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   <dc:title>Predicting sudden cardiac death in adults with congenital heart disease</dc:title>
   <dc:creator>Oliver, José M.</dc:creator>
   <dc:creator>Ávila, Pablo</dc:creator>
   <dc:creator>Alonso, Andrés</dc:creator>
   <dc:creator>Datino, Tomás</dc:creator>
   <dc:creator>Fernández-Avilés Díaz, Francisco Jesús</dc:creator>
   <dc:creator>Bermejo Thomas, Francisco Javier</dc:creator>
   <dcterms:abstract>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 (&lt;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.</dcterms:abstract>
   <dcterms:dateAccepted>2025-01-27T09:15:00Z</dcterms:dateAccepted>
   <dcterms:available>2025-01-27T09:15:00Z</dcterms:available>
   <dcterms:created>2025-01-27T09:15:00Z</dcterms:created>
   <dcterms:issued>2021-06-16</dcterms:issued>
   <dc:type>journal article</dc:type>
   <dc:identifier>https://hdl.handle.net/20.500.14352/116176</dc:identifier>
   <dc:identifier>1355-6037</dc:identifier>
   <dc:identifier>10.1136/HEARTJNL-2020-316791</dc:identifier>
   <dc:identifier>1468-201X</dc:identifier>
   <dc:language>eng</dc:language>
   <dc:relation>PI14/02099</dc:relation>
   <dc:relation>PI17/01327</dc:relation>
   <dc:relation>Oliver JM, Gallego P, Gonzalez AE On behalf of the Spanish ACHD Network, et alPredicting sudden cardiac death in adults with congenital heart diseaseHeart 2021;107:67-75.</dc:relation>
   <dc:rights>restricted access</dc:rights>
   <dc:publisher>British Cardiovascular Society</dc:publisher>
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