5164New risk prediction score for life-threatening ventricular tachyarrhythmias in laminopathies

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
K Wahbi ◽  
R Ben Yaou ◽  
E Gandjbakhch ◽  
F Anselme ◽  
T Gossios ◽  
...  

Abstract Background An accurate estimation of the risk of life-threatening (LT) ventricular tachyarrhythmia (VTA) in patients with LMNA mutations is crucial to select candidates for implantable cardioverter defibrillator (ICD) implantation. Methods We included 839 adult patients with LMNA mutations, including 660 from a French nationwide registry in the development sample, and 179 from other countries, referred to 5 tertiary centers for cardiomyopathies, in the validation sample. LTVTA was defined as a) sudden cardiac death or b) ICD-treated or hemodynamically unstable VTA. The prognostic model was derived using Fine-Gray's regression model. The net reclassification was compared with current clinical practice guidelines. The results are presented as means (standard deviation) or medians [interquartile range]. Results We included 444 patients 40.6 (14.1) years of age in the derivation sample and 145 patients 38.2 (15.0) years in the validation sample, of whom 86 (19.3%) and 34 (23.4%) suffered LTVTA over 3.6 [1.0–7.2] and 5.1 [2.0–9.3] years of follow-up, respectively. Predictors of LTVTA in the derivation sample were: male sex, non-missense LMNA mutation, 1st degree and higher atrioventricular block, non-sustained ventricular tachycardia, and left ventricular ejection fraction. In the derivation sample, C-index (95% CI) of the model was 0.776 (0.711–0.842). In the external validation sample, the C-index was 0.800 (0.642–0.959) and calibration slope 1.082 (95% CI, 0.643–1.522). A 5-year estimated risk threshold ≥7% predicted 96.2% of LTVTA and net reclassified 28.8% of patients with LTVTA compared with the guidelines-based approach. Conclusions Compared to the current standard of care, this risk prediction model for LTVTA in laminopathies facilitated significantly the choice of ICD candidates. Acknowledgement/Funding AFM Téléthon

PLoS ONE ◽  
2021 ◽  
Vol 16 (6) ◽  
pp. e0252903
Author(s):  
Mufaddal Mahesri ◽  
Kristyn Chin ◽  
Abheenava Kumar ◽  
Aditya Barve ◽  
Rachel Studer ◽  
...  

Background Ejection fraction (EF) is an important prognostic factor in heart failure (HF), but administrative claims databases lack information on EF. We previously developed a model to predict EF class from Medicare claims. Here, we evaluated the performance of this model in an external validation sample of commercial insurance enrollees. Methods Truven MarketScan claims linked to electronic medical records (EMR) data (IBM Explorys) containing EF measurements were used to identify a cohort of US patients with HF between 01-01-2012 and 10-31-2019. By applying the previously developed model, patients were classified into HF with reduced EF (HFrEF) or preserved EF (HFpEF). EF values recorded in EMR data were used to define gold-standard HFpEF (LVEF ≥45%) and HFrEF (LVEF<45%). Model performance was reported in terms of overall accuracy, positive predicted values (PPV), and sensitivity for HFrEF and HFpEF. Results A total of 7,001 HF patients with an average age of 71 years were identified, 1,700 (24.3%) of whom had HFrEF. An overall accuracy of 0.81 (95% CI: 0.80–0.82) was seen in this external validation sample. For HFpEF, the model had sensitivity of 0.96 (95%CI, 0.95–0.97) and PPV of 0.81 (95% CI, 0.81–0.82); while for HFrEF, the sensitivity was 0.32 (95%CI, 0.30–0.34) and PPV was 0.73 (95%CI, 0.69–0.76). These results were consistent with what was previously published in US Medicare claims data. Conclusions The successful validation of the Medicare claims-based model provides evidence that this model may be used to identify patient subgroups with specific EF class in commercial claims databases as well.


Author(s):  
Oghenesuvwe Eboh ◽  
Yuxuan Mao ◽  
Elisabeth Lee ◽  
Victor Okunrintemi ◽  
Ouassim Derbal ◽  
...  

Introduction: The implantable cardioverter defibrillator (ICD) reduces mortality in patients at risk for potentially life-threatening arrhythmias. Access and distribution of ICDs in rural or economically disadvantaged populations is suspected to be low. This study examined Out of Hospital Premature Natural Death data (OHPND) and electronic medical record (EMR) data to identify rates of non-implantation of ICDs in a sample of decedents in eastern North Carolina. Methods and Results: Death certificate information on 1,316 decedents were matched with EMR data (N = 967, 73.4%). Chart review identified 70 (7.2%) potential ICD candidates with a left ventricular ejection fraction (LVEF) ≤35%. Of the 70 identified patients, 5 (7.1%) did not meet criteria because LVEF subsequently improved. Of the remaining 65 patients, 32 (49.2%) already received an ICD or a wearable cardioverter-defibrillator (WCD), and 33 patients (50.7%) met ICD implantation criteria but had not received one. The reasons identified for non-implantation of ICDs included: limited life expectancy secondary to comorbidities, principally chronic kidney disease (CKD) (N=11, 17%), lack of physician adherence to guidelines (N=9, 14%), lost to follow-up (N=7, 11%), patient refusal (N=5, 8%), and patients yet to commence guideline-directed medical therapy (N=1, 2%). Among our cohort of 967 individuals who died unexpectedly at home, 9 (0.9%) patients may have avoided unexpected death with an ICD/WCD. Conclusion: This study using decedent data shows low rates of ICD-underutilization in a rural population, but also emphasizes the role that advanced comorbidities such as CKD play in ICD-underutilization.


2020 ◽  
Author(s):  
Mufaddal Mahesri ◽  
Kristyn Chin ◽  
Abheenava Kumar ◽  
Aditya Barve ◽  
Rachel Studer ◽  
...  

ABSTRACTBACKGROUNDEjection fraction (EF) is an important prognostic factor in heart failure (HF), but administrative claims databases lack information on EF. We previously developed a model to predict EF class from Medicare claims. Here, we evaluated the performance of this model in an external validation sample of commercial insurance enrollees.METHODTruven MarketScan claims linked to electronic medical records (EMR) data (IBM Explorys) containing EF measurements were used to identify a cohort of US patients with HF between 01-01-2012 and 10-31-2019. By applying the previously developed model, patients were classified into HF with reduced EF (HFrEF) or preserved EF (HFpEF). EF values recorded in EMR data were used to define gold-standard HFpEF (LVEF ≥45%) and HFrEF (LVEF<45%). Model performance was reported in terms of overall accuracy, positive predicted values (PPV), and sensitivity for HFrEF and HFpEF.RESULTSA total of 7,001 HF patients with an average age of 71 years were identified, 1,700 (24.3%) of whom had HFrEF. An overall accuracy of 0.81 (95% CI: 0.80-0.82) was seen in this external validation sample. For HFpEF, the model had sensitivity of 0.96 (95%CI, 0.95-0.97) and PPV of 0.81 (95% CI, 0.81-0.82); while for HFrEF, the sensitivity was 0.32 (95%CI, 0.30-0.34) and PPV was 0.73 (95%CI, 0.69-0.76). These results were consistent with what was previously published in US Medicare claims data.CONCLUSIONSThe successful validation of the Medicare claims-based model provides evidence that this model may be used to identify patient subgroups with specific EF class in commercial claims databases as well.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Ramtin Anousheh ◽  
David E Krummen ◽  
Navinder S Sawhney ◽  
Wei Chung Chen ◽  
Linda Tone ◽  
...  

To investigate the association between resting heart rate (HR) and defibrillation threshold (DFT) in patients (pts) undergoing ICD implantation. DFT testing is usually considered standard of care during ICD implantation. However, the risk factors for high DFTs remain ill defined and the extent of testing required at implant has not been well defined. Baseline HR has been associated with higher DFTs in prior studies. We studied 128 pts undergoing ICD implantation. Baseline HR and DFTs were determined. HR was determined using ECGs obtained in the resting position on the day of ICD implantation. DFT testing was done during ICD implantation. We excluded 13 pts who were on amiodarone. The baseline characteristics of pts in the study are shown below in the table below (values in parenthesis represents standard error of the mean): First, a multivariate analysis of the association between baseline HR and DFT was performed, adjusting for left ventricular ejection fraction (LVEF), gender, body surface area (BSA) and beta blocker therapy. For every 10 beat increase in heart rate, DFT increased by 1 joule (p=0.02). Gender and beta blocker therapy did not effect this association. Second, pts were dichotomized based on DFTs to low (<15 joules) and high (≥15 joules). Mean resting HR was significantly higher among pts with high DFT (79 bpm) compared to those with low DFT (70 bpm) after adjusting for LVEF and BSA (p=0.01). Baseline resting HR is a risk factor for high DFT and may help define a higher risk pt population undergoing DFT testing.


Cardiology ◽  
2017 ◽  
Vol 138 (2) ◽  
pp. 80-86
Author(s):  
Sandra Amorim ◽  
Raquel Garcia ◽  
Teresa Pinho ◽  
João Rodrigues ◽  
Filipe Macedo ◽  
...  

Patients with severely depressed left ventricular ejection fractions (LVEFs) receive implantable cardioverter-defibrillators (ICDs) for the primary prevention of sudden death. However, in some patients, LVEFs may improve or even normalize over time, and these patients would no longer be qualified for ICD implantation based on the original criteria for which they have initially received an ICD. We report a patient with idiopathic dilated cardiomyopathy whose LVEF recovered to normal values after pharmacological therapy. Meanwhile, the patient had life-threatening ventricular fibrillation, aborted by the ICD. We reflect on the pathological features of left ventricular reverse remodelling and ventricular arrhythmogenesis, where the myocardial substrate appears to play an important role. Also, after LVEF improvement in a patient with a cardiac device, there is still a debate on whether we should perform a battery replacement.


Author(s):  
Rory Hachamovitch ◽  
Benjamin Nutter ◽  
Manuel D Cerqueira ◽  

Background . The use of implantable cardiac defibrillators has been associated with improved survival in several well-defined patient (pt) subsets. Its utilization for primary prevention in eligible pts, however, is unclear. We sought to examine the frequency of ICD implantation (ICD-IMP) for primary prevention in a cohort prospectively enrolled in a prospective, multicenter registry of ICD candidates. Methods . We identified 961 pts enrolled in the AdreView Myocardial Imaging for Risk Evaluation in Heart Failure (ADMIRE-HF) study, a prospective, multicenter study evaluating the prognostic usefulness of 123I-mIBG scintigraphy in a heart failure population. Inclusion criteria limited patients to those meeting guideline criteria for ICD implantation; these criteria included left ventricular ejection fraction ≤35% and New York Heart Association functional class II-III. We excluded pts with an ICD at the time of enrollment, leaving a study cohort of 934 patients. Pts were followed up for 24 months after enrollment. Pts undergoing ICD-IMP after enrollment for secondary prevention were censored at the time of intervention. The association between ICD-IMP utilization and demographic, clinical, laboratory, and imaging data was examined using Cox proportional hazards analysis (CPH). Results . Of 934 pts, 196 (21%) were referred for ICD-IMP over a mean follow-up of 612±242 days. Implantations occurred 167±164 days after enrollment. Patients referred for ICD were younger (61±12 vs. 63±12), but did not differ with respect to proportion female (17% vs. 21%), African-American race (12% vs. 15%), diabetics (37% vs. 36%) (All p=NS). The frequency of ICD-IMP did not differ as a function of age, race, sex, LVEF, or imaging result (All p=NS). CPH revealed that a model including age, race, sex, diabetes, smoking, BMI, NYHA class, hypertension, heart failure etiology, and prior MI identified none of these as predictive of ICD-IMP. Conclusion: This analysis of prospective registry data reveals that in patients who are guideline-defined candidates for ICD-IMP, only about one in five receive an ICD over a two year follow-up interval. Multivariable modeling failed to identify any factor associated with ICD use.


2021 ◽  
Vol 17 ◽  
Author(s):  
Issa Pour-Ghaz ◽  
Mark Heckle ◽  
Ikechukwu Ifedili ◽  
Sharif Kayali ◽  
Christopher Nance ◽  
...  

: Implantable cardioverter-defibrillator (ICD) therapy is indicated for patients at risk for sudden cardiac death due to ventricular tachyarrhythmia. The most commonly used risk stratification algorithms use left ventricular ejection fraction (LVEF) to determine which patients qualify for ICD therapy, even though LVEF is a better marker of total mortality than ventricular tachyarrhythmias mortality. This review evaluates imaging tools and novel biomarkers proposed for better risk stratifying arrhythmic substrate, thereby identifying optimal ICD therapy candidates.


EP Europace ◽  
2019 ◽  
Vol 21 (10) ◽  
pp. 1537-1542 ◽  
Author(s):  
Kevin Willy ◽  
Florian Reinke ◽  
Nils Bögeholz ◽  
Julia Köbe ◽  
Lars Eckardt ◽  
...  

Abstract Aims The subcutaneous implantable cardioverter-defibrillator (S-ICDTM) is an important advance in device therapy for the prevention of sudden cardiac death (SCD). Although current guidelines recommend S-ICDTM use, long-term data are still limited, especially in subgroups such as adult patients with congenital heart diseases. This cohort is of high interest because of the difficult anatomic conditions in these patients. Methods and results All S-ICDTM patients with an underlying congenital heart disease (CHD) resulting in an indication for ICD implantation (n = 20 patients) in our large-scaled single-centre S-ICDTM registry (n = 249 patients) were included in this study. Baseline characteristics, appropriate and inappropriate shocks, and complications were documented in a mean follow-up of 36 months. Primary prevention of SCD was the indication for implantation of an S-ICDTM in six patients (30%). Of all 20 patients with an overall mean age of 40.5 ± 11.5 years, 12 were male (60%). The mean left ventricular ejection fraction was 46.5 ± 11.3%. Nine episodes of ventricular tachycardia (two monomorphic and seven polymorphic) were adequately terminated in three patients (15%). In two patients, T-Wave-Oversensing resulting in an inappropriate shock was observed, which could be managed by changing the sensing vector or activation of the SMART PASSTM filter. There were no S-ICDTM system-related infections. In one patient, surgical revision was necessary due to a persistent haematoma. Conclusion The S-ICDTM seems to be a valuable option for the prevention of SCD in patients with various CHDs and complex anatomical anomalies. The S-ICDTM is safe and works effectively, also in these complex patients. Inadequate shock delivery was rare and could be managed by reprogramming.


Heart ◽  
2020 ◽  
Vol 106 (9) ◽  
pp. 656-664 ◽  
Author(s):  
Antonio Cannatà ◽  
Giulia De Angelis ◽  
Andrea Boscutti ◽  
Camilla Normand ◽  
Jessica Artico ◽  
...  

Sudden cardiac death and arrhythmia-related events in patients with non-ischaemic dilated cardiomyopathy (NICM) have been significantly reduced over the last couple of decades as a result of evidence-based pharmacological and non-pharmacological therapeutic strategies. Nevertheless, the arrhythmic stratification in patients with NICM remains extremely challenging, and the simple indication based on left ventricular ejection fraction appears to be insufficient. Therefore, clinicians need to go beyond the current criteria for implantable cardioverter-defibrillator implantation in the direction of a multiparametric evaluation of arrhythmic risk. Several parameters for arrhythmic risk stratification, ranging from electrocardiographic, echocardiographic, imaging-derived and genetic markers, are crucial for proper arrhythmic risk stratification and a multiparametric evaluation of risk in patients with NICM. In particular, integration of cardiac magnetic resonance parameters (mostly late gadolinium enhancement) and specific genetic information (ie, presence of LMNA, PLN, FLNC mutations) appears fundamental for proper implementation of the current arrhythmic risk stratification. Finally, a novel approach focused on both arrhythmic risk and prediction of left ventricular reverse remodelling during follow-up might be useful for effective multiparametric and dynamic arrhythmic risk stratification in NICM. In the future, a complete and integrated evaluation might be mandatory to implement arrhythmic risk prediction in patients with NICM and to discriminate the competing risk between heart failure-related events and life-threatening arrhythmias.


2020 ◽  
Vol 41 (36) ◽  
pp. 3437-3447 ◽  
Author(s):  
Markus Zabel ◽  
Rik Willems ◽  
Andrzej Lubinski ◽  
Axel Bauer ◽  
Josep Brugada ◽  
...  

Abstract Aims The EUropean Comparative Effectiveness Research to Assess the Use of Primary ProphylacTic Implantable Cardioverter-Defibrillators (EU-CERT-ICD), a prospective investigator-initiated, controlled cohort study, was conducted in 44 centres and 15 European countries. It aimed to assess current clinical effectiveness of primary prevention ICD therapy. Methods and results We recruited 2327 patients with ischaemic cardiomyopathy (ICM) or dilated cardiomyopathy (DCM) and guideline indications for prophylactic ICD implantation. Primary endpoint was all-cause mortality. Clinical characteristics, medications, resting, and 12-lead Holter electrocardiograms (ECGs) were documented at enrolment baseline. Baseline and follow-up (FU) data from 2247 patients were analysable, 1516 patients before first ICD implantation (ICD group) and 731 patients without ICD serving as controls. Multivariable models and propensity scoring for adjustment were used to compare the two groups for mortality. During mean FU of 2.4 ± 1.1 years, 342 deaths occurred (6.3%/years annualized mortality, 5.6%/years in the ICD group vs. 9.2%/years in controls), favouring ICD treatment [unadjusted hazard ratio (HR) 0.682, 95% confidence interval (CI) 0.537–0.865, P = 0.0016]. Multivariable mortality predictors included age, left ventricular ejection fraction (LVEF), New York Heart Association class &lt;III, and chronic obstructive pulmonary disease. Adjusted mortality associated with ICD vs. control was 27% lower (HR 0.731, 95% CI 0.569–0.938, P = 0.0140). Subgroup analyses indicated no ICD benefit in diabetics (adjusted HR = 0.945, P = 0.7797, P for interaction = 0.0887) or those aged ≥75 years (adjusted HR 1.063, P = 0.8206, P for interaction = 0.0902). Conclusion In contemporary ICM/DCM patients (LVEF ≤35%, narrow QRS), primary prophylactic ICD treatment was associated with a 27% lower mortality after adjustment. There appear to be patients with less survival advantage, such as older patients or diabetics.


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