Effect of Migalastat on cArdiac InvOlvement in FabRry DiseAse: MAIORA study.

BACKGROUND: A small but significant reduction in left ventricular (LV) mass after 18 months of migalastat treatment has been reported in Fabry disease (FD). This study aimed to assess the effect of migalastat on FD cardiac involvement, combining LV morphology and tissue characterisation by cardiac magnetic resonance (CMR) with cardiopulmonary exercise testing (CPET). METHODS: Sixteen treatment-naïve patients with FD (4 women, 46.4±16.2 years) with cardiac involvement (reduced T1 values on CMR and/or LV hypertrophy) underwent ECG, echocardiogram, troponin T and NT-proBNP (N-Terminal prohormone of Brain Natriuretic Peptide) assay, CMR with T1 mapping, and CPET before and after 18 months of migalastat. RESULTS: No change in LV mass was detected at 18 months compared to baseline (95.2 g/m2 (66.0-184.0) vs 99.0 g/m2 (69.0-121.0), p=0.55). Overall, there was an increase in septal T1 of borderline significance (870.0 ms (848-882) vs 860.0 ms (833.0-875.0), p=0.056). Functional capacity showed an increase in oxygen consumption (VO2) at anaerobic threshold (15.50 mL/kg/min (13.70-21.50) vs 14.50 mL/kg/min (11.70-18.95), p=0.02), and a trend towards an increase in percent predicted peak VO2 (72.0 (63.0-80.0) vs 69.0 (53.0-77.0), p=0.056) was observed. The subset of patients who showed an increase in T1 value and a reduction in LV mass (n=7, 1 female, age 40.5 (28.6-76.0)) was younger and at an earlier disease stage compared to the others, and also exhibited greater improvement in exercise tolerance. CONCLUSION: In treatment-naïve FD patients with cardiac involvement, 18-month treatment with migalastat stabilised LV mass and was associated with a trend towards an improvement in exercise tolerance. A tendency to T1 increase was detected by CMR. The subset of patients who had significant benefits from the treatment showed an earlier cardiac disease compared to the others. TRIAL REGISTRATION NUMBER: NCT03838237.

AI Cardiac MRI Scar Analysis Aids Prediction of Major Arrhythmic Events in the Multicenter DERIVATE Registry.

Background Scar burden with late gadolinium enhancement (LGE) cardiac MRI (CMR) predicts arrhythmic events in patients with postinfarction in single-center studies. However, LGE analysis requires experienced human observers, is time consuming, and introduces variability. Purpose To test whether postinfarct scar with LGE CMR can be quantified fully automatically by machines and to compare the ability of LGE CMR scar analyzed by humans and machines to predict arrhythmic events. Materials and Methods This study is a retrospective analysis of the multicenter, multivendor CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy (DERIVATE) registry. Patients with chronic heart failure, echocardiographic left ventricular ejection fraction (LVEF) of less than 50%, and LGE CMR were recruited (from January 2015 through December 2020). In the current study, only patients with ischemic cardiomyopathy were included. Quantification of total, dense, and nondense scars was carried out by two experienced readers or a Ternaus network, trained and tested with LGE images of 515 and 246 patients, respectively. Univariable and multivariable Cox analyses were used to assess patient and cardiac characteristics associated with a major adverse cardiac event (MACE). Area under the receiver operating characteristic curve (AUC) was used to compare model performances. Results In 761 patients (mean age, 65 years ± 11, 671 men), 83 MACEs occurred. With use of the testing group, univariable Cox-analysis found New York Heart Association class, left ventricle volume and/or function parameters (by echocardiography or CMR), guideline criterion (LVEF of ≤35% and New York Heart Association class II or III), and LGE scar analyzed by humans or the machine-learning algorithm as predictors of MACE. Machine-based dense or total scar conferred incremental value over the guideline criterion for the association with MACE (AUC: 0.68 vs 0.63, P = .02 and AUC: 0.67 vs 0.63, P = .01, respectively). Modeling with competing risks yielded for dense and total scar (AUC: 0.67 vs 0.61, P = .01 and AUC: 0.66 vs 0.61, P = .005, respectively). Conclusion In this analysis of the multicenter CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy (DERIVATE) registry, fully automatic machine learning-based late gadolinium enhancement analysis reliably quantifies myocardial scar mass and improves the current prediction model that uses guideline-based risk criteria for implantable cardioverter defibrillator implantation. ClinicalTrials.gov registration no.: NCT03352648 Published under a CC BY 4.0 license. Supplemental material is available for this article.

Bempedoic acid: what prospective uses?

There is still the need to lower LDL-c, although the use of statins, ezetimibe and proprotein convertase subtilisin/kexin type 9. Patients with atherosclerotic cardiovascular disease and/or familial hypercholesterolaemia are treated with statins at maximum tolerated dose, with or without further lipid-lowering drugs, but very often, we can't reach the goal, so bempedoic acid treatment lead to a significant reduction in low-density lipoprotein cholesterol, in different groups of patients, with a favourable safety profile.

Cardiac magnetic resonance in Fabry disease.

Fabry disease (FD) is a rare X-linked inherited lysosomal storage disorder caused by deficient a-galactosidase A activity that leads to an accumulation of glycolipids, mainly globotriaosylceramide (Gb3) and globotriaosylsphingosine, in affected tissues, including the heart. Cardiovascular involvement usually manifests as left ventricular hypertrophy (LVH), myocardial fibrosis, heart failure, and arrhythmias, which limit the quality of life and represent the most common causes of death. Following the introduction of enzyme replacement therapy, early diagnosis and treatment have become essential in slowing down the disease progression and preventing major cardiac complications. Recent advances in the understanding of FD pathophysiology suggest that in addition to Gb3 accumulation, other mechanisms contribute to the development of cardiac damage. FD cardiomyopathy is characterized by an earlier stage of glycosphingolipid accumulation and a later one of hypertrophy. Morphological and functional aspects are not specific in the echocardiographic evaluation of Anderson-Fabry disease. Cardiac magnetic resonance with tissue characterization capability is an accurate technique for the differential diagnosis of LVH. Progress in imaging techniques has improved the diagnosis and staging of FD-related cardiac disease: a decreased myocardial T1 value is specific of FD. Late gadolinium enhancement is typical of the later stage of cardiac involvement but as in other cardiomyopathy is also valuable to predict the outcome and cardiac response to therapy.

Electrocardiogram in arrhytmogenic cardiomyopathy.

Criteria for diagnosis of arrhythmogenic cardiomyopathy (ACM) were first proposed in 1994 and subsequently revised in 2010 and in 2020 by an international task force. According to the last consensus of 2020, ACM is defined as a heart muscle disease affecting right ventricle, left ventricle or both, whose principal pathologic feature is fibrofatty myocardial replacement that impairs systolic ventricular function and predisposes to lethal ventricular arrhythmias. ECG findings not only could help to early recognize affected patients but also could identify the ones with maximum risk of ventricular arrhythmias and sudden cardiac death.

The electrocardiogram in non-ischaemic-dilated cardiomyopathy.

This article summarizes the main electrocardiogram (ECG) findings in dilated cardiomyopathy (DCM) patients. Recent reports are described in the great 'pot' of DCM peculiar ECG patterns that are typical of specific forms of DCM. Patients with late gadolinium enhancement on CMR, who are at greatest arrhythmic risk, have also distinctive ECG features. Future studies in large DCM populations should evaluate the diagnostic and prognostic value of the ECG.

CarDiac magnEtic Resonance for prophylactic Implantable-cardioVerter defibrillAtor ThErapy in Non-Ischaemic dilated CardioMyopathy: an international Registry.

AIMS: The aim of this registry was to evaluate the additional prognostic value of a composite cardiac magnetic resonance (CMR)-based risk score over standard-of-care (SOC) evaluation in a large cohort of consecutive unselected non-ischaemic cardiomyopathy (NICM) patients. METHODS AND RESULTS: In the DERIVATE registry (www.clinicaltrials.gov/registration: RCT#NCT03352648), 1000 (derivation cohort) and 508 (validation cohort) NICM patients with chronic heart failure (HF) and left ventricular ejection fraction <50% were included. All-cause mortality and major adverse arrhythmic cardiac events (MAACE) were the primary and secondary endpoints, respectively. During a median follow-up of 959 days, all-cause mortality and MAACE occurred in 72 (7%) and 93 (9%) patients, respectively. Age and >3 segments with midwall fibrosis on late gadolinium enhancement (LGE) were the only independent predictors of all-cause mortality (HR: 1.036, 95% CI: 1.0117-1.056, P < 0.001 and HR: 2.077, 95% CI: 1.211-3.562, P = 0.008, respectively). For MAACE, the independent predictors were male gender, left ventricular end-diastolic volume index by CMR (CMR-LVEDVi), and >3 segments with midwall fibrosis on LGE (HR: 2.131, 95% CI: 1.231-3.690, P = 0.007; HR: 3.161, 95% CI: 1.750-5.709, P < 0.001; and HR: 1.693, 95% CI: 1.084-2.644, P = 0.021, respectively). A composite clinical and CMR-based risk score provided a net reclassification improvement of 63.7% (P < 0.001) for MAACE occurrence when added to the model based on SOC evaluation. These findings were confirmed in the validation cohort. CONCLUSION: In a large multicentre, multivendor cohort registry reflecting daily clinical practice in NICM work-up, a composite clinical and CMR-based risk score provides incremental prognostic value beyond SOC evaluation, which may have impact on the indication of implantable cardioverter-defibrillator implantation.

Appropriate use criteria for cardiovascular magnetic resonance imaging (CMR): SIC-SIRM position paper part 1 (ischemic and congenital heart diseases, cardio-oncology, cardiac masses and heart transplant).

Cardiac magnetic resonance (CMR) has emerged as new mainstream technique for the evaluation of patients with cardiac diseases, providing unique information to support clinical decision-making. This document has been developed by a joined group of experts of the Italian Society of Cardiology and Italian society of Radiology and aims to produce an updated consensus statement about the current state of technology and clinical applications of CMR. The writing committee consisted of members and experts of both societies who worked jointly to develop a more integrated approach in the field of cardiac radiology. Part 1 of the document will cover ischemic heart disease, congenital heart disease, cardio-oncology, cardiac masses and heart transplant.

Double-chambered left ventricle: An unusual finding in a middle-aged woman.

Double-chambered left ventricle (DCLV) is a rare congenital abnormality and is characterized by the subdivision of the left ventricle into two separate chambers by an abnormal septum or muscle band. This rare congenital entity is usually diagnosed in the neonatal or pediatric age. Nevertheless, it can be asymptomatic and incidentally found in the adulthood. A comprehensive multimodality imaging approach is crucial for the correct final diagnosis.

Contact-force monitoring increases accuracy of right ventricular voltage mapping avoiding "false scar" detection in patients with no evidence of structural heart disease.

PURPOSE: Electroanatomical mapping (EAM) could increase cardiac magnetic resonance imaging (CMR) sensitivity in detecting ventricular scar. Possible bias may be scar over-estimation due to inadequate tissue contact. Aim of the study is to evaluate contact-force monitoring influence during EAM, in patients with idiopathic right ventricular arrhythmias. METHODS: 20 pts (13 M; 43 ± 12 y) with idiopathic right ventricular outflow tract (RVOT) arrhythmias and no structural abnormalities were submitted to Smarttouch catheter Carto3 EAM. Native maps included points collected without considering contact-force. EAM scar was defined as area ≥1 cm2 including at least 3 adjacent points with signal amplitude (bipolar <0.5 mV, unipolar 3,5 mV), surrounded by low-voltage border zone. EAM were re-evaluated offline, removing points collected with contact force <5 g. Finally, contact force-corrected maps were compared to the native ones. RESULTS: An EAM was created for each patient (345 ± 85 points). After removing poor contact points, a mean of 149 ± 60 points was collected. The percentage of false scar, collected during contact force blinded mapping compared to total volume, was 6.0 ± 5.2% for bipolar scar and 7.1 ± 5.9% for unipolar scar, respectively. No EAM scar was present after poor contact points removal. Right ventricular areas analysis revealed a greater number of points with contact force < 5 g acquired in free wall, where reduced mean bipolar and unipolar voltage were recorded. CONCLUSIONS: To date this is the first work conducted on structurally normal hearts in which contact-force significantly increases EAM accuracy, avoiding "false scar" related to non-adequate contact between catheter and tissue.

Acute echocardiographic optimization of multiple stimulation configurations of cardiac resynchronization therapy through quadripolar left ventricular pacing: a tailored approach.

BACKGROUND: Cardiac resynchronization therapy (CRT) is ineffective in approximately 30% of recipients, in part due to sub-optimal left ventricular (LV) pacing location. The Quartet LV lead, with 2 additional electrodes proximal to conventional bipolar lead electrodes, enables 10 different pacing configurations at four independent LV locations. In a CRT patient cohort, we sought to evaluate the spectrum of echocardiographic and electrocardiographic response over these 10 configurations, to select the optimal one in each patient. Moreover, we sought to evaluate the 6-months clinical and echocardiographic response to a "tailored approach" in which the optimal LV pacing configuration for CRT was determined by echocardiographic measures, QRSd and pacing capture thresholds. METHODS: Twenty-two consecutive CRT indicated patients were implanted with a quadripolar CRT system (St. Jude Medical). Optimal LV pacing configuration was determined by echocardiographic measures, including velocity time integral (VTI), myocardial performance index (MPI) and mitral regurgitation (MR), and an electrocardiographic measure (QRS duration) during pacing from each of the configurations at pre-discharge. The optimal LV pacing vector was chosen for every patient. Clinical and echocardiographic assessment was repeated after 6 months. RESULTS: Various configurations provided different VTI, MPI, MR and QRSd values. Conventional bipolar vectors (ie, D1-M2, D1-RVc, M2-RVc) were rarely associated with the best echocardiographic improvements and provided significantly worse VTI, MR, MPI, and QRSd values than the best configuration for every patient (P = .005, P = .05 and P = .03 for VTI; P = .01, P = .005 and P = .001 for MPI; P = .003, P = .01 and P = .005 for MR, P > .5, P = .01 and P = .05 for QRSd) Conversely, "unconventional" proximal configurations (ie, making use of P4 and M3 electrodes) were generally characterized by higher acute VTI, MR and MPI improvements. CRT devices were reprogrammed with an "unconventional" LV pacing configuration in 50% of patients. A significant improvement in New York Heart Association class (81%), LV ejection fraction (76%), end-diastolic and end-systolic volumes was observed after 6 months (P = .02, P < .001, P = .02 and P = .003, respectively). CONCLUSIONS: In this study, conventional bipolar vectors of quadripolar-CRT were rarely associated with the best echocardiographic improvements. Quadripolar CRT utilizing optimal LV pacing configuration was associated with a significant improvement in New York Heart Association class and LV ejection fraction after 6 months.

Reducing agalsidase beta infusion time in Fabry patients: low incidence of antibody formation and infusion-associated reactions in an Italian multicenter study.

BACKGROUND: Fabry disease is a rare progressive X-linked lysosomal storage disease caused by mutations in the GLA gene that encodes α-galactosidase A. Agalsidase beta is a recombinant enzyme replacement therapy authorized in Europe at a standard dose of 1.0 mg/kg intravenously every other week at an initial infusion rate of ≤ 0.25 mg/min until patient tolerance is established, after which the infusion rate may be increased gradually. However, specific practical guidance regarding the progressive reduction in infusion time is lacking. This study investigated a new and specific protocol for reducing agalsidase beta infusion time in which a stable dosage of 15 mg/h is infused for the first four months, and the infusion rate is increased progressively from 15 to 35 mg/h for the subsequent four infusions. The shortest infusion time is reached after six months and maintained thereafter. The incidence of infusion-associated reactions (IARs) and the development of anti-drug antibodies were analyzed, and the disease burden and the clinical evolution of the disease at 12 months were evaluated. RESULTS: Twenty-five of the 31 patients were naïve to enzyme or chaperone treatment at baseline and six patients had been switched from agalsidase alfa. The reduced infusion time protocol was well tolerated. Only one patient exhibited an IAR, with mild symptoms that resolved with low-dose steroids. Six patients globally seroconverted during treatment (4 with a classic phenotype and 2 with late-onset disease). All but three patients were seronegative at month 12. All patients were stable at the study's end (FAbry STabilization indEX value < 20%); reducing infusion time did not negatively impact clinical outcomes in any patient. The perceived medical assessment showed that the quality of life of all patients improved. CONCLUSIONS: The study demonstrates that reducing agalsidase beta infusion time is possible and safe from both an immunogenic and clinical point of view. The use of a low infusion rate in the first months when the probability of onset of the development of antibodies is higher contributed to very limited seroconversion to antibody-positive status.

Radial versus femoral approach comparison in percutaneous coronary intervention with intraaortic balloon pump support: the RADIAL PUMP UP registry.

BACKGROUND: The role of intraaortic balloon pump (IABP) during percutaneous coronary intervention (PCI) in high-risk acute patients remains debated. Device-related complications and the more complex patient management could explain such lack of clinical benefit. We aimed to assess the impact of transradial versus transfemoral access for PCI requiring IABP support on vascular complications and clinical outcome. METHODS: We retrospectively analyzed 321 consecutive patients receiving IABP support during transfemoral (n = 209) or transradial (n = 112) PCI. Thirty-day net adverse clinical events (NACEs) (composite of postprocedural bleeding, cardiac death, myocardial infarction, target lesion revascularization, and stroke) were the primary end point, with access-related bleeding and hospital stay as secondary end points. RESULTS: Cardiogenic shock and hemodynamic instability were the most common indications for IABP support. Cumulative 30-day NACE rate was 50.2%, whereas an access site-related bleeding occurred in 14.3%. Patients undergoing transfemoral PCI had a higher unadjusted rate of NACEs when compared with the transradial group (57.4% vs 36.6%, P < .01), mainly due more access-related bleedings (18.7% vs 6.3%, P < .01). Such increased risk of NACEs was confirmed after propensity score adjustment (hazard ratio 0.57 [0.4-0.9], P = .007), whereas hospital stay appeared comparable in the 2 groups. CONCLUSIONS: In this observational registry, high-risk patients undergoing PCI and requiring IABP support appeared to have fewer NACEs if transradial access was used instead of transfemoral, mainly due to fewer access-related bleedings. Given the inherent limitations of this retrospective work, including the inability to adjust for unknown confounders, further controlled studies are warranted to confirm or refute these findings.

Pharmacoeconomic impact evaluation of statin adherence in high-risk unselected post myocardial infarction population: an administrative database-guided analysis.

BACKGROUND: The compliance to statins in secondary prevention is very low, increasing health-care costs principally for rehospitalization. OBJECTIVES: To evaluate the cost of lack of persistence to statin therapy together with identification and cost-estimation of poor compliance. METHODS: Retrospective observational study starting from administrative database analysis of statin prescription after myocardial infarction. RESULTS: Among 463 patients enrolled, 25.1% were never treated, 70.8% received statins regularly; 14.9% received only 1-2 prescriptions (spot prescription), and 12% were occasional users. Among the 288 nonoccasional users, we found a compliance rate of 80% only in the 59.7%. The cost analysis shows that 59.787,72 euros (23.4%) have been spent for patients with compliance of less than 80% (ineffective adherence). CONCLUSIONS: As the lower compliance affects the health-care costs, the identification of occasional users and spot prescriptions of the nonoccasional users, has a potential role in reducing medical expense with limited increase in costs.

The pivotal role of ECG in cardiomyopathies.

Cardiomyopathies are a heterogeneous group of pathologies characterized by structural and functional alterations of the heart. Recent technological advances in cardiovascular imaging offer an opportunity for deep phenotypic and etiological definition. Electrocardiogram (ECG) is the first-line diagnostic tool in the evaluation of both asymptomatic and symptomatic individuals. Some electrocardiographic signs are pathognomonic or fall within validated diagnostic criteria of individual cardiomyopathy such as the inverted T waves in right precordial leads (V1-V3) or beyond in individuals with complete pubertal development in the absence of complete right bundle branch block for the diagnosis of arrhythmogenic cardiomyopathy of the right ventricle (ARVC) or the presence of low voltages typically seen in more than 60% of patients with amyloidosis. Most other electrocardiographic findings such as the presence of depolarization changes including QRS fragmentation, the presence of epsilon wave, the presence of reduced or increased voltages as well as alterations in the repolarization phase including the negative T waves in the lateral leads, or the profound inversion of the T waves or downsloping of the ST tract are more non-specific signs which can however raise the clinical suspicion of cardiomyopathy in order to initiate a diagnostic procedure especially using imaging techniques for diagnostic confirmation. Such electrocardiographic alterations not only have a counterpart in imaging investigations such as evidence of late gadolinium enhancement on magnetic resonance imaging, but may also have an important prognostic value once a definite diagnosis has been made. In addition, the presence of electrical stimulus conduction disturbances or advanced atrioventricular blocks that can be seen especially in conditions such as cardiac amyloidosis or sarcoidosis, or the presence of left bundle branch block or posterior fascicular block in dilated or arrhythmogenic left ventricular cardiomyopathies are recognized as a possible expression of advanced pathology. Similarly, the presence of ventricular arrhythmias with typical patterns such as non-sustained or sustained ventricular tachycardia of LBBB morphology in ARVC or non-sustained or sustained ventricular tachycardia with an RBBB morphology (excluding the "fascicular pattern") in arrhythmogenic left ventricle cardiomyopathy could have a significant impact on the course of each disease. It is therefore clear that a learned and careful interpretation of ECG features can raise suspicion of the presence of a cardiomyopathy, identify diagnostic "red flags" useful for orienting the diagnosis toward specific forms, and provide useful tools for risk stratification. The purpose of this review is to emphasize the important role of the ECG in the diagnostic workup, describing the main ECG findings of different cardiomyopathies.

Low QRS voltages and left ventricular hypertrophy: a risky association.

AIMS: Low QRS voltages (LQRSV) are an unexpected finding in left ventricular hypertrophy, i.e. hypertrophic cardiomyopathy (HCM) or athlete's heart. METHODS AND RESULTS: Prevalence and clinical correlates of LQRSV were investigated in 197 consecutive HCM patients, aged 58 ± 13 years and comparatively in 771 Olympic athletes, aged 23 ± 4. Clinical characterization included family/personal history, symptoms, New York Heart Association (NYHA) functional class, electrocardiographic pattern, ventricular arrhythmias, and cardiac magnetic resonance (CMR). Twenty-two (11%) of HCM and 18 (2.3%) of athletes presented LQRSV. At initial evaluation, in HCM, LQRSV showed no differences vs. non-LQRSV for functional class (90% vs. 91%, in Classes I and II; P = 0.983), symptoms (27% vs. 18%; P = 0.478), and ventricular arrhythmias (40% vs. 39%; P = 857) but showed larger extent of late gadolinium enhancement (LGE) at CMR (4.1 ± 1.5 vs. 1.5 ± 0.7 affected segments; P < 0.001). In athletes, LQRSV was associated with larger prevalence of inverted T-waves (22% vs. 9%; P < 0.001) and ventricular arrhythmias (28% vs. 8%; P = 0.005). In one LQRSV athlete, arrhythmogenic cardiomyopathy was identified. Over 4.5 ± 2.6-year follow-up, presence of LQRSV in HCM was associated with larger incidence of functional deterioration (31% vs. 14%; P = 0.038), stroke (22% vs. 6%; P = 0.008), and implantable cardioverter defibrillator (ICD) implant (27% vs. 10%; P = 0.015). No clinical events occurred in LQRSV athletes without initial evidence of cardiac disease. CONCLUSION: LQRSV are relatively common (11%) in HCM and have clinical relevance, being predictive over a medium term for a worsening functional class, incidence of stroke, and ICD implant. Instead, LQRSV are rare (2.3%) in athletes but may occasionally be a marker that raises suspicion for underlying cardiac disease at risk.

In the present investigation, we sought to assess prevalence and clinical correlates of LQRSV in 197 consecutive HCM patients and, comparatively, in 771 Olympic athletes. Twenty-two (11%) of HCM presented LQRSV. At initial evaluation, LQRSV patients showed no differences vs. non-LQRSV for functional class (90% vs. 91%, in Classes I and II; P = 0.983), symptoms (27% vs. 18%; P = 0.478), and ventricular arrhythmias (40% vs. 39%; P = 857) but showed larger extent of LGE at CMR (4.1 ± 1.5 vs. 1.5 ± 0.7 affected segments; P < 0.001). Over 4.5 ± 2.6-year follow-up, presence of LQRSV was associated with larger incidence of functional class deterioration (31% vs. 14%; P = 0.038), stroke (22% vs. 6%; P = 0.008), and ICD implant (27% vs. 10%; P = 0.015).Eighteen (2.3%) of athletes presented LQRSV. In athletes, LQRSV was associated with larger prevalence of inverted T-waves (22% vs. 9%; P < 0.001) and ventricular arrhythmias (28% vs. 8%; P = 0.005). In one LQRSV athlete, arrhythmogenic cardiomyopathy was identified.In conclusion, LQRSV are relatively common (11%) in HCM and have clinical relevance, being predictive over a medium term for a worsening functional class, incidence of stroke, and ICD implant. Instead, LQRSV are rare (2.3%) in athletes but may be a marker that raises suspicion for underlying cardiac disease at risk.

Cardiovascular Magnetic Resonance in Patients with Cardiac Electronic Devices: Evidence from a Multicenter Study.

BACKGROUND: Most recent cardiac implantable electronic devices (CIEDs) can safely undergo a cardiovascular magnetic resonance (CMR) scan under certain conditions, but metal artifacts may degrade image quality. The aim of this study was to assess the overall diagnostic yield of CMR and the extent of metal artifacts in a multicenter, multivendor study on CIED patients referred for CMR. METHODS: We analyzed 309 CMR scans from 292 patients (age 57 ± 16 years, 219 male) with an MR-conditional pacemaker (n = 122), defibrillator (n = 149), or loop recorder (n = 38); CMR scans were performed in 10 centers from 2012 to 2020; MR-unsafe implants were excluded. Clinical and device parameters were recorded before and after the CMR scan. A visual analysis of metal artifacts was performed for each sequence on a segmental basis, based on a 5-point artifact score. RESULTS: The vast majority of CMR scans (n = 255, 83%) were completely performed, while only 32 (10%) were interrupted soon after the first sequences and 22 (7%) were only partly acquired; CMR quality was non-diagnostic in 34 (11%) scans, poor (<1/3 sequences were diagnostic) in 25 (8%), or acceptable (1/3 to 2/3 sequences were diagnostic) in 40 (13%), while most scans (n = 201, 68%) were of overall good quality. No adverse event or device malfunctioning occurred, and only nonsignificant changes in device parameters were recorded. The most affected sequences were SSFP (median score 0.32 [interquartile range 0.07-0.91]), followed by GRE (0.18 [0.02-0.59]) and LGE (0.14 [0.02-0.55]). ICDs induced more artifacts (median score in SSFP images 0.87 [0.50-1.46]) than PMs (0.11 [0.03-0.28]) or ILRs (0.11 [0.00-0.56]). Moreover, most artifacts were located in the anterior, anteroseptal, anterolateral, and apical segments of the LV and in the outflow tract of the RV. CONCLUSIONS: CMR is a versatile imaging technique, with a high safety profile and overall good image quality even in patients with MR-conditional CIEDs. Several strategies are now available to optimize image quality, substantially enhancing overall diagnostic yield.

The Diagnostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy: Novel ECG Signs.

BACKGROUND: Electrocardiographic (ECG) findings in arrhythmogenic left ventricular cardiomyopathy (ALVC) are limited to small case series. OBJECTIVES: This study aimed to analyze the ECG characteristics of ALVC patients and to correlate ECG with cardiac magnetic resonance and genotype data. METHODS: We reviewed data of 54 consecutive ALVC patients (32 men, age 39 ± 15 years) and compared them with 84 healthy controls with normal cardiac magnetic resonance. RESULTS: T-wave inversion was often noted (57.4%), particularly in the inferior and lateral leads. Low QRS voltages in limb leads were observed in 22.2% of patients. The following novel ECG findings were identified: left posterior fascicular block (LPFB) (20.4%), pathological Q waves (33.3%), and a prominent R-wave in V1 with a R/S ratio ≥0.5 (24.1%). The QRS voltages were lower in ALVC compared with controls, particularly in lead I and II. At receiver-operating characteristic analysis, the sum of the R-wave in I to II ≤8 mm (AUC: 0.909; P < 0.0001) and S-wave in V1 plus R-wave in V6 ≤12 mm (AUC: 0.784; P < 0.0001) effectively discriminated ALVC patients from controls. It is noteworthy that 4 of the 8 patients with an apparently normal ECG were recognized by these new signs. Transmural late gadolinium enhancement was associated to LPFB, a R/S ratio ≥0.5 in V1, and inferolateral T-wave inversion, and a ringlike pattern correlated to fragmented QRS, SV1+RV6 ≤12 mm, low QRS voltage, and desmoplakin alterations. CONCLUSIONS: Pathological Q waves, LPFB, and a prominent R-wave in V1 were common ECG signs in ALVC. An R-wave sum in I to II ≤8 mm and SV1+RV6 ≤12 mm were specific findings for ALVC phenotypes compared with controls.

Cardiac magnetic resonance for prophylactic implantable-cardioverter defibrillator therapy international study: prognostic value of cardiac magnetic resonance-derived right ventricular parameters substudy.

AIMS: Right ventricular systolic dysfunction (RVSD) is an important determinant of outcomes in heart failure (HF) cohorts. While the quantitative assessment of RV function is challenging using 2D-echocardiography, cardiac magnetic resonance (CMR) is the gold standard with its high spatial resolution and precise anatomical definition. We sought to investigate the prognostic value of CMR-derived RV systolic function in a large cohort of HF with reduced ejection fraction (HFrEF). METHODS AND RESULTS: Study cohort comprised of patients enrolled in the CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DefibrillAtor ThErapy registry who had HFrEF and had simultaneous baseline CMR and echocardiography (n = 2449). RVSD was defined as RV ejection fraction (RVEF) <45%. Kaplan-Meier curves and cox regression were used to investigate the association between RVSD and all-cause mortality (ACM). Mean age was 59.8 ± 14.0 years, 42.0% were female, and mean left ventricular ejection fraction (LVEF) was 34.0 ± 10.8. Median follow-up was 959 days (interquartile range: 560-1590). RVSD was present in 936 (38.2%) and was an independent predictor of ACM (adjusted hazard ratio = 1.44; 95% CI [1.09-1.91]; P = 0.01). On subgroup analyses, the prognostic value of RVSD was more pronounced in NYHA I/II than in NYHA III/IV, in LVEF <35% than in LVEF ≥35%, and in patients with renal dysfunction when compared to those with normal renal function. CONCLUSION: RV systolic dysfunction is an independent predictor of ACM in HFrEF, with a more pronounced prognostic value in select subgroups, likely reflecting the importance of RVSD in the early stages of HF progression.

Cardiac Magnetic Resonance for Prophylactic Implantable-Cardioverter Defibrillator Therapy in Ischemic Cardiomyopathy: The DERIVATE-ICM International Registry.

BACKGROUND: Implantable cardioverter-defibrillator (ICD) therapy is the most effective prophylactic strategy against sudden cardiac death (SCD) in patients with ischemic cardiomyopathy (ICM) and left ventricle ejection fraction (LVEF) ≤35% as detected by transthoracic echocardiograpgy (TTE). This approach has been recently questioned because of the low rate of ICD interventions in patients who received implantation and the not-negligible percentage of patients who experienced SCD despite not fulfilling criteria for implantation. OBJECTIVES: The DERIVATE-ICM registry (CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy; NCT03352648) is an international, multicenter, and multivendor study to assess the net reclassification improvement (NRI) for the indication of ICD implantation by the use of cardiac magnetic resonance (CMR) as compared to TTE in patients with ICM. METHODS: A total of 861 patients with ICM (mean age 65 ± 11 years, 86% male) with chronic heart failure and TTE-LVEF <50% participated. Major adverse arrhythmic cardiac events (MAACE) were the primary endpoints. RESULTS: During a median follow-up of 1,054 days, MAACE occurred in 88 (10.2%). Left ventricular end-diastolic volume index (HR: 1.007 [95% CI: 1.000-1.011]; P = 0.05), CMR-LVEF (HR: 0.972 [95% CI: 0.945-0.999]; P = 0.045) and late gadolinium enhancement (LGE) mass (HR: 1.010 [95% CI: 1.002-1.018]; P = 0.015) were independent predictors of MAACE. A multiparametric CMR weighted predictive derived score identifies subjects at high risk for MAACE compared with TTE-LVEF cutoff of 35% with a NRI of 31.7% (P = 0.007). CONCLUSIONS: The DERIVATE-ICM registry is a large multicenter registry showing the additional value of CMR to stratify the risk for MAACE in a large cohort of patients with ICM compared with standard of care.