Complex Ventricular Arrhythmias Research Articles

Long-term risk assessment in athletes with ventricular arrhythmias: the key roles of arrhythmia morphology, complexity, and substrate

Abstract Background Ventricular arrhythmias (VAs) are an important source of concern among athletes. Purpose To determine the prognostic role of non-invasive and invasive diagnostic assessments among athletes with complex VAs. Methods We included 215 athletes (83% male; 28 [19-43] years) with complex VAs in a prospective, multi-center cohort study. At baseline, each athlete underwent a prespecified comprehensive diagnostic workup, which included cardiac magnetic resonance (CMR) in 202 subjects, and electrophysiology study (EPS)/electroanatomical mapping (EAM) in 190. The primary study endpoint was the occurrence of sudden death or sustained VAs during long-term follow-up. Results During a median follow-up of 6.2 (4.4-8.1) years, 16 (7%) athletes had a primary outcome event, including one sudden cardiac death. In univariable Cox models, sustained VAs at presentation (p<0.001), uncommon VA morphology(p=0.004), syncope(p=0.005), abnormal ECG (p=0.003), VAs induction/persistence at exercise testing(p=0.021), late gadolinium enhancement (LGE;p=0.006), low-voltage areas (p=0.003) and late potentials (p<0.001) at EAM, and sustained VA induction by EPS (p<0.001) were all associated with increased risk of primary outcome events. The association of late potentials and EPS inducibility with primary outcome events was confirmed after controlling for all non-invasive predictors at univariable analysis, and the incorporation of findings of invasive tests allowed improved prediction of primary outcome events over clinical/non-invasive findings in isolation (log-likelihood ratio for nested models, p=0.043). Younger age (p=0.032), being a professional athlete (p=0.006), and absence of LGE (p=0.029) were independently associated with higher odds of uninterrupted competitive sports practice until last follow-up. Conclusions A comprehensive diagnostic assessment integrating clinical/ECG findings with CMR and EPS/EAM in selected cases may provide important prognostic information among athletes with complex VAs.

Arrhythmic mitral valve prolapse with versus without prior mitral valve repair: clinical profiles, electrophysiological substrates, and long-term clinical outcomes

Abstract Background Arrhythmic mitral valve prolapse (AMVP) is characterized by an arrhythmogenic left ventricular substrate, with high prevalence of myocardial scar. Whether patients with prior mitral valve repair for mitral valve prolapse (MVP) and new-onset complex ventricular arrhythmias (VAs) after cardiac surgery have different electroanatomical substrate and arrhythmic outcomes compared with AMVP patients without prior mitral valve surgery is currently unknown. Purpose We sought to compare the electroanatomical substrate and long-term clinical outcomes of patients with new-onset complex VAs after mitral valve surgery to AMVP patients without prior surgery. Methods We conducted a multicenter, prospective, observational study, enrolling MVP patients with prior mitral valve repair and new-onset complex VAs (>1000 premature ventricular contractions [PVCs]/day, nonsustained ventricular tachycardia [NSVT], and sustained VT) after cardiac surgery (group A,n=9), and patients with MVP, complex VAs and no prior surgery (group B,n=20). Each patient underwent a comprehensive diagnostic workup, including left ventricular electroanatomical mapping. Clinical-imaging data, as well as the location and segmental extension of both low-voltage areas and late potentials were assessed and compared between the two groups. The primary outcome was the occurrence of sustained VT or ventricular fibrillation(VF) during follow-up. Results In group A, complex VAs were first diagnosed after a median of 30(8-56) months following mitral valve repair. The characteristics of patients from the two study groups are presented in the Table. Patients from group A were older, sustained VT was nonsignificantly more common at baseline, the PVC count nonsignificantly higher, and LVEF nonsignificantly lower. Mitral annular disjunction was never observed in group A, while it was found in 7 patients from group B (p=0.066). At LV electroanatomical voltage mapping, bipolar and unipolar low-voltage areas (LVAs) were equally common in the two groups overall, and typically involved basal perimitral regions. However, patients from group B had concomitant bipolar and unipolar LVAs 50% of times, with larger unipolar LVAs, while patients from group A mostly showed isolated bipolar LVAs. Over a median follow-up of 15 (12-41) months, patients from group A (n=3, 33%) had slightly higher incidence of sustained VT or VF (HR: 1.91; 95% CI, 1.06-42.65; log-rank p=0.02, Figure) than patients from group B (n=2, 10%). Conclusion Compared to patients with AMVP and no prior cardiac surgery, patients with new-onset complex VAs after mitral valve repair for MVP have a slightly different clinical profile and electroanatomical substrate, characterized by predominant bipolar LVAs in the perimitral region. The long-term risk of major ventricular arrhythmias appears slightly higher in MVP patients with prior mitral valve surgery, mandating at least an equally careful risk assessment in both groups.

Study on attenuation of miR-1-mediated ischemia-induced arrhythmias and infarction in rats by means of fulvning granule

Patients post myocardial infarction (MI) have a high incidence of frequent and complex ventricular arrhythmias. miR-1 is involved in ischemia-induced arrhythmias. Fulvning Granule (FG) is a prescription for treating ischemia-induced arrhythmias. This research investigated therapeutic effect of FG on ischemia-induced arrhythmias in an depth way, focusing on expression of miR-1. 60 healthy Sprague Dawly rats were assigned to operation group, MI+normal saline group, MI+low dose of FG group, MI+moderate dose of FG group and MI+high dose of FG group, MI+propranolol group and MI+moderate dose of FG+propranolol group. Hemodynamic measurement, arrhythmia classification, infarct area evaluation and miR-1 level quantification with expression of PKA and SRF were adopted 4 weeks after operation. FG improved hemodynamic indexes and inhibited expression of miR-1. The optimal dose of FG was medium (P < 0.05). Combination of FG and propranolol further improved the hemodynamics indexes and inhibited the expression of miR-1, PKA and SRF (P < 0.05). FG regulated miR-1 expression via inhibition of Protein Kinase A (PKA) and serum response factor (SRF) expressions. Meanwhile, β-adrenoceptor/PKA signaling pathway played a role in regulating miR-1 expression, while Fulvning granule combined with propranolol and showed an antiarrhythmic role and improved cardiac function after myocardial infarction.

Noncomplex ventricular arrhythmia associated with greater freedom from recurrent ectopy at 1 year after mitral repair surgery

ObjectiveThe effect of mitral valve (MV) surgery on the natural history of ventricular arrhythmia (VA) in patients with arrhythmic MV prolapse remains unknown. We sought to evaluate the cumulative incidence of VA at 1 year after surgical mitral repair. MethodsA retrospective review of progressively captured data identified 204 consecutive patients who underwent elective MV repair for significant degenerative mitral regurgitation as a first-time cardiovascular intervention in a quaternary reference center between January 2018 and December 2020. A subset of 62 consecutive patients with diagnosed arrhythmic MV prolapse was further evaluated for recurrent VA after MV repair. ResultsThe median age was 62 years (range, 27-77 years) and 26 of 62 (41.9%) were female. The median time from initial mitral regurgitation/MV prolaspe diagnosis-to-referral was 13.8 years (interquartile range [IQR], 5.4-25) and from VA diagnosis-to-referral was 8 years (IQR, 3-10.6). Using the Lown-Wolf classification, complex VA (Lown grade ≥3) was identified in 36 of 62 patients (58%) at baseline, whereas 8 of 62 (13%) had a cardioverter/defibrillator implanted for primary (4/8) or secondary (4/8) prevention. Left ventricular myocardial scar was confirmed in 23 of 34 (68%) of patients scanned at baseline. The prevailing valve phenotype was bileaflet Barlow (59/62; 95.2%). All patients underwent surgical MV repair by the same team. Surgical repair was stabilized with an annuloplasty prosthesis (median size 36 mm [IQR, 34-38]). Concomitant procedures included tricuspid valve repair (51/62; 82.3%), cryo-maze ± left atrial appendage exclusion (14/62, 23%), and endocardial cryoablation of VA ectopy (4/62; 6.5%). The 30-day and 1-year freedom from recurrent VA were 98.4% and 75.9%, respectively. Absent VA after mitral repair was uniformly observed in patients with minor VA at baseline. Absent VA after mitral repair was uniformly observed in patients with minor VA preoperatively. Complex baseline VA was the strongest predictor of recurrent VA (hazard ratio, 10.8; 95% confidence interval, 1.4-84.2; P = .024), irrespective of myocardial fibrosis. ConclusionsIn a series of 62 consecutive patients operated electively for arrhythmic mitral prolapse, VA remained undetected in 75.9% of patients at 1 year. Freedom from recurrent VA was greater among patients without complex VA preoperatively, whereas baseline Lown grade ≥3 was the strongest independent risk factor for recurrent VA at 1 year. These findings attest to the importance of early recognition and prompt referral of patients with mitral prolapse and progressive VA to specialty interdisciplinary care.

Impact of left ventricular hypertrophy on frequency and complexity of ventricular arrhythmia among hypertensive Egyptian patients

BackgroundLeft ventricular hypertrophy (LVH) is associated with an increased risk of ventricular arrhythmias and cardiovascular mortality. The study objective was to investigate the effect of LVH severity on the complexity and severity of ventricular arrhythmias among a subset of Egyptian hypertensive patients.ResultsThe study cohort consisted of 60 hypertensive patients with LVH as diagnosed by echocardiography. Their mean age was (53.7 ± 12.3) years. 36 males (40%) and 24 females (60%). Diabetes mellitus was found in 26 patients (43%), 41% of these cases were smokers. 48-h Holter monitoring was performed in all cases to assess the frequency of ventricular premature contractions (VPC) and their complexity using the Lown grading. Increasing posterior wall thickness end diastole (PWTd) was an independent predictor of increasing VPC frequency, each 1 mm increase in the PWTd is associated with a 1.26% increase in the VPC% among total heart beats (b = 12.6, p < 0.001). Higher-grade VPCs—defined as grade 4a, 4b, and 5—were seen in 29 patients (48.3%). Interventricular septum thickness end diastole, PWTd, left ventricular mass, and left ventricular mass index (LVMI), were significantly higher among patients with higher Lown grading (p < 0.001). Using logistic regression analysis, female sex and LVMI were independent predictors of more complex VPC (OR = 8.766, p = .014), (OR = 1.096, p < 0.001), respectively. Among females, LVMI of more than 120 g/m2 can differentiate between high- and low-grade VPCs with 71% sensitivity and 80% specificity, while among males, LVMI of more than 129.5 g/m2 can differentiate between high and low-grade VPCs with 86% sensitivity and 66% specificity.ConclusionsThe frequency and complexity of ventricular arrhythmias among hypertensive heart disease are correlated with the severity of ventricular hypertrophy. Female sex and increasing left ventricular mass index are independent predictors of more complex forms of ventricular arrhythmias.

Predictors of and outcomes in arrhythmic mitral valve prolapse

Abstract Background Recently, the European Heart Rhythm Association (EHRA) defined the phenotype of the arrhythmic mitral valve prolapse (AMVP) complex and proposed management approaches. Assessing the incidence of SCD in this small subset of patients is challenging due to the low event-rate and longitudinal real-world data remain scarce. Purpose To determine the predictors of AMVP and study the clinical outcome of patients stratified by ICD indication as proposed by the EHRA consensus statement. Methods All consecutive patients with MVP who underwent cardiac magnetic resonance imaging (CMR) were enrolled in a single-center retrospective registry. AMVP was defined as the presence of frequent or complex ventricular arrhythmias (&amp;gt;5% total PVC burden, non-sustained ventricular tachycardia (VT), VT or ventricular fibrillation) in a patient with MVP without alternative arrhythmic substrates. The medical records of these patients included a comprehensive clinical, rhythmic and echocardiographic characterization allowing to collect the key date of transition to AMVP. A forward conditional multivariable logistic regression was performed to identify clinical risk factors for AMVP reporting the odds ratio (OR) and corresponding 95% confidence interval (95% CI). Incidence rates were calculated for the composite endpoint of SCD, sustained VT, ICD shocks, and all-cause mortality. Results A total of 169 patients (52.1% male, median age 51.4 years) were included, of which 99 (58.6%) were classified as AMVP. Clinical characteristics are presented in Image 1. Patients with non-arrhythmic MVP and AMVP had comparable left ventricular function, mitral valve regurgitation grade and extent of MVP. Multivariable logistic regression identified the presence of late gadolinium enhancement (LGE, OR 2.82, 95%CI 1.45–5.50, p=0.002) and mitral annular disjunction (MAD, OR 1.98, 95%CI 1.02–3.86, p=0.045) on CMR as only predictors of AMVP. Cardiac CMR results are presented in Image 2. The median overall follow-up since first cardiac contact was 8.0 years (IQR 5.0–15.6), while this was 5.0 years (IQR 2.1–7.4 years) after transition to AMVP. The incidence for the composite endpoint in the overall population was 0.4%/year (95%CI 0.2 – 0.9). The 5-year cumulative event rate was 1.8%. According to the EHRA risk stratification the implantation of an ICD would have been reasonable in 69 patients (69.7%). In total, 5 patients were implanted with an ICD for secondary prevention, but all those patients did not classify as AMVP prior to the life threathening arrhythmic event. Conclusion CMR is an important tool in the challenging risk stratification of AMVP given that the presence of MAD and LGE enhancement are predictors of AMVP. There is a lack of longitudinal data to determine when patients transition from MVP to AMVP and how this modifies their outcome.Clinical characteristics and ECG resultsOverview of CMR measurements

Arrhythmic form of mitral valve prolapse: presentation, risk stratification and management

Abstract Background Mitral valve prolapse (MVP) is a common finding that has been considered as benign condition for a long time. However, some anatomic forms have been recently recognized to be associated with life-threatening ventricular arrhythmia and sudden cardiac death (SCD), recently known as arrhythmic MVP (AMVP). Objectives This study is aiming to clinically characterize and risk stratify patients with malignant phenotype of MVP of high risk for ventricular arrhythmia which may lead to SCD. We aim to explore characteristics and assess potential markers for MVP induced ventricular arrhythmia that might need monitoring and follow up with medical or invasive treatment as implantable cardioverter-defibrillator, catheter ablation or even surgical repair. Methods A prospective cohort study of 45 (age 35±8, 29 females), consecutive patients with MVP and/or mitral annular disjunction (MAD) and comprehensive clinical arrhythmia on resting electrocardiography (ECG) or 48-hours Holter monitoring , Doppler echocardiography and contrast based cardiac magnetic resonance (CMR) characterization were identified. Patients were followed-up for a period of maximum one year to identify any further management needed. Results Frequent ventricular arrhythmia was evidently seen associated to MVP (69% with at least ventricular ectopy ≥5%), as well as complex ventricular arrhythmia (31% with sustained or non-sustained ventricular tachycardia ) P&amp;lt;0.001. AMVP was strongly associated with premature ventricular contractions (PVCs) having Right bundle branch block (RBBB) morphology on ECG and late gadolinium enhancement (LGE) on CMR (all p&amp;lt;0.05). Furthermore, it was shown to be associated with bileaflet prolapse and higher left ventricular volumes, however not statistically significant. On Follow-up, Out of 36 patients with ventricular arrhythmia, 64% were controlled on medical anti-arrhythmic and some others were referred either for ablation after worsening of symptoms despite of medical treatment (27.8%), implantable cardioverter-defibrillator device for secondary VT prophylaxes (5.6%) or surgery (2.8%) due to progression in mitral regurgitation severity and deterioration of symptoms. Conclusion In this small cohort study, characteristic form of MVP was found to be associated with ventricular arrhythmia more common in the form of frequent PVCs and less common in the complex form. Although majority of patients were controlled on medical treatment, few patients required further invasive management despite of medical treatment . Thus, arrhythmia associated with a special form of MVP could be life threatening and needs close monitoring, long follow-up and proper management approach to prevent SCD. Large prospective studies with comprehensive approach and long term follow up is pivotal. CMR T1 mapping and electro anatomical CARTO mapping could help in better detection and proper defining of arrhythmia substrate in future studies.AMVP illustration

Abstract 16985: Spatial Accuracy of Non-Invasive Activation Mapping Modalities for Complex Ventricular Arrhythmias: A Direct Comparison of Electromechanical Wave Imaging (EWI) Using High Frame Rate Transthoracic Echocardiography and Electrocardiographic Imaging (ECGi) With Cardiac CT

Background: Non-invasive identification of the site of origin (SOO) of ventricular arrhythmias is vital in informing ablation strategy. ECGi is an established method to generate 3D activation maps with a multielectrode vest combined with cardiac CT. EWI is an emerging echocardiography based modality that provides a low cost &amp; non-ionizing mapping alternative. Hypothesis: EWI more precisely localises SOO of Complex Ventricular Ectopy (VEs) &amp; intramural location than commercial ECGi. Aim: Compare spatial accuracy of EWI and ECGi to estimate SOO and validate against contact mapping. Methods: VE-ablation patients underwent preprocedural EWI &amp; ECGi to estimate SOO on the AHA segment-model. A commercial ECGi system with cardiac CT was used for reconstruction of epicardial VE activation maps. EWI was performed using a research ultrasound acquiring B-mode and high frame rate (2000fps) images with simultaneous ECG. Local electromechanical activation was defined as time-point of the downward zero-crossing on the incremental axial strain curve (250 strain curves/view) and displayed on 3D rendered maps. The site of earliest activation &amp; successful VE ablation was defined as ground truth for VE SOO. Results: 10 patients were enrolled: 50% male, age 40.8 +/- 18.1 years, LV EF 41+/-15%, 50% with scar on MRI. CT ECGi correctly identified the VE AHA segment in 8/10 (80%) cases (misclassified 2 papillary muscle (PM) VEs) but did not afford transmural localization. After excluding 1 patient with insufficient VE’s for EWI, EWI correctly identified the VE-SOO segment in 8/9 (88%) cases locating 2 subepicardial, 2 septal intramural &amp; 3 VEs at the base or intramural segment adjacent to a PM. It misclassified 1 PM VE. Conclusion Both EWI &amp; ECGI identified the VE-SOO segment in at least 80% of cases irrespective of presence of scar. EWI also correctly determined the transmural VE origin which cannot be located using commercial ECGI. This has important implications in planning ablation procedures.

Periodic health evaluation in athletes competing in Tokyo 2020: from SARS-CoV-2 to Olympic medals

BackgroundThe Tokyo Olympic games were the only games postponed for a year in peacetime, which will be remembered as the COVID-19 Olympics. No data are currently available on the effect...

Use of PET/CT to detect myocardial inflammation and the risk of malignant arrhythmia in chronic Chagas disease.

Chagas heart disease (CHD) is characterized by progressive myocardial inflammation associated with myocardial fibrosis and segmental abnormalities that may lead to malignant ventricular arrhythmia and sudden cardiac death. This arrhythmia might be related to the persistence of parasitemia or inflammation in the myocardium in late-stage CHD. Positron emission tomography/computed tomography (PET/CT) has been used to detect myocardial inflammation in non-ischemic cardiomyopathies, such as sarcoidosis, and might be useful for risk prediction in patients with CHD. Twenty-four outpatients with chronic CHD were enrolled in this prospective cross-sectional study between May 2019 and March 2022. The patients were divided into two groups: those with sustained ventricular tachycardia and/or aborted sudden cardiac death who required implantable cardioverter-defibrillators, and those with the same stages of CHD and no complex ventricular arrhythmia. Patients underwent 18F-fluorodeoxyglucose (18F-FDG) and 68Ga-DOTATOC PET/CT, and blood samples were collected for qualitative parasite assessment by polymerase chain reaction. Although similar proportions of patients with and without complex ventricular arrhythmia showed 18F-FDG and 68Ga-DOTATOC uptake, 68Ga-DOTATOC corrected SUVmax was higher in patients with complex arrhythmia (3.4 vs 1.7; P = .046), suggesting that inflammation could be associated with the presence of malignant arrhythmia in the late stages of CHD. We also detected Trypanosoma cruzi in both groups, with a nonsignificant trend of increased parasitemia in the group with malignant arrhythmia (66.7% vs 33.3%). 18F-FDG and 68Ga-DOTATOC uptake on PET/CT may be useful for the detection of myocardial inflammation in patients with Chagas cardiomyopathy, and 68Ga-DOTATOC uptake may be associated with the presence of malignant arrhythmia, with potential therapeutic implications.

Prevalence and prognosis of structural heart disease among athletes with negative T waves and normal transthoracic echocardiography.

The aim of the present study was to evaluate the prevalence and prognosis of structural heart disease (SHD) among competitive athletes with negative T waves without pathological findings at transthoracic echocardiogram. From a prospective register of 450 athletes consecutively evaluated during a second-level cardiological examination, we retrospectively identified all subjects with the following inclusion criteria: (1) not previously known cardiovascular disease; (2) negative T waves in leads other than V1-V2; (3) normal transthoracic echocardiogram. Patients underwent cardiac MRI and CT. The primary endpoint was the diagnosis of definite SHD after multimodality imaging evaluation. A follow-up was collected for a combined end-point of sudden death, resuscitated sudden cardiac death and hospitalization for any cardiovascular causes. A total of 55 competitive athletes were finally enrolled (50 males, 90%) with a mean age of 27.5 ± 14.1 years. Among the population enrolled 16 (29.1%) athletes had a final diagnosis of SHD. At multivariate analysis, only deep negative T waves remained statistically significant [OR (95% CI) 7.81 (1.24-49.08), p = 0.0285]. Contemporary identification of deep negative T waves and complex arrhythmias in the same patients appeared to have an incremental diagnostic value. No events were collected at 49.3 ± 12.3 months of follow-up. In a cohort of athletes with negative T waves at ECG, cardiac MRI (and selected use of cardiac CT) enabled the identification of 16 (29.1%) subjects with SHD despite normal transthoracic echocardiography. Deep negative T waves and complex ventricular arrhythmias were the only clinical characteristic associated with SHD diagnosis.

Ventricular arrhythmias recorded on 12-lead ambulatory electrocardiogram monitoring in healthy volunteer athletes and controls: what is common and what is not.

Premature ventricular beats (PVBs) in athletes are often benign, but sometimes they may be a sign of an underlying disease. We evaluated the prevalence, burden, and morphology of PVBs in healthy voluntary athletes and controls with the main purpose of defining if certain PVB patterns are 'common' and 'training related' and, as such, are more likely benign. We studied 433 healthy competitive athletes [median age 27 (18-43) years, 74% males] and 261 age- and sex-matched sedentary subjects who volunteered to undergo 12-lead 24 h ambulatory electrocardiogram (ECG) monitoring (24H ECG), with a training session in athletes. Ventricular arrhythmias (VAs) were evaluated in terms of their number, complexity [i.e. couplet, triplet, or non-sustained ventricular tachycardia (NSVT)], exercise inducibility, and morphology. Eighty-six percent of athletes and controls exhibited a total of ≤10 PVBs/24 h, and >90% did not show any couplets, triplets, or runs of NSVT > 3 beats. An higher number of PVBs correlated with increasing age (P < 0.01) but not with sex and level of training. The most frequent morphologies among the 36 athletes with >50 PVBs were the infundibular (44%) and fascicular (22%) ones. In a comparison between athletes and sedentary individuals, and male and female athletes, no statistically significant differences were found in PVBs morphologies. The prevalence and complexity of VAs at 24H ECG did not differ between athletes and sedentary controls and were not related to the type and amount of sport or sex. Age was the only variable associated with an increased PVB burden. Thus, no PVB pattern in the athlete can be considered 'common' or 'training related'.

Dry suction water seal system for management of pericardial fluid during epicardial ablation.

Epicardial ablation is an important approach in the management of patients with complex ventricular arrhythmias. Irrigated ablation catheters present a challenge in this potential space due to fluid accumulation that can cause hemodynamic compromise, requiring frequent manual fluid aspiration. In this series, we report our initial experience with the use of a dry suction water seal system for pericardial fluid management during epicardial ablation. Consecutive patients undergoing epicardial ventricular tachycardia (VT) ablation at a single center were included. All patients underwent epicardial access via a subxiphoid approach with a single operator. A deflectable sheath was advanced into the pericardial space, and the side port was attached to a dry suction water seal system attached to wall suction at -20 mmHg. Procedural information including patient characteristics, outcomes, and adverse events. After a period of initial experience, pericardial fluid infusion and aspiration volumes were recorded. Eleven patients were included in this series. All patients underwent epicardial ablation with complete success achieved in 8 of the 11 patients and partial success in the remaining patients. Pericardial fluid intake ranging from 485 to 3050 mL with aspiration of 350-3050 mL using the dry suction water seal system. No adverse events occurred. Dry suction water seal drainage systems can provide a safe strategy for efficient pericardial fluid management during epicardial VT ablation, potentially shortening procedure duration.

The impact of fragmented QRS on clinical findings and outcomes in children with dilated cardiomyopathy with or without left ventricular non-compaction.

The aim of this study is to investigate the frequency of fragmented QRS and its associations with clinical findings and prognosis in children diagnosed with dilated cardiomyopathy with or without left ventricular non-compaction. This retrospective study was conducted between 2010 and 2020. Patients with dilated cardiomyopathy were classified into two groups according to the presence of left ventricular non-compaction: Dilated cardiomyopathy with left ventricular non-compaction and dilated cardiomyopathy without left ventricular non-compaction. Patients were also divided into two groups according to the presence of fragmented QRS (fragmented QRS group and non-fragmented QRS group). Twenty-three of 44 patients (52.3%) were male. Among left ventricular non-compaction patients, the fragmented QRS group had more complex ventricular arrhythmias (p = 0.003). Patients with fragmented QRS had a significantly higher rate of major adverse cardiac events and/or cardiac death in both cardiomyopathy groups (p = 0.003 and p = 0.005). However, the rate of major adverse cardiac events and/or cardiac death was similar between dilated cardiomyopathy patients with and without left ventricular non-compaction. Multivariate logistic regression analysis showed that the presence of fragmented QRS strongly predicts major adverse cardiac events and/or cardiac death (odds ratio, 31.186; 95% confidence interval, 2.347-414.307). Although the survival rates between cardiomyopathy groups were similar, patients with fragmented QRS had a markedly lower survival rate during the follow-up period, as mean of 15 months (p = 0.001). Our study showed that the presence of fragmented QRS may be an important ECG sign predicting an major adverse cardiac event and/or cardiac death in patients with dilated cardiomyopathy. We believe that recognising fragmented QRS could be valuable in forecasting patient prognosis and identifying high-risk patients who require additional support.

Does Rassi score caracterize patients with Chagas disease with complex ventricular arrhythmias?

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Rassi score (NYHA functional class III/IV HF [5 points], cardiomegaly [5 points], contractions abnormalities [3 points], non-sustained ventricular tachycardia [3 points], low amplitude QRS [2 points] and male sex [2 points]) is used in the clinic for risk stratification due to cardiovascular death in patients with Chagas disease (P-CD). Whether this score has any role in identifying patients at risk of complex ventricular arrhythmias has not been determined. Objective Assess whether there is an association between Rassi score and the presence of potentially malignant complex ventricular arrhythmias in P-CD. Methods 79 P-CD (34 men, age 61±11 years-old), consecutively referred for 24h-Holter were divided into 2 groups: low risk Rassi score (57 P, 0-6 points) and medium-high risk (22 P, 7-20 points). The amount and complexity of ventricular arrhythmia (&amp;gt; 30/ h, ventricular tachycardia [VT]), LVEF, heart rate turbulence (HRT) indexes (turbulence onset [TO] and turbulence slope [TS]) and HRT category (0 [without disautonomy], 1 and/or 2 [presence of disautonomy]) were evaluated. Results Compared to P-CD with low risk score, those with medium-high risk had more ventricular arrhythmias, lower LVEF, worse HRT indexes and more HRT categories 1 and/or 2 (see table below). Conclusions 1) P-CD and Rassi score of medium to high risk have a greater amount of ventricular arrhythmias; 2) These findings can be explained by the presence of greater degree of disautonomy, assessed by HRT, and by the lower LVEF; 3) Prospective studies are necessary to confirm these findings in clinical practice.

Multimodality imaging for negative T waves among athletes with normal transthoracic echocardiography: prevalence and prognosis of structural heart disease

Abstract Funding Acknowledgements Type of funding sources: None. Introduction The aim of the present study was to evaluate the prevalence of structural heart disease (SHD) among competitive athletes with negative T waves without pathological findings at transthoracic echocardiogram, evaluated at cardiac MRI and cardiac CT. Methods From a prospective register of 450 athletes consecutively evaluated during a second-level cardiological examination, we retrospectively identified all subjects with the following inclusion criteria: 1)not previously known cardiovascular disease; 2)negative T waves in leads other than V1-V2; 3)normal transthoracic echocardiogram. Patients underwent cardiac MRI and CT. The primary endpoint was the diagnosis of definite SHD after multimodality imaging evaluation. A follow-up was collected for a combined end-point of sudden death, resuscitated sudden cardiac death and hospitalization for any cardiovascular causes. Results A total of 55 competitive athletes were finally enrolled (50 males, 90%) with a mean age of 27.5±14.1 years old. Among the population enrolled 16 (29.1%) athletes had a final diagnosis of SHD. At multivariate analysis, only deep negative T waves remained statistically significant [OR(95%CI) 7.81(1.24–49.08), p=0.0285]. Contemporary identification of deep negative T waves and complex arrhythmias in the same patients appeared to have an incremental diagnostic value. No events were collected at 49.3±12.3 months of follow-up. Conclusion In an unselected cohort of athletes with negative T waves at ECG, cardiac MRI (and selected use of cardiac CT) enabled the identification of 16 (29.1%) subjects with SHD despite normal transthoracic echocardiography. Deep negative T waves and complex ventricular arrhythmias were the only clinical characteristic associated with SHD diagnosis.

P403 MITRAL VALVE PROLAPSE AND RISK OF SUDDEN CARDIAC DEATH IN A PATIENT WITH VENTRICULAR ARRHYTHMIAS: THE PROGNOSTIC ROLE OF CARDIAC MAGNETIC RESONANCE IMAGING

Abstract Introduction in most cases mitral valve prolapse is a benign condition, however in 2% of cases it has been associated with the risk of sudden cardiac death (SCD). Basso et al have demonstrated papillary muscle’s and left ventricle’s inferolateral wall fibrosis on cardiac magnetic resonance imaging (MRI), in patients who died from SCD with mitral valve prolapse. Clinical case A 59–year–old patient was admitted to the Cardiology Department of Pertini Hospital (Rome, Italy) for episodes of vertigo and not sustained ventricular tachycardia in two morphologies at 24–hour ECG monitoring. The patient had negative history for cardiovascular risk factors, but an echocardiogram had demonstrated a moderate mitral valve regurgitation, secondary to valve prolapse with mitral annular disjunction (MAD) (Fig.1). Considering patient‘s age associated with low pre–test probability of coronary artery disease, the patient underwent coronary CT that showed a coronary artery tree free from injury. Cardiac MRI showed increased volumes in the left ventricle and slightly reduced systolic function and valve prolapse of both mitral leaflets determining moderate regurgitation. In addition, intra–myocardial Late Gadolinium Enhancement (LGE) showed fibrosis of the inferior and inferolateral wall with extension to the proximal portion of the inferior papillary muscle (Fig.2). Considering such findings, an intracavitary electrophysiological study (EPS) was performed for an evaluation of the arhythmic burden, by administrating the patient a double extra stimulus from the outflow tract of the right ventricle; the treatment induced a polymorphic ventricular tachycardia, starting from the inferior papillary muscle and degenerating into ventricular fibrillation (Fig.3). Discussion considering all the above findings, a mitral valve prolapse at high risk of malignant arrhythmias has been diagnosed and a subcutaneous cardiac defibrillator was implanted. Conclusions in mitral valve prolapse associated with complex ventricular arrhythmias, evaluation with cardiac MRI increasingly plays a crucial role in prognostic stratification for defibrillator implantation in primary prevention.

P325 CARDIAC SARCOIDOSIS PRESENTING WITH ATRIOVENTRICULAR BLOCK AND SUBSEQUENT CARDIAC ARREST: A CASE REPORT

Abstract A 53–year–old male patient with no significant past medical history and no familiar history for cardiac diseases presented to the emergency department for asymptomatic bradycardia. The ECG showed complete atrioventricular block in the absence of pathological findings on the echocardiogram and no coronary lesions were found on coronary angiography. The patient underwent pacemaker (PM) implantation. A few months later the patient suffered from cardiac arrest in sustained ventricular tachycardia, effectively treated with DC shock. The echocardiogram showed mild right ventricular dilation and dysfunction with regional wall motion abnormalities, especially in the subtricuspid site. Cardiac magnetic resonance imaging (MRI) showed focal areas of delayed enhancement with a non–ischaemic pattern and distribution with a hook sign, highly suggestive of cardiac sarcoidosis. FDG PET showed cardiac, hepatic, splenic, and lymph nodal uptake. In the suspicion of systemic sarcoidosis, an hepatic biopsy was performed, being the liver the most accessible site. The histological examination showed the presence of non–caseating granulomas. Therefore, a diagnosis of Systemic Sarcoidosis (SS) with Cardiac involvement was made and the PM was upgraded to CRT–D in secondary prevention. The patient was discharged with cortisone therapy. Three months after discharge, a new total body PET–CT scan was performed and showed, in comparison to the previous one, the absence of areas of significant hypermetabolism in the cardiac, hepatic and bone fields. Cardiac sarcoidosis (CS) is a rare inflammatory granulomatous myocardial disease of unknown etiology that should be suspected in young patients with atrioventricular conduction disturbances and complex ventricular arrhythmias. The diagnostic approach is challenging: imaging techniques (MRI, PET) can be suggestive of CS but biopsy with histological demonstration of the non–caseous granuloma is mandatory. Endomyocardial biopsy (which has suboptimal sensitivity due to focal lesion) could be avoided if there is a more accessible and less invasive extracardiac site for biopsy. Early diagnosis relies on multidisciplinary collaboration and is essential for staging and long–term management of the disease and prevention of sudden cardiac death. Cortisone therapy remains the mainstay of treatment.

P356 MARCHE REGIONAL NETWORK FOR CARDIAC AMYLOIDOSIS AND HYPERTOPHIC CARDIOMYOPATHY: INSIGHTS FROM 3–YEAR COLLABORATIVE EXPERIENCE

Abstract Background and Methods The management of patients with Cardiac Amyloidosis (CA) and Hypertrophic Cardiomyopathy (HCM) is complex and requires specific skills. Collaboration between centres remains essential because not all centres can perform complex diagnostic techniques (such as endomyocardial biopsy and mass spectrometry) or prescribe disease–modifying therapies, Therefore, in 2019 we created a collaborative network for diagnosis and treatment of CA (TTR and AL) and HCM (sarcomeric and phenocopies) with the aim of enhancing disease awareness among physicians and favoring appropriate access to innovative diagnostic tools/therapies. The network includes all 14 Cardiology Departments and outpatient clinics from 5 Local Health Units. At Referral Centre of the Regional University Hospital, all diagnostic and therapeutic tools necessary for a comprehensive management of CM patients including genetic testing, endomyocardial biopsy, ablation of complex ventricular arrhythmias, and septal myectomy for HOCM, are currently available and at disposal for the entire network. Results Thanks to this network, from December 2019 to December 2022, 283 patients were referred to our Center (fig. 1). Of these, 136 (48%) had CA, 122 (43%) were diagnosed with HCM (68 with obstructive form and 54 with non–obstructive form), 12 (4%) with Anderson Fabry Disease (AFD) and 13 (5%) with other forms of cardiomyopathy (miscellanea: arrhythmogenic, LVNC, etc). As for temporal trends in diagnosis of CM patients, in the first 2 years the diagnosis of HCM was more frequent than that of CA, while the number of CA patients has steadily grown, overtaking that of HCM patients in the last year (fig.2). The number of AFD diagnoses was consistently low over the 3 years, despite the spread use of genetic testing in all HCM patients. All patients with CA (136) were managed according to current algorythm with need of EMB in uncertain cases (8). Of the 136 patients with AC, 122 had TTR while 14 had AL (Fig.3). All patients with ATTR (122) underwent genetic testing: in 14 cases the presence of mutations in the TTR gene was documented (8 Ile68Leu; 4 Val30Met; 2 Val122Ile). 51 ATTR patients were treated with tafamidis, while 3 patients with variant–TTR and neuropathy received patisiran iv. Conclusions Implementing specific clinical network provided excellent results, allowing a precise phenotype/genotype characterization and favoring appropriate access to specific disease–modifying drugs.

Protective role of chronic beta-blocker intake against the appearance of complex ventricular arrhythmias in acute coronary syndrome

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Although the presence of ventricular arrhythmias (ventricular tachycardia and ventricular fibrillation) in the context of acute coronary syndromes (ACS) has decreased in the era of early reperfusion, their occurrence may lead to fatal consequences. However, clear predisposing or protective factors have not been identified yet. Purpose Our purpose is to describe the prevalence of ventricular arrhythmias before reperfusion treatment in a population of patients hospitalized due to ACS, as well as the possible protective role of chronic intake of beta-blockers, among other factors. Material and methods Retrospective, single-center and observational registry of patients admitted to a Coronary Care Unit from July 2011 to September 2022 with a diagnosis of ACS. The baseline characteristics were described, as well as factors related to diagnosis and treatment. A multivariate analysis was performed to study possible predictors of arrhythmic events. Results A total of 3123 patients diagnosed with ACS were included. 682 patients (21.8%) had beta- blockers as a chronic treatment and 201 patients (7.1%) presented ventricular arrhythmias. In Table 1 we can see the baseline characteristics of both groups, as well as the differences between them. Previous intake of beta-blockers was associated with a lower incidence of ventricular arrhythmias in patients with ST-segment elevation myocardial infarction (STEMI) (11% vs 6%, p 0.002), while no differences were observed between patients without ST-segment elevation myocardial infarction (NSTEMI) (2.3% vs 1.6%, p = 0.435). As shown in the multivariate analysis (Table 2), prior intake of beta-blockers turned out to be a protective factor against the development of ventricular arrhythmias, while ST elevation and worse initial Killip were risk factors. Conclusion Chronic intake of beta-blockers could be a protective factor against the development of ventricular arrhythmias before revascularization in patients diagnosed with acute coronary syndrome, specially those with STEMI.