Validation of an Arrhythmogenic Right Ventricular Cardiomyopathy Risk-Prediction Model in a Chinese Cohort

BACKGROUND: The novel arrhythmogenic right ventricular cardiomyopathy (ARVC)-associated ventricular arrhythmias (VAs) risk-prediction model endorsed by Cadrin-Tourigny et al. was recently developed to estimate visual VA risk and was identified to be more effective for predicting ventricular events than the International Task Force Consensus (ITFC) criteria, and the Heart Rhythm Society (HRS) criteria. Data regarding its application in Asians are lacking. OBJECTIVES: We aimed to perform an external validation of this algorithm in the Chinese ARVC population. METHODS: The study enrolled 88 ARVC patients who received implantable cardioverter-defibrillator (ICD) from January 2005 to January 2020. The primary endpoint was appropriate ICD therapies. The novel prediction model was used to calculate a priori predicted VA risk that was compared with the observed rates. RESULTS: During a median follow-up of 3.9 years, 57 (64.8%) patients received the ICD therapy. Patients with implanted ICDs for primary prevention had non-significantly lower rates of ICD therapy than secondary prevention (5-year event rate: 0.46 (0.13-0.66) and 0.80 (0.64-0.89); log-rank p = 0.098). The validation study revealed the C-statistic of 0.833 (95% confidence interval (CI) 0.615-1.000), and the predicted and the observed patterns were similar in primary prevention patients (mean predicted-observed risk: -0.07 (95% CI -0.21, 0.09)). However, in secondary prevention patients, the C-statistic was 0.640 (95% CI 0.510-0.770) and the predicted risk was significantly underestimated (mean predicted-observed risk: -0.32 (95% CI -0.39, -0.24)). The recalibration analysis showed that the performance of the prediction model in secondary prevention patients was improved, with the mean predicted-observed risk of -0.04 (95% CI -0.10, 0.03). CONCLUSIONS: The novel risk-prediction model had a good fitness to predict arrhythmic risk in Asian ARVC patients for primary prevention, and for secondary prevention patients after recalibration of the baseline risk

Changes in Exercise Capacity and Ventricular Function in Arrhythmogenic Right Ventricular Cardiomyopathy: The Impact of Sports Restriction during Follow-Up

(1) Background: Physical exercise has been suggested to promote disease progression in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). We aimed to investigate the exercise performance and ventricular function of ARVC patients during follow-up, while taking into account their adherence to exercise restriction recommendations. (2) Methods: This retrospective study included 49 patients (33 male, 67%) who had an exercise test at baseline and after 4.2 ± 1.6 years. Of the 49 ARVC patients, 27 (55%) were athletes, while 22 (45%) were non-athletes. Of the athletes, 12 (44%) continued intensive sports activity (non-adherent), while 15 (56%) stopped intensive physical activity upon recommendation (adherent). The maximum workload in Watts (W), percentage of the target workload (W%), and double product (DP) factor were measured for all patients. (3) Results: The non-adherent cohort had a significant decrease in physical performance (W at baseline vs. follow-up, p = 0.012; W% at baseline vs. follow-up, p = 0.025; DP-factor at baseline vs. follow-up, p = 0.012) over time. Left ventricular (LV) function (LV ejection fraction at baseline vs. follow-up, p = 0.082) showed a decreasing trend in the non-adherent cohort, while the performance of the adherent cohort remained at a similar level. (4) Conclusions: If intensive sports activities are not discontinued, exercise capacity and left ventricular function of athletes with ARVC deteriorates during follow-up. All patients with ARVC need to strictly adhere to the recommendation to cease intense sports activity in order to halt disease progression

Arrhythmic Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an heritable cardiomyopathy characterized by a predominantly arrhythmic presentation. It represents the leading cause of sudden cardiac death (SCD) among athletes and poses a significant morbidity treat in the general population. As a causative treatment for ARVC is still not available, the placement of an implantable cardioverter defibrillator (ICD) represent the current cornerstone for SCD prevention in this setting. Thanks to international ARVC-dedicated efforts, significant steps have been achieved in recent years towards an individualized, patient-centered risk stratification approach. A novel risk calculator algorithm estimating the 5 year risk of arrhythmias of patients with ARVC have been introduced in clinical practice and subsequently validated. The purpose of this article is to summarize the body of evidence that has allowed the development of this tool and to discuss the best way to implement its use in the care of an individual patient

Gender differences in percutaneous coronary intervention for chronic total occlusions from the ERCTO study

Gender-specific data addressing percutaneous coronary intervention (PCI) of chronic total occlusion (CTO) in female patients are scarce and based on small sample size studies.We aimed to analyze gender-differences regarding in-hospital clinical outcomes after CTO-PCI.Data from 35,449 patients enrolled in the prospective European Registry of CTOs were analyzed. The primary outcome was the comparison of procedural success rate in the two cohorts (women vs. men), defined as a final residual stenosis less than 20%, with Thrombolysis In Myocardial Infarction grade flow = 3. In-hospital major adverse cardiac and cerebrovascular events (MACCEs) and procedural complications were deemed secondary outcomes.Women represented 15.2% of the entire study population. They were older and more likely to have hypertension, diabetes, and renal failure, with an overall lower J-CTO score. Women showed a higher procedural success rate (adjusted OR [aOR] = 1.115, confidence interval [CI]: 1.011-1.230, p = 0.030). Apart from previous myocardial infarction and surgical revascularization, no other significant gender differences were found among predictors of procedural success. Antegrade approach with true-to-true lumen techniques was more commonly used than retrograde approach in females. No gender differences were found regarding in-hospital MACCEs (0.9% vs. 0.9%, p = 0.766), although a higher rate of procedural complications was observed in women, such as coronary perforation (3.7% vs. 2.9%, p < 0.001) and vascular complications (1.0% vs. 0.6%, p < 0.001).Women are understudied in contemporary CTO-PCI practice. Female sex is associated with higher procedural success after CTO-PCI, yet no sex differences were found in terms of in-hospital MACCEs. Female sex was associated with a higher rate of procedural complications

Effective nonapical left ventricular pacing with quadripolar leads for cardiac resynchronization therapy

Background: Current guidelines recommend avoiding apical left ventricular (LV) pacing for cardiac resynchronization therapy (CRT). Aims: We investigated the feasibility of nonapical pacing with the current quadripolar LV lead technology. Methods: We analyzed consecutive patients who received CRT with an LV quadripolar lead. The post­­implantation position of each electrode of the LV lead was designated as basal, mid, or apical. The pacing capture threshold (PCT) and phrenic nerve stimulation (PNS) threshold were assessed for each electrode. Results: We enrolled 168 patients. A total of 8 CRT defibrillators were from Biotronik (with Sentus OTW QP leads), 98 were from Boston Scientific (with 21 Acuity X4 Spiral and 77 Acuity X4 Straight leads), and 62 from St. Jude Medical (with Quartet leads). The median (interquartile range) number of electrodes at nonapical segments per patient was 3 (1–4) with Biotronik Sentus leads, 4 (3–4) with spiral ­design Boston Scientific leads, 4 (3–4) with straight Boston Scientific leads, and 3 (3–4) with St. Jude Medical Quartet leads (P = 0.045). Three patients (38%) with Biotronik Sentus leads, 21 (100%) with spiral ­design Boston Scientific leads, 69 (90%) with straight ­design Boston Scientific leads, and 49 (79%) with St. Jude Medical Quartet leads (P &lt; 0.001) had at least 1 electrode located at nonapical segments linked with a PNS ­PCT safety margin of more than 2 V. During the 6­month follow ­up, PNS was detected in 4 patients and was eliminated with reprogramming. No significant changes in PCT were detected during follow ­up. Conclusions: Quadripolar leads allowed nonapical pacing with acceptable electrical parameters in the majority of CRT recipients, although differences were found among the currently available devices

Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy : external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator

Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published guidelines and expert consensus. Methods and results: In a retrospective cohort of 429 individuals from 29 centres in North America and Europe, 103 (24%) experienced sustained VA during a median follow-up of 5.02 (2.05-7.90) years following diagnosis of ARVC. External validation yielded good discrimination [C-index of 0.70 (95% confidence interval-CI 0.65-0.75)] and calibration slope of 1.01 (95% CI 0.99-1.03). Compared with the three published consensus-based decision algorithms for ICD use in ARVC (Heart Rhythm Society consensus on arrhythmogenic cardiomyopathy, International Task Force consensus statement on the treatment of ARVC, and American Heart Association guidelines for VA and SCD), the risk calculator performed better with a superior net clinical benefit below risk threshold of 35%. Conclusion: Using a large independent cohort of patients, this study shows that the ARVC risk model provides good prognostic information and outperforms other published decision algorithms for ICD use. These findings support the use of the model to facilitate shared decision making regarding ICD implantation in the primary prevention of SCD in ARVC

Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator

Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published guidelines and expert consensus.Methods and results In a retrospective cohort of 429 individuals from 29 centres in North America and Europe, 103 (24%) experienced sustained VA during a median follow-up of 5.02 (2.05-7.90) years following diagnosis of ARVC. External validation yielded good discrimination [C-index of 0.70 (95% confidence interval-CI 0.65-0.75)] and calibration slope of 1.01 (95% CI 0.99-1.03). Compared with the three published consensus-based decision algorithms for ICD use in ARVC (Heart Rhythm Society consensus on arrhythmogenic cardiomyopathy, International Task Force consensus statement on the treatment of ARVC, and American Heart Association guidelines for VA and SCD), the risk calculator performed better with a superior net clinical benefit below risk threshold of 35%.Conclusion Using a large independent cohort of patients, this study shows that the ARVC risk model provides good prognostic information and outperforms other published decision algorithms for ICD use. These findings support the use of the model to facilitate shared decision making regarding ICD implantation in the primary prevention of SCD in ARVC

Electrocardiographic findings in patients with arrhythmogenic cardiomyopathy and right bundle branch block ventricular tachycardia

AIMS: Little is known about patients with right bundle branch block (RBBB)-ventricular tachycardia (VT) and arrhythmogenic cardiomyopathy (ACM). Our aims were: (i) to describe electrocardiogram (ECG) characteristics of sinus rhythm (SR) and VT; (ii) to correlate SR with RBBB-VT ECGs; and (iii) to compare VT ECGs with electro-anatomic mapping (EAM) data. METHODS AND RESULTS: From the European Survey on ACM, 70 patients with spontaneous RBBB-VT were included. Putative left ventricular (LV) sites of origin (SOOs) were estimated with a VT-axis-derived methodology and confirmed by EAM data when available.  Overall, 49 (70%) patients met definite Task Force Criteria. Low QRS voltage predominated in lateral leads (n = 37, 55%), but QRS fragmentation was more frequent in inferior leads (n = 15, 23%). T-wave inversion (TWI) was equally frequent in inferior (n = 28, 42%) and lateral (n = 27, 40%) leads. TWI in inferior leads was associated with reduced LV ejection fraction (LVEF; 46 ± 10 vs. 53 ± 8, P = 0.02). Regarding SOOs, the inferior wall harboured 31 (46%) SOOs, followed by the lateral wall (n = 17, 25%), the anterior wall (n = 15, 22%), and the septum (n = 4, 6%). EAM data were available for 16 patients and showed good concordance with the putative SOOs. In all patients with superior-axis RBBB-VT who underwent endo-epicardial VT activation mapping, VT originated from the LV. CONCLUSIONS: In patients with ACM and RBBB-VT, RBBB-VTs originated mainly from the inferior and lateral LV walls. SR depolarization and repolarization abnormalities were frequent and associated with underlying variants