Catheter ablation of parahisian premature ventricular complexes in patients with and without cardiac scar.

BACKGROUND: Ablation of premature ventricular complexes (PVCs) originating from the parahisian area is challenging. Late gadolinium-enhanced cardiac magnetic resonance (LGE-CMR) scar may influence procedural outcomes; the impact of cardiac scar on parahisian PVCs has not been described. OBJECTIVE: The objective of this study was to examine the incidence and significance of LGE-CMR scarring in patients undergoing ablation for parahisian PVCs. METHODS: Consecutive patients who underwent preprocedure LGE-CMR imaging and ablation of parahisian PVCs were included. Acute and long-term outcomes were examined. RESULTS: Forty-eight patients were included (male, n = 37; age, 66 ± 10 years; ejection fraction, 50% ± 12%; preprocedure PVC burden, 21% ± 12%). Intramural LGE-CMR scar was present in 33 of 48 (69%) patients. Cryoablation was used in 9 patients; ablation in multiple chambers was required in 28 (58%) patients. The PVC site of origin (SOO) was intramural (n = 25 patients), left ventricular (n = 5), and right ventricular (n = 18). Patients with LGE-CMR scar were more likely to have intramural PVCs (64% vs 27%; P < .04) and to require ablation in multiple cardiac chambers (58% vs 13%; P < .02). Patients with intramural scar required longer duration of ablation delivery (31 ± 20 minutes vs 17 ± 8 minutes; P < .02). Acute procedural success was 69%; PVC burden on follow-up was 6% ± 9% and similar for those with and without scar. CONCLUSION: Ablation of parahisian PVCs often requires mapping and ablation of multiple cardiac chambers, with an intramural SOO identified in most patients. An intramural scar was associated with an intramural SOO of the PVCs requiring more extensive ablation procedures, with similar long-term outcomes compared with those without scar.

Ventricular arrhythmias in patients with bicuspid aortic valves.

INTRODUCTION: Bicuspid aortic valves (BAV) are the most common congenital heart defects and the extent of ventricular arrhythmias (VA) in patients with BAV is unclear. The objective of this study is to describe VAs and late gadolinium enhancement cardiac magnetic resonance imaging (LGE-CMR) in patients with BAV. METHODS: A total of 19 patients with BAV (18 males, age: 58 ± 13 years) were referred for VA ablation procedures. Ten patients had BAVs at the time of ablation, nine patients had prior aortic valve replacement for a BAV. All but one patient had LGE-CMR and all patients underwent programmed ventricular stimulation at the time of the ablation. RESULTS: Frequent PVCs were the targeted VAs in 17/19 patients and VT in 2/19 patients. Monomorphic ventricular tachycardia (VT) was inducible in 6 patients. A total of 15 VTs were inducible (2.5 ± 1.0 VTs per patient with a mean cycle length of 322 ± 83 msec). LGE was present in 13 patients. Patients with inducible VT had larger borderzone and core scar compared to non-inducible patients (7.8 ± 2.1 cm3 vs. 2.5 ± 3.1 cm3 and 5.1 ± 2.6 cm3 vs. 1.9 ± 3.0 cm3, p-value < .05 for both). PVCs and VTs were mapped to the periaortic valve area in 12 patients and 4 patients, respectively. The PVC burden was reduced from 27 ± 13 to 3 ± 6 (p < .001) and the ejection fraction improved from 49 ± 13% to 55 ± 9% (p = .005). CONCLUSIONS: VAs in patients with BAV often originate from the perivalvular area and patients often have LGE and inducible VT. LGE may be due to ventricular remodeling secondary to the presence of BAV and harbors the arrhythmogenic substrate for VT.

Ventricular Tachycardia Ablation in Patients With Desmoplakin Cardiomyopathy.

BACKGROUND: Desmoplakin (DSP) pathogenic variants are rare causes of arrhythmogenic cardiomyopathy and often involve the right and left ventricles. Ventricular tachycardia (VT) ablations may be required in these patients, but procedural characteristics have not been reported. OBJECTIVES: In this study, the authors sought to report a multicenter experience of VT ablation in patients with DSP pathogenic variants. METHODS: VT ablations performed in patients with known DSP pathogenic variants were analyzed across 6 centers in 3 countries. Patient characteristics and acute and long-term procedural outcomes were reported. RESULTS: A total of 20 patients (13 men, median age 43 years [Q1-Q3: 41.5-53.0 years], left ventricular ejection fraction 43.0% [Q1-Q3: 41.5%-53.0%], 11 previous failed ablations) were referred for VT ablation procedures. All patients had symptomatic VTs, with ICD therapy in 19 patients. Epicardial procedures were performed in 16 of the 20 patients. VT target sites were located in the right ventricular (RV) endocardium (n = 11), the RV epicardium (n = 4), the left ventricular (LV) endocardium (n = 2) and the LV epicardium (n = 7). In 3 patients, the VT target sites were in close proximity to coronary arteries, limiting ablation. During follow-up, VTs recurred in 11 patients, and repeated ablations were performed in 9 patients. Allowing for multiple procedures, 19 of the 20 patients remained free of VT recurrence after a median follow-up of 18 months [Q1-Q3: 5-60 months]. CONCLUSIONS: Patients with DSP cardiomyopathy often have biventricular involvement, and ablation procedures often require ablation in both ventricles and the epicardium. Recurrences are not uncommon, and the pathologic substrate can be located in close proximity to epicardial coronary arteries, limiting the success rate of ablations.

SCMR expert consensus statement for cardiovascular magnetic resonance of patients with a cardiac implantable electronic device.

Cardiovascular magnetic resonance (CMR) is a proven imaging modality for informing diagnosis and prognosis, guiding therapeutic decisions, and risk stratifying surgical intervention. Patients with a cardiac implantable electronic device (CIED) would be expected to derive particular benefit from CMR given high prevalence of cardiomyopathy and arrhythmia. While several guidelines have been published over the last 16 years, it is important to recognize that both the CIED and CMR technologies, as well as our knowledge in MR safety, have evolved rapidly during that period. Given increasing utilization of CIED over the past decades, there is an unmet need to establish a consensus statement that integrates latest evidence concerning MR safety and CIED and CMR technologies. While experienced centers currently perform CMR in CIED patients, broad availability of CMR in this population is lacking, partially due to limited availability of resources for programming devices and appropriate monitoring, but also related to knowledge gaps regarding the risk-benefit ratio of CMR in this growing population. To address the knowledge gaps, this SCMR Expert Consensus Statement integrates consensus guidelines, primary data, and opinions from experts across disparate fields towards the shared goal of informing evidenced-based decision-making regarding the risk-benefit ratio of CMR for patients with CIEDs.

Pleomorphism of premature ventricular complexes originating from papillary muscles and their myocardial connections.

BACKGROUND: Patients with arrhythmias originating from papillary muscles (PAPs) often have pleomorphic ventricular arrhythmias (PVAs) that can result in failed ablations. The mechanism of PVAs is unknown. OBJECTIVE: The purpose of this study was to assess the prevalence and mechanisms of PVAs and the impact on outcomes in patients with focal left ventricular PAP ventricular arrhythmias (VAs). METHODS: The sites of origin (SOOs) of VAs in 43 consecutive patients referred for ablation of focal left ventricular PAP VAs were determined by activation and pacemapping. SOOs were classified as (1) unifocal generating a single VA morphology; (2) unifocal from a deeper-seated origin generating multiple VA morphologies; (3) unifocal located on a PAP branching site; (4) multifocal from a single or multiple PAPs generating multiple VA morphologies; and (5) multifocal from a PAP and a different anatomic source. RESULTS: Most patients had multiple morphologies (n = 34 [79%]) and multiple mechanisms (79%) generating the different VA morphologies. Most of the patients with PVAs had multiple SOOs from a single or different PAPs (n = 23 [68%]), followed by patients with SOOs from PAP and non-PAP sites (n = 19 [56%]). In 13 patients (38%), single SOOs accounted for the observed PVAs. The frequent observation (n = 20) of changing QRS morphologies after radiofrequency energy delivery targeting a single VA suggests the presence of a deeper focus with changing sites of preferential conduction. CONCLUSION: VA pleomorphism in patients with PAP arrhythmias is most often due to premature ventricular complexes originating from different SOOs. The second most common cause is preferential conduction from a single SOO via PAP branching sites.

Long-term outcomes of patients with ventricular arrhythmias and negative programmed ventricular stimulation followed with implantable loop recorders: Impact of delayed-enhancement cardiac magnetic resonance imaging.

BACKGROUND: Programed ventricular stimulation (PVS) is a risk stratification tool in patients at risk for adverse arrhythmia outcomes. Patients with negative PVS may yet be at risk for adverse arrhythmia-related events, particularly in the presence of symptomatic ventricular arrhythmias (VA). OBJECTIVE: To investigate the long-term outcomes of real-world patients with symptomatic VA without indication for device therapy and negative PVS, and to examine the role of cardiac scaring on arrhythmia recurrence. METHODS: Patients with symptomatic VA, and late gadolinium enhancement cardiac magnetic resonance imaging (LGE-CMR), and negative PVS testing were included. All patients underwent placement of implantable cardiac monitors (ICM). Survival analysis was performed to investigate the impact of LGE-CMR findings on survival free from adverse arrhythmic events. RESULTS: Seventy-eight patients were included (age 60 ± 14 years, women n = 36 (46%), ejection fraction 57 ± 9%, cardiomyopathy n = 26 (33%), mitral valve prolapse [MVP] n = 9 (12%), positive LGE-CMR scar n = 49 (62%), history of syncope n = 23 (29%)) including patients with primarily premature ventricular contractions (n = 21) or nonsustained VA (n = 57). Patients were followed for 1.6 ± 1.5 years during which 14 patients (18%) experienced VA requiring treatment (n = 14) or syncope due to bradycardia (n = 2). Four/9 patients (44%) with MVP experienced VA (n = 3) or syncope (n = 1). Baseline characteristics between those with and without adverse events were similar (p > 0.05); however, the presence of cardiac scar on LGE-CMR was independently associated with an increased risk of adverse events (hazard ratio: 5.6 95% confidence interval: [1.2-27], p = 0.03, log-rank p = 0.03). CONCLUSIONS: In a real-world cohort with long-term follow-up, adverse arrhythmic outcomes occurred in 18% of patients with symptomatic VA despite negative PVS, and this risk was significantly greater in patients with positive DE-CMR scar. Long term-monitoring, including the use of ICM, may be appropriate in these patients.

Gender and race-related disparities in the management of ventricular arrhythmias.

Modern studies have revealed gender and race-related disparities in the management and outcomes of cardiac arrhythmias, but few studies have focused on outcomes for ventricular arrhythmias (VAs) such as ventricular tachycardia (VT) or ventricular fibrillation (VF). The aim of this article is to review relevant studies and identify outcome differences in the management of VA among Black and female patients. We found that female patients typically present younger for VA, are more likely to have recurrent VA after catheter ablation, are less likely to be prescribed antiarrhythmic medication, and are less likely to receive primary prevention ICD placement as compared to male patients. Additionally, female patients appear to derive similar overall mortality benefit from primary prevention ICD placement as compared to male patients, but they may have an increased risk of acute post-procedural complications. We also found that Black patients presenting with VA are less likely to undergo catheter ablation, receive appropriate primary prevention ICD placement, and have significantly higher risk-adjusted 1-year mortality rates after hospital discharge as compared to White patients. Black female patients appear to have the worst outcomes out of any demographic subgroup.

Value of genotyping and scar-phenotyping for VT ablation procedures in patients with nonischemic left ventricular cardiomyopathies.

INTRODUCTION: Variants of cardiomyopathy genes in patients with nonischemic cardiomyopathy (NICM) generate various phenotypes of cardiac scar and delayed enhancement cardiac magnetic resonance (DE-CMR) imaging which may impact ventricular tachycardia (VT) management. METHODS: The objective was to compare the findings of cardiomyopathy genetic testing on DE-CMR imaging and long-term outcomes among patients with NICM undergoing VT ablation procedures. Image phenotyping and genotyping were performed in a consecutive series of patients referred for VT ablation and correlated to survival free of VT. Scar depth index (SDI) (% of scar at 0-3 mm, 3-5 mm and >5 mm projected on the closest endocardial surface) was determined. RESULTS: Forty-three patients were included (11 women, 55 ± 14 years, ejection fraction (EF) 45 ± 16%) and were followed for 3.4 ± 2.9 years. Pathogenic variants (PV) were identified in 16 patients (37%) in the following genes: LMNA (n = 5), TTN (n = 5), DSP (n = 2), AMLS1 (n = 1), MYBPC3 (n = 1), PLN (n = 1), and SCN5A (n = 1). A ring-like septal scar (RLSS) pattern was more often seen in patients with pathogenic variants (66% vs 15%, p = .001). RLSS was associated with deeper seated scars (SDI >5 mm 30.6 ± 22.6% vs 12.4 ± 16.2%, p = .005), and increased VT recurrence (HR 5.7 95% CI[1.8-18.4], p = .003). After adjustment for age, sex, EF, and total scar burden, the presence of a PV remained independently associated with worse outcomes (HR 4.7 95% CI[1.22-18.0], p = .02). CONCLUSIONS: Preprocedural genotyping and scar phenotyping is beneficial to identify patients with a favorable procedural outcome. Some PVs are associated with an intramural, deeper seated scar phenotype and have an increase of VT recurrence after ablation.

Implications of the anatomy of papillary muscle connections for mapping and ablation of focal ventricular arrhythmias.

BACKGROUND: Ventricular arrhythmias (VAs) originating from papillary muscles (PAPs) can be challenging when targeted with catheter ablation. Reasons may include premature ventricular complex pleomorphism, structurally abnormal PAPs, or unusual origins of VAs from PAP-myocardial connections (PAP-MYCs). OBJECTIVE: The purpose of this study was to correlate PAP anatomy with mapping and ablation of PAP VAs. METHODS: In a series of 43 consecutive patients with frequent PAP arrhythmias referred for ablation, the anatomy and structure of PAPs and VA origins were analyzed using multimodality imaging. Successful ablation sites were analyzed for location on the PAP body or a PAP-MYC. RESULTS: In a total of 17 of 43 patients (40%), VAs originated from a PAP-MYC (in 5 of 17 patients, the PAP inserted into the mitral valve anulus); and in 41 patients, VAs originated from a PAP body. VAs from a PAP-MYC more often had delayed R-wave transition than did other PAP VAs (69% vs 28%; P < .001). Patients with failed procedures had more PAP-MYCs (24.8 ± 8 PAP-MYCs per patient vs 16 ± 7 PAP-MYCs per patient; P < .001). CONCLUSION: Multimodality imaging identifies anatomic details of PAPs that facilitate mapping and ablation of VAs. In more than a third of patients with PAP VAs, VAs originate from connections between PAPs and the surrounding myocardium or between other PAPs. VA electrocardiographic morphologies are different when VAs originate from PAP-connection sites as compared with VAs originating from the PAP body.

Risk of Adverse Outcomes Associated With Cardiac Sarcoidosis Diagnostic Schemes.

BACKGROUND: Multiple cardiac sarcoidosis (CS) diagnostic schemes have been published. OBJECTIVES: This study aims to evaluate the association of different CS diagnostic schemes with adverse outcomes. The diagnostic schemes evaluated were 1993, 2006, and 2017 Japanese criteria and the 2014 Heart Rhythm Society criteria. METHODS: Data were collected from the Cardiac Sarcoidosis Consortium, an international registry of CS patients. Outcome events were any of the following: all-cause mortality, left ventricular assist device placement, heart transplantation, and appropriate implantable cardioverter-defibrillator therapy. Logistic regression analysis evaluated the association of outcomes with each CS diagnostic scheme. RESULTS: A total of 587 subjects met the following criteria: 1993 Japanese (n = 310, 52.8%), 2006 Japanese (n = 312, 53.2%), 2014 Heart Rhythm Society (n = 480, 81.8%), and 2017 Japanese (n = 112, 19.1%). Patients who met the 1993 criteria were more likely to experience an event than patients who did not (n = 109 of 310, 35.2% vs n = 59 of 277, 21.3%; OR: 2.00; 95% CI: 1.38-2.90; P < 0.001). Similarly, patients who met the 2006 criteria were more likely to have an event than patients who did not (n = 116 of 312, 37.2% vs n = 52 of 275, 18.9%; OR: 2.54; 95% CI: 1.74-3.71; P < 0.001). There was no statistically significant association between the occurrence of an event and whether a patient met the 2014 or the 2017 criteria (OR: 1.39; 95% CI: 0.85-2.27; P = 0.18 or OR: 1.51; 95% CI: 0.97-2.33; P = 0.067, respectively). CONCLUSIONS: CS patients who met the 1993 and the 2006 criteria had higher odds of adverse clinical outcomes. Future research is needed to prospectively evaluate existing diagnostic schemes and develop new risk models for this complex disease.

Mitral Annular Substrate and Ventricular Arrhythmias in Arrhythmogenic Mitral Valve Prolapse With Mitral Annular Disjunction.

BACKGROUND: In patients with bileaflet mitral valve prolapse (MVP), mitral annular disjunction (MAD) is associated with increased risk of sudden cardiac death via incompletely understood mechanisms. OBJECTIVES: This study assessed the substrate for ventricular arrhythmias in patients with bileaflet MVP and MAD as well as outcomes of catheter ablation with an emphasis on sustained, monomorphic ventricular tachycardia (VT). METHODS: A total of 18 consecutive patients (11 women, mean age 54 ± 15 years) with bileaflet MVP and MAD underwent catheter ablation for VT, and/or premature ventricular complexes (PVCs). Eight patients had a prior cardiac arrest. RESULTS: PVCs were targeted for ablation in all 18 patients (symptomatic PVCs n = 15, PVC-induced ventricular fibrillation n = 3). Sustained monomorphic VT was targeted in 7 of 18 patients. Electroanatomic mapping showed low voltage in the area of the mitral annulus corresponding to VT target sites in 6 of 7 patients with sustained VT. Four of 7 patients had low voltage in the areas of MAD. Six of 7 patients with VT were rendered noninducible post-ablation. The PVC burden was reduced from 11.0% ± 10.4% to 4.0% ± 5.5% (P = 0.004). Over a mean follow-up of 33.9 ± 43.4 months, no VTs recurred. There were no major complications. No repeat ablations for VT occurred. Five of 18 patients required repeat ablation for PVCs. CONCLUSIONS: In patients with bileaflet MVP and MAD undergoing catheter ablation, the mitral valve annulus often contains low-voltage areas harboring the substrate for monomorphic VT and PVCs. Ablation in these patients was safe and improved arrhythmia control.

Strain Analysis in Patients with Frequent Premature Ventricular Complexes and Preserved Left Ventricular Function Undergoing Ablation.

BACKGROUND: Frequent premature ventricular complexes (PVCs) can cause PVC-induced cardiomyopathy. The value of PVC ablation in patients with preserved left ventricular function in the low-normal range (ejection fraction: 50-55%) is not established. Strain analysis has been used to estimate changes in left ventricular function beyond assessment of the ejection fraction (EF). Longitudinal strain has been proposed as a method to detect changes over time in the setting of frequent asymptomatic premature ventricular complexes and preserved left ventricular (LV) function. A decrease in strain may be evidence of PVC-induced cardiomyopathy. OBJECTIVE: In this study, we assessed the role of PVC ablation in patients with low-normal EF and the effect on EF and myocardial strain before and after PVC ablation. METHODS: A total of 70 consecutive patients with either low-normal EF (0.5-<0.55, n = 35) or high-normal EF (≥0.55; n = 35), using available imaging and Holter data, were referred for ablation due to frequent PVCs. EF and longitudinal strain were assessed pre- and post-ablation. RESULTS: There was a significant increase in EF (53.2 ± 0.4% to 58.3 ± 0.5%, p < 0.001) and improvement in longitudinal strain (-15.2 ± 3.3 to -16.6 ± 3, p = 0.007) post-ablation in patients with low-normal EF and successful ablation. There was no change in EF or longitudinal strain in patients with high-normal EF and a successful ablation pre- vs. post-ablation. CONCLUSIONS: Patients with frequent PVCs and low-normal LV EF compared to patients with frequent PVCs and high-normal LV EF have evidence of PVC-induced cardiomyopathy and may benefit from ablation despite a preserved left ventricular EF.

Left and Right PVC-Induced Ventricular Dysfunction.

BACKGROUND: Frequent premature ventricular complexes (PVCs) can result in a reversible form of cardiomyopathy that usually affects the left ventricle (LV). OBJECTIVES: The objective of this study was to assess whether frequent PVCs have an impact on right ventricular (RV) function. METHODS: Serial cardiac magnetic resonance (CMR) studies were performed in a series of 47 patients before and after ablation of frequent PVCs. RESULTS: Patients with RV cardiomyopathy (ejection fraction [EF] <0.45) had more frequent PVCs than did patients without decreased RV function (23% ± 11% vs 15% ± 11%, P = 0.03). Likewise, patients with LV cardiomyopathy (EF <0.50) had more frequent PVCs than did patients without decreased LV function (23% ± 10% vs 14% ± 12%, P = 0.003). LV dysfunction was present in 21 patients (45%). In patients with LV dysfunction, 15 patients (32%) had biventricular dysfunction, and 6 patients (13%) had isolated LV dysfunction. A total of 19 patients (40%) had RV dysfunction, and 4 of the patients with RV dysfunction (9%) had isolated RV dysfunction. Cardiac magnetic resonance was repeated 1.9 ± 1.3 years after ablation. In patients with successful ablation, RV function improved, and in patients without successful ablation, RV function did not significantly change (before and after ablation RVEF 0.45 ± 0.09 and 0.52 ± 0.09; P < 0.001 vs. 0.46 ± 0.07 and 0.48 ± 0.04; P = 0.14, respectively). CONCLUSIONS: Frequent PVCs can cause RV cardiomyopathy that parallels LV cardiomyopathy and is reversible with successful ablation.