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Predicting sudden cardiac death in adults with congenital heart disease
Jose M Oliver1,2, Pastora Gallego3, Ana Elvira Gonzalez2
1Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital General Universitario Gregorio Marañon, Instituto de Investigación Sanitaria Gregorio Marañón, Facultad de Medicina, Universidad Complutense de Madrid and CIBERCV, Madrid, Spain.
Insights
A new logistic regression model accurately predicts sudden cardiac death (SCD) and sudden cardiac arrest (SCA) in adults with congenital heart disease (ACHD). This model, using lesion-specific risk and clinical factors, improves risk stratification for primary prevention strategies.
Area of Science:
- Cardiology
- Medical Informatics
Background:
- Adults with congenital heart disease (ACHD) have an increased risk of sudden cardiac death (SCD) and sudden cardiac arrest (SCA).
- Accurate risk prediction is crucial for guiding primary prevention strategies in this population.
Purpose of the Study:
- To develop, calibrate, test, and validate a logistic regression model for predicting SCD and SCA risk in ACHD patients.
- To incorporate baseline lesion-specific risk stratification and individual characteristics into the predictive model.
Main Methods:
- Combined data from a single-center cohort (3311 ACHD patients) and a multicenter case-control group (207 cases, 2287 controls).
- Derived a risk model using logistic regression on development and validation datasets.
- Stratified patients into four lesion-specific risk clusters based on cumulative incidence.
Main Results:
- Identified key predictors: lesion-specific cluster, young age, male sex, syncope, ischemic heart disease, ventricular arrhythmias, QRS duration, and ventricular dysfunction.
- The model demonstrated high accuracy in discrimination (C-index 0.91) and calibration in the validation dataset.
- Achieved a 29% increase in sensitivity for SCD/SCA prediction compared to current guidelines, with minimal specificity change.
Conclusions:
- A lesion-specific risk stratification combined with clinical variables significantly improves SCD/SCA risk prediction in ACHD.
- The developed model offers a more accurate approach to identify high-risk individuals for targeted primary prevention.
Objectives:
To develop, calibrate, test and validate a logistic regression model for accurate risk prediction of sudden cardiac death (SCD) and non-fatal sudden cardiac arrest (SCA) in adults with congenital heart disease (ACHD), based on baseline lesion-specific risk stratification and individual's characteristics, to guide primary prevention strategies.
Methods:
We combined data from a single-centre cohort of 3311 consecutive ACHD patients (50% male) at 25-year follow-up with 71 events (53 SCD and 18 non-fatal SCA) and a multicentre case-control group with 207 cases (110 SCD and 97 non-fatal SCA) and 2287 consecutive controls (50% males). Cumulative incidences of events up to 20 years for specific lesions were determined in the prospective cohort. Risk model and its 5-year risk predictions were derived by logistic regression modelling, using separate development (18 centres: 144 cases and 1501 controls) and validation (two centres: 63 cases and 786 controls) datasets.
Results:
According to the combined SCD/SCA cumulative 20 years incidence, a lesion-specific stratification into four clusters-very-low (<1%), low (1%-4%), moderate (4%-12%) and high (>12%)-was built. Multivariable predictors were lesion-specific cluster, young age, male sex, unexplained syncope, ischaemic heart disease, non-life threatening ventricular arrhythmias, QRS duration and ventricular systolic dysfunction or hypertrophy. The model very accurately discriminated (C-index 0.91; 95% CI 0.88 to 0.94) and calibrated (p=0.3 for observed vs expected proportions) in the validation dataset. Compared with current guidelines approach, sensitivity increases 29% with less than 1% change in specificity.
Conclusions:
Predicting the risk of SCD/SCA in ACHD can be significantly improved using a baseline lesion-specific stratification and simple clinical variables.
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