Left Ventricular Lateral Wall Research Articles

A novel method combining gated SPECT and vectorcardiography to guide left ventricular lead placement to improve response to cardiac resynchronization therapy: A Proof of Concept study

BackgroundThe segment of the latest mechanical contraction (LMC) does not always overlap with the site of the latest electrical activation (LEA). By integrating both mechanical and electrical dyssynchrony, this proof-of-concept study aimed to propose a new method for recommending left ventricular (LV) lead placements, with the goal of enhancing response to cardiac resynchronization therapy (CRT). MethodsThe LMC segment was determined by single-photon emission computed tomography myocardial perfusion imaging (SPECT MPI) phase analysis. The LEA site was detected by vectorcardiogram. The recommended segments for LV lead placement were as follows: (1) the LMC viable segments that overlapped with the LEA site;(2) the LMC viable segments adjacent to the LEA site;(3) If no segment met either of the above, the LV lateral wall was recommended. The response was defined as ≥15% reduction in left ventricular end-systolic volume (LVESV) 6-months after CRT. Patients with LV lead located in the recommended site were assigned to the recommended group, and those located in the non-recommended site were assigned to the non-recommended group. ResultsThe cohort comprised of 76 patients, including 54 (71.1%) in the recommended group and 22 (28.9%) in the non-recommended group. Among the recommended group, 74.1% of the patients responded to CRT, while 36.4% in the non-recommended group were responders (p=.002). Compared to pacing at the non-recommended segments, pacing at the recommended segments showed an independent association with an increased response by univariate and multivariable analysis (odds ratio 5.00, 95% confidence interval 1.73-14.44, p=.003; odds ratio 7.33, 95% confidence interval 1.53-35.14, p=.013). Kaplan-Meier curves showed that pacing at the recommended LV lead position demonstrated a better long-term prognosis. ConclusionsOur findings indicate that pacing at the recommended segments, by integrating of mechanical and electrical dyssynchrony, is significantly associated with an improved CRT response and better long-term prognosis.

Myocardial deformation characteristics assessed by cardiovascular magnetic resonance feature tracking in a healthy Chinese population

PurposeTo explore global/regional myocardial deformation across various layers, vascular distributions, specific levels and distinct walls in healthy individuals using cardiovascular magnetic resonance feature tracking (CMR-FT). MethodsWe selected a cohort of 55 healthy participants and CMR cine images were used to obtain the left ventricular (LV) peak longitudinal, circumferential, radial strains (LS, CS, RS). The characteristics of normal LV strain in various layers (endocardium, myocardium, epicardium), territories [left anterior descending artery (LAD), left circumflex artery (LCX), and right coronary artery (RCA)], levels (basal, middle, apical) and walls (anterior, septum, inferior, lateral) were compared. ResultsThe absolute values of the LV global LS and CS gradually decreased from endocardium to epicardium. The absolute LV global RS (65.7 ± 47.7%) was maximum relative to LS (−22.0 ± 10.8%) and CS (−22.8 ± 7.7%). The absolute values of the LCX territorial strain were the largest compared with the LAD and RCA territorial strains. Regional RS, endo-CS and endo-LS gradually increased from the basal to the apical level. The LV lateral walls had the highest strain values (CS, LS, and RS). ConclusionsVariations in normal LV strain values across various layers, territories, levels, and walls were observed, suggesting the necessity for careful clinical interpretation of these strain values. These findings also partially revealed the complexity of normal cardiac mechanics.

Radiofrequency catheter ablation of ventricular arrhythmias in patients with hypertrophic cardiomyopathy

BackgroundCompared to other non-ischemic substrates, there is limited data on the role and outcome of catheter ablation in HCM. The objective of this study is to assess the safety and efficacy of catheter ablation for the treatment of VT in patients with HCM. MethodsFourteen patients with HCM and drug refractory VT who underwent catheter ablation at a single center were included in this study. The data was evaluated retrospectively. Acute success, procedure-related complications, and long-term outcomes were documented during follow up. ResultsAmong the 14 patients (mean age 48.2 ± 8.2 years, 85.7% males, mean LVEF 42.6 ± 6.5%), 4 had an apical aneurysm. Eleven patients had evidence of scar-related VT and three patients had a bundle-branch re-entry VT. The most common sites for scar-related VT were the border-zones of the apical aneurysms, basal septum, and LV lateral wall. Patient either underwent an endocardial ablation or a combined endocardial and epicardial ablation. Acute success was achieved in all patients. In 6 patients VT was terminated during ablation. In two patients, non-clinical VTs were inducible at the end of the procedure. No major or minor complications were observed during and after the procedure in all patients. During long-term follow up, elimination of VTs reached 78%. ConclusionCatheter ablation of VT in patients with HCM is safe and successful in eliminating VT. Combining endocardial and epicardial ablation techniques can potentially lead to better outcomes in these patients. Bundle branch re-entry should be considered as a potential mechanism of VT in patients with HCM.

Abstract 11768: Regression of Inflammation by Cardiac Magnetic Resonance in Chronic Chagasic Patients Treated With Benznidazole

Introduction: Chagas disease (CD) is a neglected disease caused by Trypanosoma cruzi ( T. cruzi ), and benznidazole (BZN) is the most prescribed trypanocidal drug in Brazil in function of its best tolerability profile. While treatment with this drug is effective in acute disease, there is no consensus on its effectiveness in adults with chronic infection. Although it is now accepted that treatment directed against T. cruzi is necessary to avoid the long-term sequelae of the disease, the lack of reliable markers for assessment of treatment efficacy is a major obstacle to evaluating new drugs. Hypothesis: the objective of this study is to determine if BZN treatment reduces cardiac inflammation/fibrosis as assessed by magnetic resonance imaging (MRI), using parametric mapping techniques. Methods: Prospective, multi-center study of chronic CD patients underwent MRI T1 and T2 mapping and extracellular volume measurement (ECV) using the modified Look-Locker inversion recovery (MOLLI) sequence, undergoing treatment with BZN, at a dose of 300 mg/kg daily divided into two doses and taken for 60 days. Absolute change T1 and T2 mapping, ECV, left ventricular end-diastolic volume (LVEDV), left ventricular end-systolic volume (LVESV) and left ventricle ejection fraction (LVEF) were analyzed from baseline to 6-month follow-up. Results: 53 patients (females 83%, 53.9 ± 11.9 years old) were studied. LVEDV (149 ± 27ml vs 147 ± 29ml, p = 0.52), LVESV (66 ± 29 vs 64 ± 15, p = 0.65) and LVEF (55 ± 11% vs 57 ± 8%, p = 0,28) did not change between baseline and post treatment. Native T1 time showed reduced values among anterior (1030 ± 41ms vs 1011 ± 52ms, p = 0.011), septal (1052 ± 53ms vs 1006 ± 38ms, p = 0.000) and lateral (1042 ± 55ms vs 1019 ± 44ms, p = 0.003) LV walls. T2 mapping showed reduction in LV septal wall (53 ± 4ms vs 52 ± 3ms, p = 0.005). ECV showed significant reduction from baseline to 6-month follow-up (29.9 ± 3,3% vs 28.2 ± 3.5%, p = 0.0001). Conclusions: For the first time, we observe favorable changes in CMR ECV, T1 and T2 mapping induced by benznidazole therapy in chronic Chagas disease patients. These findings suggest that there may be an opportunity to decrease fibrosis/inflammation in chronic CD patients with the use of this therapeutic approach.

Adjustment of acquisition arc in cardiac malposition during myocardial perfusion SPECT imaging: computer simulation based on deterministic modeling

ObjectivesTo simulate cardiac malpositions, leftward and rightward shift and dextrocardia, and also to compare distribution of activity of septal and lateral walls of left ventricle acquired in standard acquisition arc and after relevant adjustment. MethodsIn this study, digital phantoms with cardiac malpositions are designed and procedure of acquisition of scan in standard arc (from right anterior oblique to left posterior oblique) and adjusted acquisition arc is simulated. The three situations of malposition including leftward and rightward shift and dextrocardia are considered. For all types, acquisition is conducted in standard and then adjusted arcs (from anterior to posterior and also from right to left for leftward and rightward shifts, respectively, and for dextrocardia, from left anterior oblique to right posterior oblique). All obtained projections are reconstructed using the algorithm of filtered back projection. During forward projection to obtain sinograms, radiation attenuation is also modeled by incorporation of a simplified transmission map to emission map. The resulting tomographic slices of the LV (septum, apex, and lateral wall) are presented visually and are compared by plotting intensity profiles of the walls. Finally, normalized error images are also computed. All the computations are performed in MATLAB software package. ResultsIn transverse slice, septum and lateral wall are attenuated progressively from apex, which is closer to the camera, to the base in similar fashion. In tomographic slices of standard acquisition arc, the septum shows remarkably higher activity compared to lateral wall. However, after adjustment, both seems equally intense and progressively being attenuated from apex to base, similar to that found in phantom with normally positioned heart. Likewise, for the phantom with rightward shift, when the scanning was done in standard arc, the septum is more intense than the lateral wall. And similarly, adjustment of the arc renders both walls equally intense. In dextrocardia, level of attenuation of basal parts of septum and lateral wall is higher in 360° arc compared to adjusted 180° arc. ConclusionAdjustment of acquisition arc exerts perceptible changes in distribution of activity over LV walls which are more compatible with normally positioned heart.

Role of radionuclide assessment of global and regional mechanical dyssynchrony of the heart in prognosis of cardiac resynchronization therapy in patients with heart failure

Aim. To determine the prognostic significance of cardiac regional mechanical dyssynchrony (MD), assessed by radionuclide equilibrium ventriculography (REVG) in candidates for cardiac resynchronization therapy (CRT).Material and methods. The study included 65 patients with indications for CRT according to current guidelines. Prior to CRT, all patients underwent REVG to assess cardiac contractile function and MD. According to the phase analysis, indicators of global and regional cardiac MD were evaluated: phase standard deviation (PSD), histogram band width (HBW), entropy, and interventricular dyssynchrony. The regional assessment included an evaluation of phase histograms obtained from the analysis of the contraction of certain walls: left ventricular (LV) anterior, lateral, posterior wall, right ventricular (RV) free wall, and the interventricular septum. To evaluate the effectiveness of treatment 6 months after CRT, all patients underwent echocardiography, on the basis of which patients were divided into groups of responders and non-responders.Results. REVG revealed significant differences in the initial regional MD values between the groups of responders and non-responders: in responders, MD values of RV free wall (PSD: 39 (28-67) vs 28 (20-50), p=0,03) and LV anterior wall (PSD: 28,5 (16-40) vs 14 (11-24), p=0,0005) were higher, and the LV lateral wall was lower (PSD: 10 (7-14) vs 15 (9-26), p=0,007) than in non-responders. Multivariate logistic regression found following independent predictors of a positive response to CRT: heart failure of ischemic origin, LV HBW, RV free wall PSD, anterior wall PSD, LV lateral wall HBW (p<0,001). The sensitivity and specificity of the model was 93% and 91%, respectively.Conclusion. Regional MD scintigraphy parameters increase the predictive value of REVG in CRT candidates. The most informative in this regard are the PSD of RV free wall and LV anterior wall, as well as the HBW of LV lateral wall.

Survivors of Pediatric Hematopoietic Stem Cell Transplantation Exhibit Progressive Diastolic Dysfunction Over Years of Follow-Up

Patients who have undergone hematopoietic stem cell transplantation (HSCT) in childhood have a higher risk of diastolic heart failure (HF). The rate of progression of diastolic dysfunction in aging pediatric patients is unknown and is more difficult to assess in young patients secondary to changes in diastolic indices as they grow. HSCT recipients at our center were previously found to have decline in diastolic function indices at 1 year after HSCT. This study provides follow-up of this cohort, using age-normalized z-scores to assess whether the decline in diastolic function noted at 1-year post-HSCT persists, worsens, or improves over time. Patients age <21 years who underwent HSCT at Boston Children's Hospital/Dana-Farber Cancer Center between 2005 and 2008 with ≥3 surveillance echocardiograms, including 1 performed pre-HSCT, were included. Diastolic measures included mitral inflow (E/A ratio) and Doppler tissue imaging of left ventricular lateral wall (LV lateral e'), LV septal wall (septal e') and right ventricular free wall (RV e'). Systolic function was measured by LV ejection fraction (LVEF). Normalization by age was done using z-scores, and >±2 SD was defined as abnormal in linear modeling of diastolic dysfunction and systolic dysfunction over time. In a subset of patients with adequate post-HSCT images of the entire left atrium (LA), LA volume and LA strain analyses also were performed. The study cohort comprised 61 patients (41% female; median age at HSCT, 10.7 years; median follow-up, 7.4 years). Diastolic index z-scores declined by -.045/year for LV lateral e', -.06/year for LV septal e', and -.14/year for RV e' (P < .01). The E/A ratio z-score increased by .034/year (P=.028). Linear modeling demonstrated that LV lateral e' and LV septal e' would become abnormal at 25 and 20 years post-HSCT, respectively, whereas RV e' would become abnormal sooner, at 12.6 years. LVEF z-score declined by -.04/year (P < .01) and was estimated to become abnormal at 40 years post-HSCT. Exposure to total body irradiation (TBI) was associated with worsening diastolic indices, lower LVEF (P ≤ .002), and decreased LA reservoir strain (42.0% versus 45.0%; P=.016) and conduit strain (-31.5% versus -35.1%; P=.029), although there was significant overlap between TBI and anthracycline exposure. Treatment with anthracyclines even at low doses (median, 150 mg/m2) was associated with declining LVEF but not with changes in diastolic indices. Long-term survivors of childhood HSCT exhibit declines in both LV and RV diastolic function indices. These results inform the rate of progression of LV and RV diastolic dysfunction indices over time in long-term survivors of pediatric HSCT. A significant association was observed between TBI and diastolic dysfunction and a decline in LVEF. Treatment with anthracyclines even at low doses was associated with a mild decline in LVEF. Our results can inform a lifespan perspective on disease management in this population, encourage clinicians and patients to be vigilant in following guideline-directed surveillance echocardiography, and inform anticipatory responses by clinicians as patients transition from pediatric care to adult care.

Left ventricular noncompaction in Ibadan, Nigeria

BackgroundThere has been an increase in the reporting of cases of left ventricular noncompaction (LVNC) cardiomyopathy in medical literature due to advances in medical imaging. Patients with LVNC may be asymptomatic or may present with arrhythmias, heart failure, thromboembolism or sudden death. LVNC is typically diagnosed by echocardiography, although there are higher-resolution cardiac imaging techniques such as cardiac magnetic resonance imaging (MRI) to make the diagnosis. The objective of the study is to report on a series of 9 cases of LVNC cardiomyopathy seen at the University College Hospital, Ibadan. Cases of LVNC seen between September 1, 2015 and July 31, 2022 in our echocardiography service is being reported.ResultsThere were a total of 6 men and 3 women. Mean age at presentation was 52.89 ± 15.02 years. The most common mode of presentation was heart failure (6 patients). Hypertension was the most common comorbidity (6 patients). Three patients had an ejection fraction of less than 40% and the mean ratio of noncompacted to compacted segment at end-systole was 2.80 ± 0.48. The most common areas of trabecular localization were the LV lateral wall and the apex. Beta blockers were highly useful in the management of the patients.ConclusionsLVNC cardiomyopathy is not uncommon in our environment and a high index of suspicion is often required.

Left bundle branch area pacing reduces epicardial dispersion of repolarization compared with biventricular cardiac resynchronization therapy

Biventricular endocardial pacing (BiV-endo) and left bundle branch area pacing (LBBAP) are novel methods of delivering cardiac resynchronization therapy. These techniques are associated with improved activation times and acute hemodynamic response compared with conventional biventricular epicardial pacing (BiV-epi); however, the effects on repolarization and arrhythmic risk are unknown. The purpose of this study was to compare the effects of temporary BiV-epi, BiV-endo, and LBBAP on epicardial left ventricular (LV) repolarization using electrocardiographic imaging (ECGi). Eleven patients indicated for cardiac resynchronization therapy underwent a temporary pacing protocol with ECGi. BiV-endo was delivered via endocardial stimulation of the LV lateral wall. LBBAP was delivered by pacing the LV septum. Epicardial LV repolarization time (LVRT-95; time taken for 95% of the LV to repolarize), LV RT dispersion, mean LV activation recovery interval (ARI), LV ARI dispersion, and RT gradients were calculated. The protocol was completed in 10 patients. During LBBAP, there were significant reductions in LVRT-95 (94.9 ± 17.4 ms vs 125.0 ± 29.4 ms; P=.03) and LV RT dispersion (29.4 ± 6.3 ms vs 40.8 ± 11.4 ms; P = .015) compared with BiV-epi. In contrast, there were no significant differences between baseline, BiV-epi, or BiV-endo. There was a nonsignificant reduction in mean RT gradients between LBBAP and baseline rhythm (0.74 ± 0.22 ms/mm vs 1.01 ± 0.31 ms/mm; P = .07). There were no significant differences in mean LV ARI or LV ARI dispersion between groups. Temporary LBBAP reduces epicardial dispersion of repolarization compared with conventional BiV-epi. Further study is required to determine whether these repolarization changes on ECGi translate into a reduced risk of ventricular arrhythmia in clinical practice.

A Clinical Case of Surgical Treatment of a Patient with Two Postinfarction Left Ventricular Aneurysms

Postinfarction left ventricular (LV) aneurysm develops as a result of untimely restoration of blood flow in the infarct-dependent artery and is associated with an increase in cardiac mortality. The presence of several postinfarction LV aneurysms of different localizations in one patient is a rare and casuistic case.&#x0D; Case description. Patient B., 53 years old, was urgently hospitalized in the National Amosov Institute with complaints of shortness of breath, pain behind the sternum with radiation to the left arm, occurring with minimal physical exertion. Electrocardiography showed scar changes in the anterior-apical-lateral area of the LV with a thrombosed aneurysm of the LV lateral wall. Echocardiography revealed a severe decrease in the global contractility of the LV with ejection fraction of 24% and aneurysms of the septal-apical and posterior-lateral segments of LV. Pericarditis was found, with a layer of fluid of 1.7 cm on the LV posterior wall and 2.0 cm on the LV apex. Emergency coronary angiography revealed an occlusion of the middle third of the left anterior descending artery and the middle third of the left circumflex coronary artery, 50-70% stenosis of the right coronary artery and confirmed widespread LV aneurysm. Cardiac magnetic resonance imaging with intravenous contrast confirmed the change in configuration and significant dilatation of the LV due to the formation of two LV aneurysms with mural thrombus in the cavity of the posterior-lateral LV aneurysm. After discussing the patient’s data, the cardiac team performed emergency on-pump coronary bypass surgery, resection of the LV aneurysm with thrombectomy. The operation took 5 hours, aorta cross-clamp time was 75 min, perfusion time was 117 min, intraoperative blood loss was 300 ml. The patient was extubated in 2 hours after the end of the operation, the length of intensive care unit stay was 2 days. On the 9th day after the operation, the patient was discharged from the Institute with positive clinical, electrocardiographic and echocardiographic (LV ejection fraction 38%) dynamics.&#x0D; Conclusions. This clinical case demonstrates successful surgical treatment of two LV aneurysms, a rare complication of acute myocardial infarction. Preoperative detailed diagnosis and discussion of the patient’s data by the heart team, selection of the optimal term and volume of the operation allowed to quickly stabilize the patient’s condition and achieve a positive immediate result.

Myocarditis during SARS-CoV-2 Infection: A Series of 15 Cases

COVID-19 is a global health problem responsible for significant morbidity and mortality and a major socioeconomic impact. While the major manifestation is respirator with a risk of acute respiratory distress syndrome, several extrapulmonary aspects, particularly cardiovascular, have emerged over time.&#x0D; The aim of this work is to study the clinical, biological, radiological, therapeutic, and evolutionary characteristics of acute myocarditis associated with SARS-CoV-2 infection in comparison to data from the literature.&#x0D; This is a prospective cohort study conducted at the Ibn Sina University Hospital in Rabat over a period of 9 months (from January 1, 2021, to September 30, 2021) and included patients hospitalized in different departments managing COVID-19 and diagnosed with acute myocarditis confirmed by cardiac MRI.&#x0D; Fifteen patients were included during the study period. The average age of patients was 40 years old with a slight male predominance (sex ratio M/F-1.14). The average time between the onset of COVID-19 infection and the appearance of myocarditis symptoms was 17 days. The symptoms were mainly dominated by chest pain (40% of cases), unexplained cardiogenic shock (40% of cases), and palpitations (20% of cases). ECG revealed diffuse repolarization abnormalities in more than half of the cases (53%) and supraventricular tachycardia (27%). The inflammatory markers were significantly disturbed (CRP = 102 +/-47.30 mg/L and ferritin - 510 +/-336.45 ng/mL). Troponin I was elevated with myocarditis kinetics in all patients. Cardiac MRI showed T2 hypersignal corresponding to edema with early and late subepicardial enhancement, mainly located at the level of the left ventricular lateral wall.&#x0D; The evolution was marked by the occurrence of death in 5 patients (33.33%) in the days following hospitalization for cardiogenic shock and dilated cardiomyopathy in only one case (6.67%). It was favourable for the rest of the patients (60%).&#x0D; The results of this study have highlighted several characteristics of myocarditis associated with SARS-CoV-2. Firstly, there is a delayed interval between virus infection and the onset of cardiac symptoms. Additionally, the kinetics of myocardial injury markers are distinct, and there is significant disruption of inflammatory markers at the time of diagnosis. Moreover, fulminant forms of myocarditis are associated with a reserved prognosis and high mortality.&#x0D; Furthermore, the study emphasizes the crucial importance of cardiac MRI availability for confirming the diagnosis in the absence of histological evidence from endomyocardial biopsy.

Prevalence and prognostic value of ventricular conduction delay in heart failure with preserved ejection fraction

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Dutch Heart Foundation; Health Foundation Limburg Background The pathophysiology of heart failure (HF) with preserved ejection fraction (HFpEF) is heterogeneous and incompletely understood. Identifying phenotypes of HFpEF is a promising approach to determine the underlying pathophysiological mechanisms and to improve treatment. Patients with electrical ventricular conduction delay (VCD) may represent a separate phenotype. Objectives To identify a VCD phenotype in HFpEF through assessment of QRS duration and vectorcardiographic QRS area. Moreover, to determine how differences in these VCD indices relate to adverse outcomes. Methods Consecutive patients underwent a comprehensive diagnostic work-up at baseline and were diagnosed with HFpEF. Baseline QRS duration was obtained clinically from a standard 12-leads electrocardiography (ECG). QRS area was derived from vectorcardiography analyses of the baseline ECG. Presence of VCD was categorically defined as QRS duration ≥120ms or QRS area ≥69µVs. Adverse outcome comprised a composite of HF hospitalization and cardiovascular mortality. Patients with cardiac devices before and up until one year after baseline were excluded in prognostic analyses. Results From a total of 346 HFpEF patients included in this study, 71.3% had a QRS duration &amp;lt;100ms compared to 20.1% with QRS duration 100-119ms and 8.6% with QRS duration ≥120ms. Only 3.8% of all patients had a QRS area ≥69µVs, indicating delayed left ventricular lateral wall activation. A history of coronary artery disease was more prevalent in patients with higher QRS duration or larger QRS area. Sex differences were mainly found in those with a QRS duration ≥100ms, which was present in 54.8% of all males and 17.5% of all females. After a median of 2 years follow-up, 16.4% of the patients had an adverse outcome. Longer QRS duration but not larger QRS area was associated with more adverse outcomes on a categorical scale (Figure). In multivariable Cox-regression analyses adjusted for sex and age, QRS duration was an independent predictor of adverse outcomes (hazard ratio per 1ms increase = 1.017, 95% confidence interval 1.004-1.030, P = 0.012) (Table). Conclusion HFpEF patients have a low prevalence of a VCD phenotype, 8.6% have a QRS duration ≥120ms and 3.8% a QRS area ≥69µVs. However, QRS duration &amp;gt;100ms was an independent predictor for adverse outcomes. Thus, future efforts are warranted to understand the biologic and mechanical mechanisms underlying the association of QRS duration and adverse outcomes, and to determine its clinical implications.

P324 LEFT VENTRICULAR ISOLATED CARDIOMYOPATHY OR DILATED CARDIOMYOPATHY: THE ROLE OF GENETIC ANALYSIS

Abstract Introduction arrhytmogenic cardiomyopathy (ACM) is a genetic heart muscle disease caracterized by substitution of the ventricular myocardium with fibrofatty tissue. The disease was originally termed “arrhytmogenic right ventricular (dysplasia)/cardiomyopathy” to define a condition which distinctively affected the right ventricle and predisposed to fatal ventricular arrhythmias. New insights led to comprehending that the left ventricle is also frequently affected. Clinical case 62 yo, male, no cardiovascular risk factors, positive history for frequent ventricular premature beats treated with Sotalol 40 mg twice a day. No other previous cardiological events. Accesses the ER with suddenly occured dyspnea for minimal exertion, without palpitation, chest discomfort or loss of consciousness. EKG: synus rhythm, low voltages, negative V4–V6 T waves, fragmented QRS. Blood testing: TnI 0,532 ng/ml (n.v. 0–0.034) NTproBNP 3840. Echocardiography: dilated left ventricle. 20% EF due to infero–lateral wall akynesia. Coronary angiography was performed and showed no coronary stenosis. Considered the frequent NSVTs showed by the telemetry, he was admitted to ICU and treated with Amiodarone, Atenolol 100 mg, Sacubistril/Valsartan 24/26 mg, diuretic therapy showing good control of major arrhytmic events. To evaluate characterize heart tissue, a magnetic resonance was performed, which showed: scalloping of the mid and apical left ventricular lateral wall, with fibrofatty substitution. Subepicardial, mid–wall and transmural LGE of the LV anterior, lateral and inferior walls. No RV abnormalities. Considered the reduced EF and the presence of arrhytmic substratum, an ICD was implanted. Finally, a genetic evaluation was discussed with the patient, but was declined. Discussion in patients presenting with typical LV abnormalities, without RV involvement, the phenotipic Padua criterias are not enough for a certain diagnosis. This is because of the phenotipic overlap between ACM and other diseases, such as dilated cardiomyopathy, myocarditis and cardiac sarcoidosis. In the presence of LV involvement, consistent with ACM, the evidence of a pathogenetic mutation is key for the diagnosis.

C60 MYOCARDIAL T1 MAPPING IN ANDERSON–FABRY DISEASE AND ITS CORRELATION WITH GENETIC ANOMALITIES

Abstract Objective Cardiac involvement in Anderson–Fabry Disease is possible even in mild forms, and new analytical methods and the quantitative value evident with the T1 Native sequence for Mapping study demonstrated a linear correlation with disease severity compared with traditional MR sequences. GLA gene alterations on the X chromosome are very heterogeneous and correlated with decreased or absent alpha–galactosidase enzyme, resulting in different timing of manifestation and clinical evolution. In addition to the classic form of central nervous system, kidney and heart involvement there is a variant with only cardiac involvement, also found in our study. Materials and Methods We retrospectively analyzed 18 consecutive Cardiac MRI examinations performed in patients with established Anderson–Fabry disease at the University Radiology Department of Policlinico Riuniti di Foggia from August 2019 to January 2022. Native T1 sequences for T1 Mapping study are currently the gold standard for evaluation of cardiac involvement in Anderson–Fabry disease. Results T1 Mapping values were lower with characteristic normality of ECV after mdc. In PSIR sequences acquired after mdc for the study of "Late Gadolinium Enhancement" usually a mesocardial signal hyperintensity of the infero–lateral wall of the left ventricle characterized by fibrotic deposits is evident but this sign usually occurs when the heart is already hypertrophied. Instead, reduced Native T1 values serve as an early marker of myocardial involvement, even before the development of hypertrophy as also found in our study population. In fact in 10 out of 18 patients the only value indicating myocardial involvement was reduced T1 Mapping Native values below 1000 in at least 3 out of 17 segments. In 6 cases there was concentric left ventricular wall hypertrophy and in 2 cases mesocardial hyperintensity was evident in the PSIR sequences for the "Late Gadolinium Enhancement" study of the basal segments of the infero–lateral left ventricular wall. Conclusions Magnetic resonance imaging plays a key role in the study of cardiac involvement in Anderson–Fabry disease with potential to predict the extent of detectable accumulation at the cardiac level based on the genetic abnormality.

PO-04-015 BIVENTRICULAR ARRHYTHMOGENIC CARDIOMYOPATHY ASSOCIATED WITH A NOVEL HETEROZYGOUS PLAKOPHILIN-2 EARLY TRUNCATING VARIANT

N/A N/A N/A A 31-year-old male patient suffered from palpitations, which occurred particularly during and shortly after physical activity. The patient was very athletic and took part in marathons. His family history was unremarkable. The electrocardiogram showed T wave inversions in V1-V3 (Figure 1A). During bicycle ergometry monomorphic premature ventricular complexes (PVC) and non-sustained ventricular tachycardia episodes (left-bundle branch block morphology, inferior axis) evolved (Figure 1B, black arrow). Of note, under maximal physical stress a second PVC with a right-bundle branch block morphology occurred (Figure 1B, red arrow) suggesting left ventricular (LV) involvement. Echocardiographic evaluation showed a dilated right ventricle (RV) with reduced systolic function, a subtricuspid aneurysm and multiple sacculations of the RV free wall (Figure 1C-D, white markers). Cardiac magnetic resonance imaging (MRI) confirmed the right ventricular findings (Figure 1E, red arrows). In addition, late gadolinium enhancement and fatty deposits were visible in the LV lateral wall (Figure 1F-G, yellow arrows and blue asterix). Genetic testing was performed using a panel covering 173 genes (Agilent, SureSelectQXTTarget Enrichment) and next generation sequencing (NGS) (Illumina MiSeq). It revealed a novel heterozygous nonsense variant in the plakophilin-2 (PKP2) gene (808C>T p. (Gln270Ter) in exon 3) that causes an early truncation, which was confirmed by Sanger sequencing. It was classified as pathogenic (class V). Cascade screening of the asymptomatic patient’s mother (64-y-old), father (70-y-old) and sister (33-y-old) showed that the patient's father and sister harbored the same PKP-2 variant. Both did not report a history of participating in competitive sports, and thorough cardiac evaluation of these two genotype positive family members showed normal findings. Previous studies have indicated that early truncation of the PKP2 C-terminus likely causes arrhythmogenic right ventricular cardiomyopathy (ARVC) irrespective of transcript position. Although desmosomal and non-desmosomal variants create certain genetic potential for the development of ARVC, exercise has an important role in determining the development, severity, and pattern of phenotypic expression. The fact that the only index patient, as a marathon runner, developed ARVC, suggests that the novel PKP2 variant poses a genetic risk, but is not sufficient for inducing an ARVC phenotype by itself.

PO-01-110 ATRIAL TORSADES DE POINTES POST COVID MYOCARDITIS

Among patients with COVID-19 infection, the risk of adverse cardiovascular outcome, particularly myocarditis and dysrhythmias remain elevated at least up to one year after infection. We present a case of atrial tachycardia and atrial Torsades de Pointes from COVID myocarditis, persisted 6 months after infection, which was successfully managed by ablation. A 25-year-old female presented with mild COVID-19 infection, Omicron variant, in May 2022. One month after, her Covid infection resolved; she presented with symptomatic atrial tachycardia, paroxysmal atrial fibrillation and flutter. ECG showed multiple blocked premature atrial contractions (PAC) (Figure 1A). Holter monitor showed PAC triggered atrial tachycardia degenerating to paroxysmal atrial fibrillation, atrial Torsades de Pointes. She has mild persistent troponin elevation. Echocardiography was normal. Cardiac MRI showed evidence of mild myocarditis with subepicardial late Gadolinium enhancement (LEG) along the lateral mid-apical left ventricular wall and edema. (Figure 1B). She was treated with Colchicine for 2 months. Repeat cardiac MRI 4 months after COVID infection showed resolution of edema and LGE. However, her symptomatic PAC and atrial tachycardia did not respond to betablocker and amiodarone. She underwent electrophysiology study. Activation mapping of PAC using CARTO revealed earliest activation at the right anterior atrial wall, with close proximity to tricuspid valve; unipolar signal showed QS pattern, bipolar signal showed 16 msec pre-PAC (Figure 1C and 1D). Mechanical pressure from ThermoCool SmartTouch ablation catheter (Biosense Webster Inc.) at this site suppressed the PAC. Radiofrequency ablation resulted with an initial acceleration and then disappearance of the PAC. We did not isolate pulmonary veins or ablate cavotricuspid isthmus. Post ablation, PAC and atrial fibrillation were not inducible on Isoproterenol. N/A Covid myocarditis can result in dysrhythmia that lingers long after Covid myocarditis has resolved. Covid myocarditis can be caused by direct viral invasion of myocytes or more commonly is inflammatory related to cytokine release and edema. Our case demonstrates that dysrhythmias can persist despite resolution of myocarditis. Catheter ablation can successfully to treat these arrhythmias. This case highlights the importance of recognizing cardiac dysrhythmia as possible the long-term cardiac complications of COVID-19, requiring specific treatment such as catheter ablation.

Single-Photon Emission Computed Tomography Assessment of Sepsis-Related Myocardial Calcification.

A 63-year-old man was admitted to the emergency department with tachypnoea, abdominal pain, hypotension, and altered mental status. Upon examination, the patient had involuntary guarding and localised rebound tenderness in the right lower quadrant, suggestive of peritonitis. The patient had no history of arterial hypertension, diabetes mellitus, or ischaemic heart disease. Arterial blood gas showed elevated lactate (4.1 mmol/L), and laboratory tests were notable for leucocytosis (14.7 × 103/mm3), elevated C-reactive protein (31.4 mg/L), and elevated procalcitonin (1.7 ng/mL), with normal calcium (8.9 mg/dL), phosphorus (4.1 mg/dL), and creatinine (1.27 mg/dL). Abdominal computed tomography (CT) revealed induration of the omentum of the right lower quadrant with bubbles of free air in the subumbilical region, suggesting a ruptured Meckel’s diverticulum. The patient received initial fluid resuscitation, antibiotics (vancomycin and piperacillin–tazobactam), and analgesia. Norepinephrine and vasopressin were initiated as the patient’s blood pressure did not increase with initial fluid resuscitation. The patient underwent an exploratory laparotomy and was hospitalised for sepsis. On the first day of hospitalisation, the patient had elevated troponin I (11.2 ng/mL; laboratory reference range ≤0.04 ng/mL) and N-terminal probrain natriuretic peptide (2,386 pg/mL), with repeated 12-lead electrocardiography showing normal sinus rhythm without ST segmental change throughout hospitalisation. He denied chest pain, tightness, palpitations, or dyspnoea, and his cardiac function remained stable. Three sets of blood cultures with one anaerobic bottle and one aerobic bottle each were collected before initiating empiric antibiotics, with two of the anaerobic bottles growing Escherichia coli 48 hours after incubation. As the patient’s troponin I normalised 4 days later (0.02 ng/mL), without ST segmental changes or wall abnormalities on point-of-care ultrasonography, coronary angiography was not performed. Upon recovery, the patient was referred to our department for further evaluation to clarify the aetiology of cardiac injury. Single-photon emission computed tomography (CT) revealed perfusion defects in the inferior and inferolateral walls, reverse redistribution, and transient dilatation (Figure 1A). The coronary artery calcium score was calculated as low risk, but a hyperdense linear image at the level of the left ventricular (LV) lateral wall was observed (Figure 1B). Cardiac CT angiography (CCTA) was performed, in which Coronary Artery Disease Reporting and Data System 1 was observed (Figure 1C), with LV lateral wall calcification in axial slices (Figure 1D). Three-dimensional reconstruction showed a partial absence of thickness of the LV lateral wall in relation to the calcified tissue (Figure 1E).

Modern approaches to the diagnosis and treatment of cardiac sarcoidosis: results of a cohort study

Aim. To analyze clinical and paraclinical data in patients with documented cardiac sarcoidosis, outlining the key points of diagnosis and selection of the optimal treatment.Material and methods. For the period from 2016 to 2021, 63 patients (50,4±14,1 years) were included in the cohort study on negotiability. Based on a standard examination, 15 patients (41±13 years old) were selected, who continued the examination to confirm the diagnosis of cardiac sarcoidosis. Contrast-enhanced cardiac magnetic resonance imaging (MRI) was performed in 10 patients, while endomyocardial biopsy in 7 patients. All patients underwent 18F-fluorodeoxyglucose positron emission tomography (PET).Results. The most common (53%) electrocardiographic abnormality was right bundle branch block. Ventricular arrhythmias and high-grade atrioventricular block were recorded mainly in patients with documented activity. Regional contractility disorders were predominantly detected in patients with cardiac fibrosis. Delayed contrast enhancement according to cardiac MRI was recorded mainly intramurally in the interventricular septum and subepicardial area of left ventricular (LV) lateral wall. When analyzing the PET results, we found the predominant radiopharmaceutical accumulation in the interventricular septum (56%), lateral (44%) and anterior (33%) LV walls. There was no significant improvement in global LV contractility against the background of immunosuppressive therapy, especially in patients with reduced ejection fraction: initially 49,2±10,1% vs 46,9±14,9% during therapy (p=0,658).Conclusion. A certain apprehensive attitude of the doctor and adherence to the algorithm for early diagnosis of cardiac sarcoidosis allows minimizing the risks of fatal cardiovascular events. On the contrary, in the case of late diagnosis, even the use of aggressive immunosuppressive therapy does not lead to an improvement in global myocardial contractility, and fibrosis zones can cause life-threatening bradyarrhythmias and ventricular arrhythmias.

Right ventricular-dominant transthyretin deposition: an early stage or a rare phenotype of wild-type transthyretin amyloid cardiomyopathy?

An 86-year-old Japanese man was referred to our hospital because of leg oedema. Electrocardiography revealed a right bundle branch block, and transthoracic echocardiography showed a left ventricular septal wall thickness of 15 mm. On cardiovascular magnetic resonance imaging (CMR), late gadolinium enhancement areas and high native T1 value areas extended in the basal region of the left ventricular lateral wall (Panels A and C), the right ventricular (RV) side of the septum, and the RV free wall (Panels B and D). Abnormal accumulation of 99mTc-labelled pyrophosphate (99mTc-PYP) in the myocardium was visualized in concordance with the abnormal areas on CMR (Panels E and F). The patient was diagnosed with wild-type transthyretin amyloid cardiomyopathy (ATTR-CM) due to the histological evidence of transthyretin deposition in the myocardium and no transthyretin gene mutation. In this case, both CMR and 99mTc-PYP scintigraphy, which are reliable imaging tools for transthyretin deposition in the myocardium, revealed RV-dominant transthyretin deposition. Based on the UK ATTR-CM staging system, this case was classified as Stage 1. Since no cases of RV-dominant transthyretin deposition have been reported in the literature, it is unclear whether RV-dominant transthyretin deposition is an early stage or a very rare clinical phenotype of wild-type ATTR-CM. Nonetheless, clinicians should be aware of an RV-dominant transthyretin deposition pattern in wild-type ATTR-CM.

Effect of scar and His-Purkinje and myocardium conduction on response to conduction system pacing.

Conduction system pacing (CSP), in the form of His bundle pacing (HBP) or left bundle branch pacing (LBBP), is emerging as a valuable cardiac resynchronization therapy (CRT) delivery method. However, patient selection and therapy personalization for CSP delivery remain poorly characterized. We aim to compare pacing-induced electrical synchrony during CRT, HBP, LBBP, HBP with left ventricular (LV) epicardial lead (His-optimizedCRT [HOT-CRT]), and LBBP with LV epicardial lead (LBBP-optimized CRT [LOT-CRT]) in patients with different conduction disease presentations using computational modeling. We simulated ventricular activation on 24four-chamber heart geometries, including His-Purkinje systems with proximal left bundle branch block (LBBB). We simulated septal scar, LV lateral wall scar, and mild and severe myocardium and LV His-Purkinje system conduction disease by decreasing the conduction velocity (CV) down to 70% and 35% of the healthy CV. Electrical synchrony was measured by the shortest interval to activate 90% of the ventricles (90% of biventricular activation time [BIVAT-90]). Severe LV His-Purkinje conduction disease favored CRT (BIVAT-90: HBP 101.5 ± 7.8 ms vs.CRT 93.0 ± 8.9 ms, p < .05), with additional electrical synchrony induced by HOT-CRT (87.6 ± 6.7 ms, p < .05) and LOT-CRT (73.9 ± 7.6 ms, p < .05). Patients with slow myocardium CV benefit more from CSP compared to CRT (BIVAT-90: CRT 134.5 ± 24.1 ms; HBP 97.1 ± 9.9 ms, p < .01; LBBP: 101.5 ± 10.7 ms, p < .01). Septal but not lateral wall scar made CSP ineffective, while CRT was able to resynchronize the ventricles in the presence of septal scar (BIVAT-90: baseline 119.1 ± 10.8 ms vs.CRT 85.1 ± 14.9 ms, p < .01). Severe LV His-Purkinje conduction disease attenuates the benefits of CSP, with additional improvements achieved with HOT-CRT and LOT-CRT. Septal but not lateral wall scars make CSP ineffective.