NR-CTO subgroup (75% (9) vs. 19.2% (10), log rank p = 0.003 and 58.3% vs. 23.1% (12), log rank p = 0.042 respectively). After adjusting for end-procedural residual SMVT inducibility, NR-CTOs predicted death during follow-up (HR 3.380, p = 0.009) however not recurrence (HR 1.986, p = 0.154). Conclusions: NR-CTO patients treated by RFCA for drug-refractory ES demonstrated a higher ratio of BZ-to-total-scar area. In this analysis, NR-CTO was associated with worse acute procedural results and may as well impact long-term outcomes which should be further assessed in larger patient populations.

Arrhythmic Risk and Treatment after Transcatheter Atrial Septal Defect Closure.

Atrial septal defect (ASD) represents the most common congenital heart defect identified in adulthood. Atrial and ventricular geometric remodeling due to intracardiac shunt increase the risk of arrhythmias, especially atrial fibrillation (AF). Clinical, echocardiography, electrocardiogram, and device-related predictors may be used to assess the risk of atrial arrhythmias after ASD closure. The underlying mechanisms in these patients are complex and at least in part independent of the structural remodeling secondary to hemodynamic overload. Device closure of the ASD itself and its timing impact future arrhythmia risk, as well as posing a challenge for when transseptal puncture is required. Sudden cardiac death (SCD) risk is higher than in the general population and an implantable cardioverter-defibrillator (ICD) may be indicated in selected cases.

Behind Enemy Lines: Vital Echocardiographic Data Prior to Ventricular Arrhythmia Ablation.

Ventricular arrhythmias (VA) are a major cause of sudden cardiac death (SCD). Echocardiography is the first widely available imaging tool which guides VA management strategies. Along with other invasive and noninvasive imaging techniques, it provides essential information for identification of VA substrate such as differentiation between ischemic and non-ischemic etiology and identification of structural heart disease. Both classic as well as novel echocardiographic techniques such as left ventricular strain measurement and mechanical dispersion assessment provide prognostic information and assist in risk stratification. Furthermore, intracardiac echocardiography may have an adjunctive role for the VA ablation by providing real-time visualization of cardiac structures, continuous monitoring of catheter location and early recognition of procedural complications. This review gathers all relevant information that echocardiography may offer prior to VA ablation procedures.

Not Just a One-Way: Mahaim Accessory Pathway Concomitantly Supporting Orthodromic Atrioventricular Re-Entrant Tachycardia.

INTRODUCTION: We report the case of a 41-year-old female with documented narrow QRS tachycardia. During electrophysiological study, both orthodromic and antidromic atrioventricular reentry tachycardia (AVRT) were demonstrated as well as short episodes of pre-excited atrial fibrillation. Programmed atrial stimulation resulted in decremental anterograde conduction on the AP, thus confirming an unexpected Mahaim accessory pathway (AP) diagnosis. DISCUSSION: Limited 3D activation maps of the right atrium during orthoAVRT, respectively, and the right ventricle (RV) during antiAVRT were constructed and helped accurately describe the atrial and ventricular insertion points, which were superposed on the tricuspid ring, confirming the existence of a single short atrio-ventricular right free wall AP. Short atrioventricular APs with anterograde Mahaim-type conduction concomitantly sustaining orthodromic AVRT are extremely rare. CONCLUSIONS: Electroanatomical 3D mapping may help both to clarify the diagnosis and increase the success rate by accurately describing the insertion points of complex accessory pathways.

Ratio between Right Ventricular Longitudinal Strain and Pulmonary Arterial Systolic Pressure: Novel Prognostic Parameter in Patients Undergoing Cardiac Resynchronization Therapy.

BACKGROUND: We aimed to evaluate whether right ventricle (RV) longitudinal strain indexed to pulmonary arterial systolic pressure (PASP) has prognostic significance in patients undergoing cardiac resynchronization therapy (CRT). METHODS: Patients undergoing CRT were prospectively included. The primary endpoint was adverse cardiovascular events (death and HF-related hospitalizations). RV global longitudinal strain (RVGLS) and RV free wall strain (RVfwS) were measured by speckle tracking and indexed to echocardiographic estimated PASP. RESULTS: A total of 54 patients (64.0 ± 13.8 years; 58% male) were included. After 33 ± 12.9 months, the primary endpoint occurred in 18 patients. Baseline RVGLS/PASP and RVfwS/PASP showed good discriminative ability for response to CRT (AUC = 0.88, 95% CI (0.74-1) and AUC = 0.87, 95% CI (0.77-1)). RVGLS/PASP and RVfwS/PASP were significantly associated with high risk of events at univariate analysis (HR 0.039, 95% CI (0.001-0.8) p < 0.05, respectively HR = 0.049, 95% CI (0.0033-0.72), p < 0.05). Upon multivariate Cox regression analysis, RVGLS/PASP and RVfwS/PASP remained associated with high risk of events (HR 0.018, 95% CI (0.0005-0.64), p = 0.02 and HR 0.015, 95% CI (0.0004-0.524), p = 0.01) after correction for gender, etiology, QRS duration and morphology. Conclusions: Indexing RV longitudinal strain (global and free wall) by PASP provides a parameter, which independently identifies patients with high risk of cardiovascular events and predicts non-response to CRT.

Right ventricular-arterial coupling - A new perspective for right ventricle evaluation in heart failure patients undergoing cardiac resynchronization therapy.

BACKGROUND: Right ventricular - arterial (RV-PA) coupling can be estimated by echocardiography using the ratio between (TAPSE) and pulmonary arterial systolic pressure (PASP). TAPSE/PASP ratio proved to be a prognostic parameter in patients with heart failure and reduced ejection fraction (HFrEF). OBJECTIVE: To evaluate the significance of RV-PA coupling in patients with HFrEF undergoing cardiac resynchronization therapy (CRT). METHODS: Patients undergoing CRT in our center between January 2017 and November 2019 were eligible. Response to CRT was defined by a reduction of more than 15% of left ventricle systolic volume (LVESV) one year after CRT. Primary endpoint was a composite of HF hospitalizations and death during follow-up. RESULTS: 54 patients (Age 64.0 ± 13.8 years; 58% male; left ventricular ejection fraction (LVEF) 28.4 ± 1.3%) were prospectively included. After a mean follow-up of 31 ± 12.9months, the primary endpoint had occurred in 18 (33.3%) patients. A lower TAPSE/PASP ratio was associated with baseline worse HF symptoms, lower LVEF and long-term less LV reverse remodeling (P < .05). After one year CRT improved RV systolic function (TAPSE, RV global longitudinal strain, P < .05), but not TAPSE/PASP ratio (P = .4). The ratio TAPSE/PASP (AUC=0.834) ≥ 0.58 mm/mm Hg showed good sensitivity (90%) and specificity (81.8%) for predicting response to CRT while a ratio <0.58 mm/mm Hg was associated with a higher risk of death and HF hospitalizations during the follow-up (HR 5.37 95%CI [1.6-18], P < .001). CONCLUSION: RV-PA coupling evaluation using TAPSE/PASP ratio predicts CRT response. A lower TAPSE/PASP ratio is associated with a higher risk of adverse cardiovascular events.

Yield of Rare Variants Detected by Targeted Next-Generation Sequencing in a Cohort of Romanian Index Patients with Hypertrophic Cardiomyopathy.

BACKGROUND: The aim of this study was to explore the rare variants in a cohort of Romanian index cases with hypertrophic cardiomyopathy (HCM). METHODS: Forty-five unrelated probands with HCM were screened by targeted next generation sequencing (NGS) of 47 core and emerging genes connected with HCM. RESULTS: We identified 95 variants with allele frequency < 0.1% in population databases. MYBPC3 and TTN had the largest number of rare variants (17 variants each). A definite genetic etiology was found in 6 probands (13.3%), while inconclusive results due to either known or novel variants were established in 31 cases (68.9%). All disease-causing variants were detected in sarcomeric genes (MYBPC3 and MYH7 with two cases each, and one case in TNNI3 and TPM1 respectively). Multiple variants were detected in 27 subjects (60%), but no proband carried more than one causal variant. Of note, almost half of the rare variants were novel. CONCLUSIONS: Herein we reported for the first time the rare variants identified in core and putative genes associated with HCM in a cohort of Romanian unrelated adult patients. The clinical significance of most detected variants is yet to be established, additional studies based on segregation analysis being required for definite classification.

The Relationship Between Psychosocial Status and Hypertensive Condition.

PURPOSE OF REVIEW: The aim of the paper is to test the influence of social status and psychological well-being (independent variables) on hypertensive condition (dependent variable), when adjusting for traditional risk factors of cardiovascular disease (control variables). The analysis is based on data collected from SEPHAR III, a nationally representative epidemiologic study of the Romanian adult population. RECENT FINDINGS: Understanding the social roots of health issues is of considerable importance in developing effective strategies and policies. In this context, most studies explain the influence of social and psychological indicators on hypertension by considering the mediating effects of class-based lifestyle practices, i.e., the full range of economic, social, or symbolic resources available to particular social classes. However, the effect of traditional risk factors of cardiovascular disease in shaping the relationship between psychosocial status and hypertension has remained mostly unexplored. The influence of socioeconomic status and psychological well-being on hypertensive condition is assimilated by age as a variable with both biological and social foundations. Age appears not only as a risk factor for high blood pressure but also as an emergent component of psychosocial status. Furthermore, people without higher education are more likely to be known hypertensives with uncontrolled blood pressure values. Social and economic vulnerabilities (e.g., age, education) are interrelated with several health conditions, which support the necessity to develop and implement integrated public policies based on interventions coordinated across several domains. Moreover, social and psychological determinants that predispose to certain health risks should be considered in medical practice.

Visit-to-Visit Blood Pressure Variability and Arterial Stiffness Independently Predict Cardiovascular Risk Category in a General Population: Results from the SEPHAR II Study.

INTRODUCTION: The aim of our study was to evaluate visit-to-visit blood pressure variability (BPV) and the association of this parameter with cardiovascular risk determinants, according to the SEPHAR II survey. METHODS: Following a selection based on the multi-stratified proportional sampling procedure, a total of 1975 subjects who gave informed consent were evaluated by means of a questionnaire, anthropometric, blood pressure (BP) and arterial stiffness measurements (pulse wave velocity and augmentation index), 12-lead ECG recordings, and blood and urine analysis. BPV was quantified in terms of the standard deviation (SD) of the mean systolic blood pressure (SBP) and high BPV was defined as SBP-SD above the 4th quartile. Total cardiovascular risk was assessed by the 2013 ESH/ESC risk stratification chart. RESULTS: Mean BP was 132.37/82.01 mmHg. Mean systolic BPV was 6.16 mmHg, with 24.62% of values above the 75th percentile (8.48 mmHg). Factors found to be associated with high systolic BPV were age, SBP, pulse pressure, total and LDL-cholesterol, triglycerides, visceral obesity, diabetes mellitus, metabolic syndrome and increased aortic stiffness. In addition, in the hypertensive group high BPV was associated with the severity of hypertension and a lack of treatment control. Both visit-to-visit systolic BPV and aortic stiffness proved to be positively and independently correlated with the risk category. Based on these parameters it was possible to predict with 72.6% accuracy the probability of finding subjects in a high and very high cardiovascular risk category. CONCLUSIONS: The results of our study indicate a notable prevalence of high BPV, affecting almost a quarter of the Romanian adult population. Visit-to-visit systolic BPV and arterial stiffness are strongly correlated and together might contribute to the improvement of cardiovascular risk prediction models.

New cardiovascular risk factors and their use for an accurate cardiovascular risk assessment in hypertensive patients.

OBJECTIVES: To analyze the predictive value of new cardiovascular (CV) risk factors for CV risk assessment in the adult Romanian hypertensive (HT) population. METHODS: Hypertensive adults aged between 40-65 years of age, identified in national representative SEPHAR II survey were evaluated by anthropometric, BP and arterial stiffness measurements: aortic pulse wave velocity (PWVao), aortic augmentation index (AIXao), revers time (RT) and central systolic blood pressure (SBPao), 12 lead ECGs and laboratory workup. Values above the 4th quartile of mean SBP' standard deviation (s.d.) defined increased BP variability. Log(TG/HDL-cholesterol) defined atherogenic index of plasma (AIP). Serum uric acid levels above 5.70 mg/dl for women and 7.0 mg/dl for males defined hyperuricemia (HUA). CV risk was assessed based on SCORE chart for high CV risk countries. Binary logistic regression using a stepwise likelihood ratio method (adjustments for major confounders and colliniarity analysis) was used in order to validate predictors of high and very high CV risk class. RESULTS: The mean SBP value of the study group was 148.46±19.61 mmHg. Over forty percent of hypertensives had a high and very high CV risk. Predictors of high/very high CV risk category validated by regression analysis were: increased visit-to-visit BP variability (OR: 2.49; 95%CI: 1.67-3.73), PWVao (OR: 1.12; 95%CI: 1.02-1.22), RT (OR: 0.95; 95% CI: 0.93-0.98), SBPao (OR: 1.01; 95%CI: 1.01-1.03) and AIP (OR: 7.08; 95%CI: 3.91-12.82). CONCLUSION: The results of our study suggests that the new CV risk factors such as increased BP variability, arterial stiffness indices and AIP are useful tools for a more accurate identification of hypertensives patients at high and very high CV risk.