Predicting sudden cardiac death in adults with congenital heart disease. Issue 1 (16th June 2020)

• Record Type: Journal Article
• Authors: Oliver, Jose M; Gallego, Pastora; Gonzalez, Ana Elvira; Avila, Pablo; Alonso, Andres; Garcia-Hamilton, Diego; Peinado, Rafael; Dos-Subirà, Laura; Pijuan-Domenech, Antonia; Rueda, Joaquín; Rodriguez-Puras, Maria-Jose; Garcia-Orta, Rocio; Martínez-Quintana, Efrén; Datino, Tomas; Fernandez-Aviles, Francisco; Bermejo, Javier
• Other Names: author non-byline.; Oliver Jose M author non-byline.; Gallego Pastora author non-byline.; Gonzalez-Garcia Ana author non-byline.; Avila Pablo author non-byline.; Alonso Andres author non-byline.; Garcia-Hamilton Diego author non-byline.; Peinado Rafael author non-byline.; Dos Laura author non-byline.; Pijuan-Domenech Antonia author non-byline.; Rueda Joaquín author non-byline.; Rodriguez-Puras Maria-Jose author non-byline.; Garcia-Orta Rocio author non-byline.; Martinez-Quintana Efren author non-byline.; Prieto Raquel author non-byline.; Datino Tomas author non-byline.; Ruiz-Cantador Jose author non-byline.; Bouzas-Zubeldia Beatriz author non-byline.; Martinez-Bendayan Isaac author non-byline.; Gordon Blanca author non-byline.; Cantalapiedra Javier author non-byline.; Gonzalez-Fernandez Víctor author non-byline.; Olivella Aleix author non-byline.; Buendia Francisco author non-byline.; Moreno Eduardo author non-byline.; Rodríguez-Hernandez Juan L author non-byline.; Robledo-Carmona Juan author non-byline.; Lopez Marta author non-byline.; García-Honrubia Antonio author non-byline.; Bastos-Fernandez María author non-byline.; Lacuey Gemma author non-byline.; Nuria Mieza MD author non-byline.; Valenzuela Luis F author non-byline.; Cano-Nieto Joaquin author non-byline.; de-la-Puerta Iris author non-byline.; Noris Marta author non-byline.; Garcia-Arandas Beatriz author non-byline.; Montserrat Silvia author non-byline.; Sánchez Inmaculada author non-byline.; Bermejo Javier author non-byline.; Fernandez-Aviles Francisco author non-byline.;
• Is Part Of: Heart. Volume 107:Issue 1(2021)
• Page Start: 67
• Page End: 75
• Publication Date: 2020-06-16
• Subjects: sudden cardiac death -- cardiac arrest -- ventricular tachycardia -- congenital heart disease -- study design; Heart -- Diseases -- Treatment -- Periodicals; Cardiology -- Periodicals; 616.12
• DOI: 10.1136/heartjnl-2020-316791
• Languages: English
• ISSNs: 1355-6037

Abstract

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 (<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.