2397Lesion-specific risk for sudden cardiac death or life-threatening ventricular arrhythmias in adult congenital heart disease

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
P Gallego Garcia De Vinuesa ◽  
A E Gonzalez Garcia ◽  
P Avila ◽  
A Alonso ◽  
D Garcia Hamilton ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Incidence Rate ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Adult Congenital Heart Disease ◽  
Low Risk ◽  
Discriminative Ability ◽  
Specific Risk ◽  
Adult Congenital ◽  
Life Threatening

Abstract Background Risk models for primary prevention strategies in adult congenital heart disease (ACHD) must incorporate the heterogeneous risk for sudden cardiac death (SCD) and life-threatening ventricular arrhythmias (LTVA) as stratified by underlying lesion. Objectives To determine lesion-specific risk for SCD and LTVA in ACHD. Methods We analyzed 3311 ACHD patients (50% males) prospectively followed-up for 37510 person/years. SCD cases were confirmed by means of the Spanish National Death Registry. In addition, we identified all cases of resuscitated cardiac arrest or ventricular tachycardia requiring cardioversion. According to the incidence rate of the composite end-point of SCD and LTVA, lesions were stratified into four groups of risk. Cumulative freedom from SCD or LTVA in patients at high, moderate, low and very low risk were compared by using Cox regression model with left truncation. The c-index of this lesion-specific risk stratification was calculated by using the β-coefficients. The discriminative ability of this lesion-specific risk stratification was also tested in an external cohort of 203 SCD-LTVA cases and 2287 controls from 20 different centers. Results 71 patients experienced an event (53 SCD, 18 LTVA). Patients at highest risk (incidence rate >1%) were those with Rastelli procedure, severe coronary abnormalities, complex Fallot and cyanotic patients, either Eisenmenger or non-Eisenmenger; at moderate risk (incidence rate 0.25–1.0%) non-complex Fallot, Mustard/Senning repair, Fontan procedures and congenitally corrected transposition; at low risk (incidence rate 0.1–0.25%) Ebstein anomaly and left heart lesions; and at very low risk (incidence rate <0.1%) left-to-right shunts and right ventricular outflow lesions. The discriminative ability in a multicenter external cohort was excellent (c-index ranged from 0.748 to 0.819 by center). Lesion-specific risk and C-index Conclusions A lesion-specific risk stratification based on the incidence rate of SCD and LTVA was performed and validated. This approach could result in a more individualized risk assessment. Acknowledgement/Funding Instituto de Salud Carlos III, Ministerio de Economía y Competividad, Spain (Exp PI14/02099 and PI17/01327) and co-financed by FEDER

Abstract 17194: Incremental Value of JTc Interval in Improving Risk Stratification for Life-Threatening Ventricular Arrhythmias in Patients Considered Low Risk by LVEF

Circulation ◽  
2018 ◽  
Vol 138 (Suppl_1) ◽  
Author(s):  
Mohsin Khan ◽  
Susan Olet ◽  
Mohammad E Mortada ◽  
Firas Zahwe ◽  
Jodi Zilinski ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Risk Stratification ◽  
Ventricular Arrhythmias ◽  
Low Risk ◽  
Operating Characteristics ◽  
Icd Implantation ◽  
P Values ◽  
Net Reclassification Improvement ◽  
Life Threatening

Introduction: ICD implantation is recommended in patients with LVEF<35%, while those with LVEF between 35 to 40% are not considered at high risk for primary prevention ICD implantation. A subset of these patients develops life threatening ventricular arrhythmias (VA) and improvement in risk stratification may help identify and implement life-saving intervention. Hypothesis: Prolonged repolarization is a marker of electrical instability and JTc interval on ECG could provide prognostic information in patients with LVEF 35-40% incremental to that from LVEF. Methods: Patients ≥18 yr with no history of VA and an ECG and echocardiogram obtained at initial encounter between 11/2011 to 12/2016 with long-term follow-up were identified. The incremental predictive ability of JTc interval on improvement in risk stratification for VA was determined by receiver operating characteristics (ROC) curve, integrated discrimination improvement (IDA) and net reclassification improvement (NRI) analysis. All tests were performed at a 5% level of significance. Results: Out of 29,700 pts that met inclusion criteria, 1,102 (3.7%) had LVEF 35-40% (mean age 70.5±14.6 yrs, 49% males, CAD 67%) and 24,894 (84%) LVEF >40% (65.9±16.3 yrs , 61.8% M). Over the mean follow-up of 4.6±4.2 years, the incidence of VT/VF/cardiac arrest was 16.1% in patients with LVEF 35-40% compared to 4.1% with LVEF >40%. For every 50 ms increase in JTc interval above 300 ms, the risk for arrhythmic event in LVEF 35-40% increased two-fold (Odds Ratio=1.83 (95 % CI 1.72-1.94, P=0.013). Incorporation of JTc to LVEF improved the C statistics (95% Confidence Limit) in the model with only LVEF from 0.56 (0.54-0.57) to 0.72 (0.70-0.73) for the model combining LVEF and JTc. In addition, NRI was estimated at 0.57, which was statistically significant with p values <0.001 while IDI was estimated as 0.015 with p values <0.001 for the model incorporating JTc to LVEF. Conclusions: In patients with LVEF 35-40% considered low risk for life threatening VA by EF, incorporating JTc interval information improved risk stratification and identified those who subsequently developed VT/VF or cardiac arrest and thus identifies a subgroup that can benefit from prophylactic ICD implantation.

scholarly journals Sudden cardiac death in adult congenital heart disease: can we predict the unpredictable?

2013 ◽  
Vol 34 (suppl 1) ◽  
pp. P2098-P2098
Author(s):  
Z. Koyak ◽  
L. Harris ◽  
J. R. De Groot ◽  
A. H. Zwinderman ◽  
C. K. Silversides ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Adult Congenital

scholarly journals The Necessity of an ICD-Therapy in Patients with Indications for Primary Prevention of Sudden Cardiac Death. One Center Experience

Kardiologiia ◽  
2021 ◽  
Vol 61 (4) ◽  
pp. 24-31
Author(s):  
A. S. Postol ◽  
N. M. Neminushchiy ◽  
G. N. Antipov ◽  
A. V. Ivanchenko ◽  
V. V. Lyashenko ◽  
...  
Keyword(s):  
Primary Prevention ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Icd Therapy ◽  
Number Of Patients ◽  
Life Threatening ◽  
Group 2 ◽  
Cardioverter Defibrillators ◽  
Group 1

Aim      Analysis of responses of cardioverter-defibrillators implanted in patients with cardiomyopathies (CMPs) of various origins and a high risk of sudden cardiac death (SCD) to assess the effectiveness of a modern strategy for primary prevention of SCD.Material and methods  In the Federal Center for High Medical Technologies in Kaliningrad from 2014 through 2018, implantable cardioverter-defibrillators (ICD) and cardiac resynchronization therapy defibrillators (CRT-D) were installed in 165 patients. Major indications for device implantation in these patients included left ventricular (LV) systolic dysfunction with ejection fraction (EF) ≤35 %; chronic heart failure (CHF) consistent with the New York Heart Association (NYHA) functional class (FC) II-III (IV for CRT-D) without previous episodes of life-threatening ventricular arrhythmias, circulatory arrest and resuscitation, which was consistent with the current international strategy for primary prevention of SCD. The study patients were divided into two groups based on the CMP origin; group 1 included 101 (61.2 %) patients with CMP of ischemic origin (ICMP) and group 2 consisted of 64 (38.8 %) patients with CMP of non-ischemic origin (NCMP). Information about arrhythmic episodes and device activation was retrieved from the device electronic memory during visits of patients to the clinic and was also transmitted to the clinic by a remote monitoring system. This information was studied and evaluated for the validity and effectiveness of the device triggering. If necessary, the parameters of detection and treatment were adjusted taking into account the obtained information. Information was analyzed and statistically processed with the SPSS Statistics 20.0 software.Results The patients were followed up for 28.3 ± 15.6 months, during which the devices delivered therapy to 55 (33.3%) patients of the entire group. In the ICMP group, the devices were activated in 44 (26.7 %) patients and in the NCMP group, the devices were activated in 11 (6.7 %) patients. In group 1 (ICMP), appropriate triggering was observed in 33 (20.0%) patients and inappropriate triggering was observed in 11 (6.7%) patients. In group 2 (NCMP), appropriate triggering was observed in 2 (1.2 %) patients and inappropriate triggering was observed in 9 (5.5 %) patients. The main cause of inappropriate triggering was atrial fibrillation (AF). 17 (10.3 %) patients with ICMP had sustained ventricular tachycardia (VT), which did not reach the detection frequency for ICD therapy; these VTs were only detected by devices and terminated spontaneously. Intragroup differences in the number of patients who received an appropriate treatment were statistically significant: 33 (32.6 %) in the ICMP group vs. 2 (3.1 %) in the NCMP group (р<0.006). Differences in the number of patients who received an inappropriate treatment were not statistically significant although their number was greater in the NCMP group than in the ICMP group (9 (14.1 %) vs. 11 (10.9 %), р>0.05).Conclusion      A higher requirement for the ICD treatment was revealed in patients with ICMP compared to patients with NCMP. The low demand for the ICD treatment in patients with NCMP and the more frequent inappropriate actuation of the devices in this patient group due to AF allow a conclusion that the criteria for primary prevention of SCD with ICD (LV EF ≤35% and clinically significant CHF) are not equally effective indications for ICD implantation in patients with ICMP and NCMP. It can be assumed that life-threatening ventricular arrhythmias are evident in patients with NCMP before the development of hemodynamically significant LV dysfunction and CHF, which warrants further research in this direction. 

scholarly journals Coronavirus disease 2019 in adults with congenital heart disease: a position paper from the ESC working group of adult congenital heart disease, and the International Society for Adult Congenital Heart Disease

2020 ◽  
Author(s):  
Gerhard-Paul Diller ◽  
Michael A Gatzoulis ◽  
Craig S Broberg ◽  
Jamil Aboulhosn ◽  
Margarita Brida ◽  
...  
Keyword(s):  
Cardiovascular Disease ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Risk Stratification ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Young Patients ◽  
Psychosocial Health ◽  
Current Data ◽  
Adult Congenital

Abstract We are witnessing an unparalleled pandemic caused by the novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) associated with coronavirus disease 2019 (COVID-19). Current data show that SARS-CoV-2 results in mild flu-like symptoms in the majority of healthy and young patients affected. Nevertheless, the severity of COVID-19 respiratory syndrome and the risk of adverse or catastrophic outcomes are increased in patients with pre-existing cardiovascular disease. Patients with adult congenital heart disease (ACHD)—by definition—have underlying cardiovascular disease. Many patients with ACHD are also afflicted with residual haemodynamic lesions such as valve dysfunction, diminished ventricular function, arrhythmias or cyanosis, have extracardiac comorbidities, and face additional challenges regarding pregnancy. Currently, there are emerging data of the effect of COVID-19 on ACHD patients, but many aspects, especially risk stratification and treatment considerations, remain unclear. In this article, we aim to discuss the broad impact of COVID-19 on ACHD patients, focusing specifically on pathophysiology, risk stratification for work, self-isolation, hospitalization, impact on pregnancy, psychosocial health, and longer-term implications for the provision of ACHD care.

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