Case Report: Remote magnetic navigation and accessory pathways ablation in a single ventricle young adult with complex corrective surgeries.

Supraventricular arrhythmias have become an increasingly significant contributor to the risk of mortality and morbidity in adults with complex congenital heart disease (CHD), especially in light of recent advances in palliative corrective surgeries. Because of their unique characteristics, they demand specific treatment approaches. While pharmaco-logical interventions are an option, they have limited effectiveness and may lead to side effects. Although performing radiofrequency ablation (RFA) can be exceptionally challenging in patients with complex CHD, due to particular vascular access and also modified anatomy, it has paved the way to enhance comprehension of the underlying mechanisms of supraventricular arrhythmias. This, in turn, enables the provision of improved therapies and, ultimately, an enhancement in the quality of life and symptom management for these patients. The purpose of this case report is to highlight the benefits of utilizing advanced technologies such as three-dimensional electro-anatomical mapping systems, remote magnetic navigation, and highly flexible mapping and ablation catheters during RFA in a young adult with complex congenital heart disease. Although he lacked venous connections to the right atrium (RA) due to multiple corrective surgeries we, remarkably, were capable to advance a decapolar deflectable diagnostic catheter inside the Fontan tunnel and from there to record and stimulate the RA. Successful ablation of two accessory pathways was achieved with no arrhythmia recurrence during follow-up.

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.

Complex Substrate Leading to PVC-Mediated Systolic Dysfunction in addition to Sustained Monomorphic VT in Repaired Tetralogy of Fallot.

Frequent premature ventricular complexes (PVCs) are associated with deleterious effects on left ventricular (LV) function in various clinical scenarios. Repaired tetralogy of Fallot (rTOF) is frequently affected by sustained ventricular arrhythmias dependent on complex post-surgical substrates. However, there is limited data regarding the potential of arrhythmogenic isthmuses to generate frequent PVCs and PVC-mediated LV systolic dysfunction development in rTOF. We present a case of rTOF experiencing relatively infrequent episodes of internal shocks for episodes of sustained monomorphic ventricular tachycardia and a high burden of PVCs associated with left ventricular systolic function deterioration, in which the successful substrate ablation of the anatomical VT isthmuses also led to PVC abolition and consequently to LV systolic function normalization. In such cases, understanding the pathogenic mechanisms that lead to LV dysfunction is only possible by rigorous clinical reasoning, which leads to a tailored specific treatment.

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.

IL-6, IL-1RA and Resistin as Predictors of Left Ventricular Remodelling and Major Adverse Cardiac Events in Patients with Acute ST Elevation Myocardial Infarction.

Despite continuous advances in diagnostic and therapeutic methods, acute myocardial infarction (AMI) remains a leading cause of morbidity and mortality worldwide. Considering the role of inflammation in AMI etiopathogenesis, we aimed to explore the role of a group of three inflammatory cytokines (IL-1RA, IL-6 and resistin) as an independent prognostic factor for LVR assessed by 3D echocardiography and MACE in patients with STEMI. We enrolled 41 patients with STEMI who underwent primary PCI. We assessed the occurrence of LVR (defined as an increase of over 20% in end-diastolic left ventricular volume at 6 months compared with baseline values) and MACE. Using the enzyme-linked immunosorbent assays (ELISA) method, we measured plasmatic levels of IL-6, IL-1RA and resistin (within 48 h after AMI and at 6 months). Out of 41 STEMI patients, 20.5% presented signs of LVR at follow up, and in 24.4%, MACE occurred. In univariate logistic regression analysis, baseline levels of IL-6 (OR = 1.042, p = 0.004), IL-1RA (OR = 1.004, p = 0.05) and resistin (OR = 1.7, p = 0.007) were all significantly associated with LVR. ROC analysis showed that the three cytokines as a group (AUC 0.946, p = 0.000) have a better predictive value for LVR than any individual cytokine. The group of cytokines also proved to have a better predictive value for MACE together than separately (AUC = 0.875, p = 0.000 for ROC regression model). IL-6, IL-1RA and resistin plasma levels at baseline have a good predictive value both as independent variables and also as a group for the development of adverse LVR and MACE at 6 months follow up after STEMI.

Right atrial phasic function and outcome in patients with heart failure and reduced ejection fraction: Insights from speckle-tracking and three-dimensional echocardiography.

BACKGROUND: Atrial phasic function can be assessed using speckle-tracking and three-dimensional (3D) echocardiography. The extent and role of right atrial (RA) dysfunction in left-sided heart failure (HF) is incompletely understood. We aimed to characterize RA phasic function in HF with reduced ejection fraction (HFrEF) and to assess its prognostic significance. METHODS: We prospectively enrolled 60 patients with HFrEF and 29 normal controls. RA phasic function was assessed using strain curves derived from speckle-tracking echocardiography and 3D volumetric analysis. Patients were followed for a composite endpoint of cardiac death or rehospitalization for HF. RESULTS: After a mean follow-up of 19 (9) months, 33 patients reached the primary endpoint. Patients with HFrEF and adverse outcomes showed an impairment of both reservoir, conduit, and booster pump RA function when compared to controls. After adjustment for age, left ventricular systolic and diastolic function, right ventricular systolic function and pulmonary artery pressure, RA maximal and minimal volumes, as well as passive emptying fraction, remained independent predictors of death or rehospitalization (hazard ratio [HR], 3.207; 95% confidence interval [CI], 1.288-7.984; P = 0.012; HR, 2.362, 95% CI, 1.004-5.552; P = 0.049; and HR, 2.367; 95% CI, 1.066-5.259; P = 0.034, respectively). CONCLUSION: All three components of RA phasic function are impaired in left-sided HF. 3D RA maximal and minimal volumes, as well as 3D RA passive emptying fraction, are independent predictors of adverse outcomes in HFrEF.

Three-dimensional right ventriculo-arterial coupling as an independent determinant of severe heart failure symptoms in patients with dilated cardiomyopathy.

BACKGROUND: Right ventricular-pulmonary artery coupling (RVPAC) is a predictor of outcome in pulmonary hypertension. However, the role of this parameter in dilated cardiomyopathy (DCM) remains to be established. The aim of this study was to assess the contribution of RVPAC to the occurrence of severe heart failure (HF) symptoms in patients with DCM using three-dimensional (3D) echocardiography. METHODS: We prospectively screened 139 outpatients with DCM, 105 of whom were enrolled and underwent 3D echocardiographic assessment. RVPAC was estimated non-invasively as the 3D right ventricular stroke volume (SV) to end-systolic volume (ESV) ratio. Severe HF symptoms were defined by New York Heart Association (NYHA) class III or IV. We evaluated differences in RVPAC across NYHA classes and the ability of RVPAC to predict severe symptoms. RESULTS: Mean left ventricular (LV) ejection fraction was 28±7%. Mean RVPAC was 0.77±0.30 and it was significantly more impaired with increasing symptom severity (p = 0.001). RVPAC was the only independent determinant of severe HF symptoms, after adjusting for age, diuretic use, LV systolic function, LV diastolic function, and pulmonary artery systolic pressure (OR 0.035 [95% CI, 0.004-0.312], p = 0.003). By receiver-operating characteristic analysis, the RVPAC cut-off value for predicting severely symptomatic status was 0.54 (area under the curve = 0.712, p < 0.001). CONCLUSION: 3D echocardiographic SV/ESV ratio is an independent correlate of severe HF symptoms in patients with DCM. 3D RVPAC might prove to be a useful risk stratification tool for these patients, should it be further validated in larger studies.

Non-invasive right ventriculo-arterial coupling as a rehospitalization predictor in dilated cardiomyopathy: A comparison of five different methods.

BACKGROUND: Right ventricular (RV) pulmonary artery coupling (RVPAC) is a predictor of outcome in left-sided heart failure (HF). Several echocardiographic estimates for RVPAC have been proposed. AIMS: This study aimed to compare multiple non-invasive methods to calculate RVPAC and to assess its prognostic role in patients with dilated cardiomyopathy (DCM). METHODS: We prospectively enrolled 60 stable patients with DCM. RVPAC was estimated using five methods: as the tricuspid annular plane excursion/pulmonary artery systolic pressure (PASP) ratio; as the RV global longitudinal strain/PASP ratio; as the RV free wall strain (RVFW-LS)/PASP ratio; as the three-dimensional (3D) RV ejection fraction (RVEF)/PASP ratio; and as the 3D RV stroke volume (SV)/end-systolic volume (ESV) ratio. Patients were followed for a mean period of 18 (9) months for the endpoint of HF rehospitalizations. RESULTS: Twenty-nine patients (48%) reached the endpoint. All RVPAC estimates were more impaired in those patients reaching the endpoint (P <0.001 for all) and all predicted rehospitalizations in un-adjusted analysis. RVFW-LS/PASP and RVEF/PASP remained independent predictors of events, after adjustment for clinical and echocardiographic confounders. Using cut-offs obtained from receiver operating characteristic (ROC) analysis, we found that patients with RVFW-LS/PASP >-0.40 and patients with RVEF/PASP <1.30 had a higher risk of HF rehospitalization (log-rank P = 0.001 and P = 0.002, respectively). CONCLUSION: RVFW-LS/PASP and RVEF/PASP as non-invasive estimates of RVPAC are independent predictors of HF rehospitalization in patients with DCM.

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.

The prognostic value of right ventricular longitudinal strain and 3D ejection fraction in patients with dilated cardiomyopathy.

Several studies showed that right ventricular (RV) dysfunction is a powerful predictor in heart failure (HF). Advanced echocardiographic techniques such as speckle-tracking imaging and three-dimensional (3D) echocardiography proved to be accurate tools for RV assessment, but their clinical significance remains to be clarified. The aim of this study was to evaluate the role of two-dimensional (2D) RV strain and 3D ejection fraction (RVEF) in predicting adverse outcome in patients with non-ischemic dilated cardiomyopathy (DCM). We prospectively screened 81 patients with DCM and sinus rhythm, 50 of whom were enrolled and underwent comprehensive echocardiography, including RV strain and 3D RV volumetric assessment. Patients were followed for a composite endpoint of cardiac death, nonfatal cardiac arrest and acute worsening of HF requiring hospitalization. After a median follow-up of 16 months, 29 patients reached the primary endpoint. Patients with events had more impaired RV global longitudinal strain (- 10.5 ± 4.5% vs. - 14.3 ± 5.2%, p = 0.009), RV free wall longitudinal strain (- 12.9 ± 8.7% vs. - 17.5 ± 7.1%, p = 0.046) and 3D RVEF (38 ± 8% vs. 47 ± 9%, p = 0.001). By Cox proportional hazards multivariable analysis, RV global longitudinal strain and RVEF were independent predictors of outcome after adjustment for age and NYHA class. RVEF remained the only independent predictor of events after further correction for echocardiographic risk factors. By receiver-operating characteristic analysis, the optimal RVEF cut-off value for event prediction was 43.4% (area under the curve = 0.768, p = 0.001). Subjects with RVEF > 43.4% showed more favourable outcome compared to those with RVEF < 43.4% (log-rank test, p < 0.001). In conclusion, 3D RVEF is an independent predictor of major adverse cardiovascular events in patients with DCM.

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.

Forgotten No More-The Role of Right Ventricular Dysfunction in Heart Failure with Reduced Ejection Fraction: An Echocardiographic Perspective.

During the last decade, studies have raised awareness of the crucial role that the right ventricle plays in various clinical settings, including diseases primarily linked to the left ventricle. The assessment of right ventricular performance with conventional echocardiography is challenging. Novel echocardiographic techniques improve the functional assessment of the right ventricle and they show good correlation with the gold standard represented by cardiac magnetic resonance. This review summarizes the traditional and innovative echocardiographic techniques used in the functional assessment of the right ventricle, focusing on the role of right ventricular dysfunction in heart failure with reduced ejection fraction and providing a perspective on recent evidence from literature.