1228 New predictors of sudden cardiac death in hypertrophic cardiomyopathy

Abstract BACKGROUND Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium. HCM is the most common cause of sudden cardiac death (SCD) in young people and competitive athletes due to fatal ventricular arrhythmias. However, in most patients, HCM has a benign course. That is why it is of utmost importance to properly evaluate patients and identify those who would benefit from a cardioverter-defibrillator (ICD) implantation. The HCM SCD-Risk Calculator is a useful tool for estimating the risk of SCD. The parameters included in the model at evaluation are: age, maximum left ventricular (LV) wall thickness, left atrial (LA) dimension, maximum gradient in left ventricular outflow tract, family history of SCD, non-sustained ventricular tachycardia (nsVT) and unexplained syncope. Nevertheless, there is potential to improve and optimize the effectiveness of this tool in clinical practice. Therefore, the following new risk factors are proposed: LV global longitudinal strain (GLS), LV average strain (ASI) and LA volume index (LAVI). GLS and ASI are sensitive and noninvasive methods of assessing LV function. LAVI more accurately characterizes the size of the left atrium in comparison to the LA dimension. METHODS 252 HCM patients (aged 20-88 years, of which 49,6% were men) treated in our Department from 2005 to 2018, were examined. The follow-up period was 0-13 years (average: 3.8 years). SCD was defined as sudden cardiac arrest (SCA) or an appropriate ICD intervention. All patients underwent an echocardiographic examination. The medical and family histories were collected and ICD examinations were performed. RESULTS 76 patients underwent an ICD implantation during the follow-up period. 20 patients have reached an SCD end-point. 1 patient died due to SCA and 19 had an appropriate ICD intervention. There were statistically significant differences of GLS and ASI values between SCD and non-SCD groups; p = 0.026389 and p = 0.006208, respectively. The average GLS in the SCD group was -12.4% ± 3.4%, and -15.1% ± 3.5% in the non-SCD group. The average ASI values were -9.9% ± 3.8% and -12.4% ± 3.5%, respectively. There was a statistically significant difference between LAVI values in SCD and non-SCD groups; p = 0.005343. The median LAVI value in the SCD group was 45.7 ml/m2 and 37.6 ml/m2 in the non-SCD group. The ROC curves showed the following cut-off points for GLS, ASI and LAVI: -13.8%, -13.7% and 41 ml/m2, respectively. Cox’s proportional hazards model for the parameters used in the Calculator was at the borderline of significance; p = 0.04385. The model with new variables (GLS and LAVI instead of LA dimension) was significant; p = 0.00094. The important factors were LAVI; p = 0.000075 and nsVT; p = 0.012267. CONCLUSIONS The proposed new SCD risk factors were statistically significant in the study population and should be taken into account when considering ICD implantation.

Download Full-text

P887Atrial fibrillation is associated with sudden cardiac death risk factors in patients with hypertrophic cardiomyopathy

European Heart Journal ◽

10.1093/eurheartj/ehz747.0484 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

I A Marcuschamer ◽

O Zusman ◽

S Schwartzenberg ◽

M Vaturi ◽

Y Shapira ◽

...

Keyword(s):

Risk Factors ◽

Ventricular Tachycardia ◽

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Cardiac Death ◽

Nearest Neighbor ◽

Nyha Class ◽

Left Ventricular ◽

Similar Distribution ◽

Icd Implantation

Abstract Background Atrial Fibrillation (AF) is prevalent in a fourth of patients with Hypertrophic Cardiomyopathy (HCM), but its clinical impact in these patients remains ill-defined. Aim To compare clinical characteristics in HCM patients with vs without AF and assess indirectly potential sudden cardiac death (SCD) risk. Methods Retrospective study in a single tertiary referral HCM center. Patients with HCM and AF were compared with matched controlled HCM patients without AF. NYHA class was assessed by a single physician. Propensity score matching was performed with a ratio of 2:1 by nearest neighbor with adjustment for age, sex, and left ventricular tract obstruction (LVOTO). Ordinal regression was used with NYHA as outcome. Results Among 298 patients with HCM, 68 patients (22.8%) had AF. After propensity matching, 66 patients with AF and 112 without AF had similar distribution of age (67.1 vs. 65.1 years), gender (57.6% vs. 61% males) and Basal Surface Area (1.88 vs. 1.87 m2) respectively. The prevalence of LVOTO (57.6% vs. 58.5%) and apical hypertrophy (19.7% vs 19.5%) was similar in the two groups. Cardiac risk factors including Hypertension (60.6% vs. 60.2%) and Diabetes Mellitus (15.2 vs. 20.3%) were similar in both groups. AF patients were diagnosed with HCM at a younger age than patients without HCM (48.5 vs. 55 years; p=0.01). HCM patients with AF had significantly lower LVOT gradients compared with patients without AF (28.1 mmHg vs 47.4 mmHg, p=0.005), had a higher prevalence of non-sustained ventricular tachycardia (39.4% vs. 9.4%; p<0.01), and ventricular tachycardia (9.1% vs 1.7%; p<0.04) and were more likely to have undergone implantation of an internal cardioverter defibrillator (ICD) (23.1% vs. 8.5%; p=0.001), respectively. Dyspnea was the most prevalent symptom in both groups (51.1% and 46.6% in AF and non-AF respectively). NYHA Class was similar in both groups: 1.88±0.69 in patients with AF vs. 1.73±0.74 in patients without AF (p=0.17). NYHA class did not differ in 26 patients with chronic persistent AF vs. 42 patients with paroxysmal AF (being in sinus rhythm at evaluation). Conclusion AF does not seem to impact functional level class in patients with HCM, but carries a higher burden of sudden cardiac death prognostic factors, incurring a higher rate of ICD implantation. Acknowledgement/Funding None

Download Full-text

Abstract 11447: Low Incidence of Appropriate Therapy Following Defibrillator Implantation for Primary Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy

Circulation ◽

10.1161/circ.132.suppl_3.11447 ◽

2015 ◽

Vol 132 (suppl_3) ◽

Author(s):

Amalie C Thavikulwat ◽

Todd T Tomson ◽

Bradley P Knight ◽

Robert O Bonow ◽

Lubna Choudhury

Keyword(s):

Risk Factors ◽

Risk Factor ◽

Hypertrophic Cardiomyopathy ◽

Primary Prevention ◽

Sudden Cardiac Death ◽

Secondary Prevention ◽

Cardiac Death ◽

Icd Implantation ◽

Icd Therapy ◽

Appropriate Therapy

Introduction: Hypertrophic cardiomyopathy (HCM) is a leading cause of sudden cardiac death (SCD) in young adults. Implantable cardioverter defibrillators (ICD) effectively terminate ventricular tachycardia (VT) and fibrillation (VF) that cause SCD, but the reported prevalence of and patient characteristics leading to appropriate ICD therapy in HCM have been variable. Hypothesis: We hypothesized that some risk factors may be more prevalent than others in patients with HCM who receive appropriate ICD therapy and that the overall incidence of appropriate therapy may be lower than that reported previously. Methods: We retrospectively studied all patients with HCM who were treated with ICDs at our referral center from 2000-2013 to determine the rates of appropriate and inappropriate ICD therapies. Results: Of 1136 patients with HCM, we identified 135 who underwent ICD implantation (125 for primary and 10 for secondary prevention), aged 18-81 years (mean 48±17) at the time of implantation. The mean follow-up time was 5.2±4.5 years. Appropriate ICD intervention occurred in 20 of 135 patients (2.8%/year) by providing a shock or antitachycardia pacing in response to VT or VF. The annual rate of appropriate ICD therapy was 2.4%/year for primary and 7.2%/year for secondary prevention devices. Commonly used risk factors were equally prevalent among patients who received appropriate therapy and those who did not; furthermore, the likelihood of receiving appropriate therapy in the presence of each risk factor was similar (Figure). Inappropriate ICD therapy occurred in 27 patients (3.8%/year). Conclusions: ICDs provide clear benefit to patients who experience life-threatening arrhythmias, particularly those being treated for secondary prevention. However, the appropriate therapy rate for primary prevention was lower than previously reported, and no single risk factor appeared to have stronger association with appropriate ICD therapy than others.

Download Full-text

Hypertrophic cardiomyopathy: prevention of sudden cardiac death

10.1093/med/9780198784906.003.0354 ◽

2018 ◽

Author(s):

Constantinos O’Mahony

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Risk Stratification ◽

Cardiac Death ◽

Clinical Care ◽

Psychological Impact ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Icd Implantation ◽

European Society Of Cardiology

Sudden cardiac death (SCD) secondary to ventricular arrhythmias is the most common mode of death in hypertrophic cardiomyopathy (HCM) and can be effectively prevented with an implantable cardioverter defibrillator (ICD). The risk of SCD in HCM relates to the severity of the phenotype and regular risk stratification is an integral part of routine clinical care. For the primary prevention of SCD, risk stratification involves the assessment of seven readily available clinical parameters (age, maximal left ventricular wall thickness, left atrial diameter, left ventricular outflow tract gradient, non-sustained ventricular tachycardia, unexplained syncope, and family history of SCD) which are used to estimate the risk of SCD within 5 years of clinical evaluation using a statistical risk prediction model (HCM Risk-SCD). The 2014 European Society of Cardiology Guidelines provide a framework to aid clinical decisions and consider patients with a 5-year risk of SCD of less than 4% as low risk and recommend regular assessment while those with a risk of 6% or higher should be considered for an ICD. In patients with an intermediate risk (4% to <6%) ICD implantation may also be considered after taking into account age, co-morbid conditions, socioeconomic factors, and the psychological impact of therapy. Survivors of ventricular fibrillation arrest should receive an ICD for secondary prevention unless their life expectancy is less than 1 year. Following device implantation, patients should be followed up for device- and disease-related complications, particularly heart failure and cerebrovascular disease.

Download Full-text

Hypertrophic cardiomyopathy: prevention of sudden cardiac death

ESC CardioMed ◽

10.1093/med/9780198784906.003.0354_update_001 ◽

2018 ◽

pp. 1462-1466

Author(s):

Constantinos O’Mahony

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Risk Stratification ◽

Cardiac Death ◽

Clinical Care ◽

Psychological Impact ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Icd Implantation ◽

European Society Of Cardiology

Download Full-text

Sudden cardiac death in asymptomatic patients with aortic stenosis

Heart ◽

10.1136/heartjnl-2019-316493 ◽

2020 ◽

Vol 106 (21) ◽

pp. 1646-1650 ◽

Cited By ~ 2

Author(s):

Jan Minners ◽

Anne Rossebo ◽

John B Chambers ◽

Christa Gohlke-Baerwolf ◽

Franz-Josef Neumann ◽

...

Keyword(s):

Risk Factors ◽

Sudden Cardiac Death ◽

Aortic Stenosis ◽

Cardiac Death ◽

Event Rate ◽

Left Ventricular ◽

Severe Stenosis ◽

Asymptomatic Patients ◽

Jet Velocity

ObjectiveWe retrospectively analysed outcome data from the Simvastatin and Ezetimibe in Aortic Stenosis (SEAS) study to assess the incidence and potential risk factors of sudden cardiac death (SCD) in this prospectively followed cohort of asymptomatic patients with aortic stenosis (AS).MethodsOf the 1873 patients included in SEAS, 1849 (99%) with mild to moderate AS (jet velocity 2.5–4.0 m/s at baseline) and available clinical, echocardiographic and follow-up data were analysed. Patients undergoing aortic valve replacement were censored at the time of operation.ResultsDuring an overall follow-up of 46.1±14.6 months, SCD occurred in 27 asymptomatic patients (1.5%) after a mean of 28.3±16.6 months. The annualised event rate was 0.39%/year. The last follow-up echocardiography prior to the event showed mild to moderate stenosis in 22 and severe stenosis (jet velocity >4 m/s) in 5 victims of SCD. The annualised event rate after the diagnosis of severe stenosis was 0.60%/year compared with 0.46%/year in patients who did not progress to severe stenosis (p=0.79). Patients with SCD were older (p=0.01), had a higher left ventricular mass index (LVMI, p=0.001) and had a lower body mass index (BMI, p=0.02) compared with patients surviving follow-up. Cox regression analysis identified age (HR 1.06, 95% CI 1.01 to 1.11 per year, p=0.02), increased LVMI (HR 1.20, 95% CI 1.10 to 1.32 per 10 g/m2, p<0.001) and lower BMI (HR 0.87, 95% CI 0.79 to 0.97 per kg/m2, p=0.01) as independent risk factors of SCD.ConclusionSCD in patients with asymptomatic mild to moderate AS is rare and strongly related to left ventricular hypertrophy but not stenosis severity.

Download Full-text

Prevention of sudden cardiac death by the implantable cardioverter-defibrillator in adults with inherited heart disease. long term follow-up

EP Europace ◽

10.1093/europace/euab116.410 ◽

2021 ◽

Vol 23 (Supplement_3) ◽

Author(s):

M Herruzo Rojas ◽

A Gomez Lopez ◽

MA Martin Toro ◽

PJ Gonzalez Perez ◽

FJ Morales Ponce

Keyword(s):

Heart Disease ◽

Genetic Testing ◽

Hypertrophic Cardiomyopathy ◽

Primary Prevention ◽

High Risk ◽

Sudden Cardiac Death ◽

Cardiac Death ◽

Supraventricular Arrhythmias ◽

Icd Implantation

Abstract Funding Acknowledgements Type of funding sources: None. BACKGROUND Inherited heart diseases include all inheritable channelopathies or cardiomyopathies. Sudden cardiac death (SCD) might be the first and last clinical presentation. ICD implantation is established as a therapeutic tool according to risk criteria. OBJETIVES The aim of this study is to analyze the population characteristics in ICD/ CRT-ICD recipients in patients with inherited heart disease and establish possible risk predictor factors of arrhytmogenic events during follow-up. MÉTHODS: This is a prospective single-center registry. We included all patients from January1, 2012, to December 31, 2020 who subsequently underwent ICD/ ICD-CRT implantation. RESULTS A total of 172 patients were included with a mean age of 60.47 ± 13,1 years and a mean follow-up duration of 49.71 ± 41.8 months. The most frequent underlying cardiac condition was dilated cardiomyopathy (58.1%), followed by hypertrophic cardiomyopathy (15.7%), arrythmogenic cardiomyopathy (2.3%), long QT syndrome (1.7%), Brugada syndrome (1.2%), catecholaminergic (2.9%) , idiopathic (7%) and others (11%). 89 ICDs (51.7%) and 83 ICD-CRTs (48.2%) were implanted for primary prevention of SCD. During follow-up, 13.4% of the patients experienced arrhythmogenic events. Only 4.1% of the patients experienced inappropriate shock, secondary to supraventricular arrhythmias all of them. Genetic testing was done in only 14.1% of our patients. In the 27 cases of hypertrophic cardiomyopathy, 25 (92.6%) underwent ICD implantation for primary prevention. 14 patients (51.9%) had family history of SCD, 8 (29.6%) had unexplained syncope and 16 (59.3%) had ventricular tachycardia in the Holter monitoring. The mean left atrial size was 44.15 ± 7.2mm, mean maximum LV-wall thickness 22.85 ± 5,25mm, and a mean LVOT gradient of 30.56 ± 31.3mmHg, with a mean actual HCM SCD-score of SCD over 5 years of 5.84 ± 3.53%. According to traditional parameters, 100% of the HCM sample meet criteria for ICD implantation in primary prevention compared to 66.7% according to the new score. During follow-up 2 patients (7.4% ) experienced arrhythmogenic events, and 3 patients (11.1%) had inappropriate shocks secondary to supraventricular arrhythmias. Genetic testing was done in 22.2% of the patients and was positive for a known pathogenic mutation in half of the cases. None of the parameters evaluated in the current score, a high risk of SCD according to it, having old high risk criteria or implanted of ICD in secondary prevention were related in our study with more arrhytmogenic events. CONCLUSIONS ICD implatation for primary prevention is the main indication in our population. Either classic factors or the new HCM SCD-score were no related in our study with the presence of arrhytmogenic events during follow-up.

Download Full-text

Evaluation of ventricular tachycardia inducibility after implementation of a standardized programmed ventricular stimulation protocol in patients with repaired Tetralogy of Fallot

European Heart Journal ◽

10.1093/eurheartj/ehab724.0330 ◽

2021 ◽

Vol 42 (Supplement_1) ◽

Author(s):

L Herrador Galindo ◽

J Francisco Pascual ◽

A Santos Ortega ◽

J Perez Rodon ◽

B Benito ◽

...

Keyword(s):

Risk Factors ◽

Ventricular Tachycardia ◽

Sudden Cardiac Death ◽

Tetralogy Of Fallot ◽

Cardiac Death ◽

Clinical Information ◽

Icd Implantation ◽

Repaired Tetralogy Of Fallot ◽

Drive Trains

Abstract Introduction The electrophysiologic (EP) evaluation with programmed electrical stimulation (PVS) is generally recommended in patients with repaired Tetralogy of Fallot and additional risk factors for sudden cardiac death. Nevertheless, different PVS protocols have been described. The aim of our study was to evaluate the differences in ventricular tachycardia (VT) inducibility of patients with TOF after the implementation of a standard PVS protocol in the EP laboratory of a Congenital Heart Disease reference center. Methods All patients with repaired TOF who underwent an EP study with PVS between January 2001 and October 2020 were included. The new standardized PVS protocol was performed in 2 ventricular sites (apex and outflow tract) with 3 drive trains (cycle lengths 400, 500 and 600ms) and up to 3 extrastimuli. In absence of VT induction, the protocol was repeated under isoprenaline infusion. This new protocol was implemented since January 2012. Non protocolized PVS studies before 2012 were defined as “Non-standardized”. Baseline clinical information about symptoms and previous arrhythmias was recorded as well as electrocardiogram, echocardiogram and cardiac MRI parameters. Finally, the follow-up events (ICD implantation, sudden cardiac death, global mortality, arrythmias and ICD therapies) were also retrospective recorded. Results A total of 154 EP studies with PVS were performed in 128 patients with repaired TOF. 31 of them were performed before the 1st January 2012 (non-standardized PVS) and 112 were performed with the new standardized protocol. The median follow-up was 6,5 years. Both groups had similar baseline characteristics except LVEF and RVEF, that were lower in the “Non-standardized PVS” group. There were no differences between the ventricular tachycardia inducibility of both protocols (22,3% vs 33,3%; p=0,162). The risk factors for VT inducibility were the QRS length (184,46ms vs 169,34 ms; p=0,038), the RVEF (36,25% vs 43,79; p=0,0007), the presence of ventricular ectopia (VE) (38,5% vs 20,0%; p=0,024) and previous VT (35,9% vs 13,9%; p=0,003). VT induction during EP study was related with ICD implantation (71,8% vs 21,7%, p≤0,001), VT (30,8% vs 20%, p<0,001) and all kind of arrythmias (VT, non-sustained VT, VE and auricular flutter) (41% vs 21,7%, p=0,005) during follow-up. A total of 6 deaths (1 in the group with induced VT and 5 in the group with non-induced VT) were recorded. Conclusions The implementation of a standardized and more complete PVS protocol in patients with repaired TOF has not shown differences in the experience of our center. The risk factors for VT inducibility were the QRS length, the RVEF, the presence of ventricular ectopia and previous VT, which have also been reported as risk factors for sudden cardiac death in previous studies. The presence of VT induction entailed more ICD implantation and more arrythmias at follow-up. FUNDunding Acknowledgement Type of funding sources: None.

Download Full-text

Is alcohol septal ablation in women performed too late?

European Heart Journal ◽

10.1093/ehjci/ehaa946.2077 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

A Batzner ◽

D Aicha ◽

H Seggewiss

Keyword(s):

Hospital Stay ◽

Pacemaker Implantation ◽

Posterior Wall ◽

Hypertrophic Obstructive Cardiomyopathy ◽

Left Ventricular ◽

Icd Implantation ◽

Alcohol Septal Ablation ◽

Significant Difference ◽

Surgical Myectomy

Abstract Introduction Alcohol septal ablation (PTSMA) was introduced as interventional alternative to surgical myectomy for symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM) 25 years ago. As gender differences in diagnosis and treatment of HOCM are still unclear we analyzed baseline characteristics and results of PTSMA in a large single center cohort with respect to gender. Methods and results Between 05/2000 and 06/2017 first PTSMA in our center was performed in 952 patients with symptomatic HOCM. We treated less 388 (40.8%) women and 564 (59.2%) men. All patients underwent clinical follow-up. At the time of the intervention women were older (61.2±14.9 vs. 51.9±13.7 years; p<0.0001) and suffered more often from NYHA grade III/IV dyspnea (80.9% vs. 68.1%; p<0.0001), whereas angina pectoris was comparable in women (62.4%) and men (59.9%). Echocardiographic baseline gradients were comparable in women (rest 65.0±38.1 mmHg and Valsalva 106.2±45.7 mmHg) and men (rest 63.1±38.3 mmHg and Valsalva 103.6±42.8 mmHg). But, women had smaller diameters of the left atrium (44.3±6.9 vs. 47.2±6.5 mm; p<0001), maximal septum thickness (20.4±3.9 vs. 21.4±4.5 mm; p<0.01), and maximal thickness of the left ventricular posterior wall (12.7±2.8 vs. 13.5±2.9 mm; p<0.0001). In women, more septal branches (1.3±0.6 vs. 1.2±0.5; p<0.05) had to be tested to identify the target septal branch. The amount of injected alcohol was comparable (2.0±0, 4 in women vs. 2.1±0.4 ml in men). The maximum CK increase was lower in women (826.0±489.6 vs. 903.4±543.0 U / l; p<0.05). During hospital stay one woman and one man died, each (n.s.). The frequency of total AV blocks in the cathlab showed no significant difference between women (41.5%) and men (38.3%). Furthermore, the rate of permanent pacemaker implantation during hospital stay did not differ (12.1% in women vs. 9.4% in men). Follow-up periods of all patients showed no significant difference between women (5.7±4.9 years) and men (6.2±5.0 years). Overall, 37 (9.5%) women died during this period compared to only 33 (5.9%) men (p<0.05). But, cardiovascular causes of death were not significantly different between women (2.8%) and men (1.6%). Furthermore, the rates of surgical myectomy after failed PTSMA (1.3% in women vs. 2.3% in men), ICD implantation for primary prevention of sudden cardiac death according to current guidelines (4.1% in women vs. 5.9% in men) or pacemaker implantation (3.6% in women vs. 2.0% in men) showed no significant differences. Summary PTSMA in women with HOCM was performed at more advanced age with more pronounced symptoms compared to men. While there were no differences in acute outcomes, overall long-term mortality was higher in women without differences in cardiovascular mortality. Therefore, women may require more intensive diagnostic approaches in order not to miss the correct time for gradient reduction treatment. Funding Acknowledgement Type of funding source: None

Download Full-text

Abstract 14760: Myocardial Scarring by Magnetic Resonance Predicts Sudden Cardiac Death in Pediatric Patients With Hypertrophic Cardiomyopathy

Circulation ◽

10.1161/circ.142.suppl_3.14760 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Lars Grosse-Wortmann ◽

Laurine van der Wal ◽

Aswathy Vaikom House ◽

Lee Benson ◽

Raymond Chan

Keyword(s):

Risk Factors ◽

Risk Factor ◽

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Cardiac Death ◽

Pediatric Patients ◽

Risk Markers ◽

C Statistic ◽

Increased Risk ◽

Traditional Risk Factors

Introduction: Cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) has been shown to be an independent predictor of sudden cardiac death (SCD) in adults with hypertrophic cardiomyopathy (HCM). The clinical significance of LGE in pediatric HCM patients is unknown. Hypothesis: LGE improves the SCD risk prediction in children with HCM. Methods: We retrospectively analyzed the CMR images and reviewed the outcomes pediatric HCM patients. Results: Amongst the 720 patients from 30 centers, 73% were male, with a mean age of 14.2±4.8 years. During a mean follow up of 2.6±2.7 years (range 0-14.8 years), 34 experienced an episode of SCD or equivalent. LGE (Figure 1A) was present in 34%, with a mean burden of 14±21g, or 2.5±8.2g/m2 (6.2±7.7% of LV myocardium). The presence of ≥1 adult traditional risk factor (family history of SCD, syncope, LV thickness >30mm, non-sustained ventricular tachycardia on Holter) was associated with an increased risk of SCD (HR=4.6, p<0.0001). The HCM Risk-Kids score predicted SCD (p=0.002). The presence of LGE was strongly associated with an increased risk (HR=3.8, p=0.0003), even after adjusting for traditional risk factors (HR adj =3.2, p=0.003) or the HCM Risk-Kids score (HR adj =3.5, p=0.003). Furthermore, the burden of LGE was associated with increased risk (HR=2.1/10% LGE, p<0.0001). LGE burden remained independently associated with an increased risk for SCD after adjusting for traditional risk factors (HRadj=1.5/10% LGE, p=0.04) or HCM Risk-Kids (HRadj=1.9/10% LGE, p=0.0018, Figure 1B). The addition of LGE burden improved the predictive model using traditional risk markers (C statistic 0.67 vs 0.77, p=0.003) and HCM Risk-Kids (C statistic 0.68 vs 0.74, p=0.045). Conclusions: Quantitative LGE is an independent risk factor for SCD in pediatric patients with HCM and improves the performance of traditional risk markers and the HCM Risk-Kids Score for SCD risk stratification in this population.

Download Full-text

LEFT VENTRICULAR LEAD IMPLANTATION FOR DETECTION OF VENTRICULAR ARRHYTHMIAS IN PATIENT WITH IMPLANTABLE CARDIOVERTER DEFIBRILLATOR AND LOW R WAVE IN RIGHT VENTRICLE

American Journal of Cardiology Research and Reviews ◽

10.28933/ajcrar-2021-10-1005 ◽

2021 ◽

pp. 17

Author(s):

Keyword(s):

Sudden Cardiac Death ◽

Right Ventricle ◽

Implantable Cardioverter Defibrillator ◽

Cardiac Death ◽

Ventricular Arrhythmias ◽

Systolic Function ◽

Left Ventricular ◽

Icd Implantation ◽

Cardioverter Defibrillator ◽

Ventricular Lead

Dilated cardiomyopathy (DCM) is a disease characterised as left ventricular (LV) or biventricular dilatation with impaired systolic function. Regardless of underlying cause patients with DCM have a propensity to ventricular arrhythmias and sudden cardiac death. Implantable Cardioverter Defibrillator (ICD) implantation for these patients results in significant reduction of sudden cardiac death [1-3]. ICD devices may be limited by right ventricle (RV) sensing dysfunction with low RV sensing amplitude. We present a clinical case of patient with DCM, implanted ICD and low R wave sensing on RV lead.

Download Full-text