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Predicting sudden cardiac death in adults with congenital heart disease
  1. Jose M Oliver1,2,
  2. Pastora Gallego3,
  3. Ana Elvira Gonzalez2,
  4. Pablo Avila1,
  5. Andres Alonso1,
  6. Diego Garcia-Hamilton2,
  7. Rafael Peinado2,
  8. Laura Dos-Subirà4,
  9. Antonia Pijuan-Domenech4,
  10. Joaquín Rueda5,
  11. Maria-Jose Rodriguez-Puras3,
  12. Rocio Garcia-Orta6,
  13. Efrén Martínez-Quintana7,
  14. Tomas Datino1,
  15. Francisco Fernandez-Aviles1,
  16. Javier Bermejo1
  17. On behalf of the Spanish ACHD Network
    1. 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
    2. 2Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitario La Paz, Madrid, Spain
    3. 3Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitario Virgen del Rocio, Instituto de BioMedicina de Sevilla (IBIS) and CIBERCV, Sevilla, Spain
    4. 4Unitat Integrada de Cardiopaties Congènites de l'Adolescent i de l’Adult Vall d'Hebron-Sant Pau, Department of Cardiology, Vall d'Hebron University Hospital and CIBERCV, Barcelona, Spain
    5. 5Adult congenital heart disease unit, Department of Cardiology, Hospital Universitari i Politecnic La Fe, Valencia, Spain
    6. 6Adult Congenital Heart Disease Unit, , Hospitales Virgen de las Nieves y Clínico San Cecilio, Granada, Spain
    7. 7Adult Congenital Heart Disease Unit, Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria, Spain
    1. Correspondence to Dr Pastora Gallego, Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitario Virgen del Rocio, Sevilla 41013, Spain; antoniap.gallego.sspa{at}juntadeandalucia.es

    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.

    • sudden cardiac death
    • cardiac arrest
    • ventricular tachycardia
    • congenital heart disease
    • study design
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    Footnotes

    • Twitter @apgalgar

    • Collaborators The Spanish ACHD Network investigators: Jose M Oliver, PhD (Department of Cardiology, CIBERCV, Hospital Universitario La Paz, Madrid; Department of Cardiology, CIBERCV, Hospital General Universitario Gregorio Marañón, Madrid); Pastora Gallego, PhD (Department of Cardiology, Hospital Universitario Virgen del Rocío, Instituto de Biomedicina, CIBERCV, Sevilla); Ana Gonzalez-Garcia, MD (Department of Cardiology, CIBERCV, Hospital Universitario La Paz, Madrid); Pablo Avila, MD (Department of Cardiology, CIBERCV, Hospital General Universitario Gregorio Marañón, Madrid); Andres Alonso, MD (Department of Cardiology, CIBERCV, Hospital General Universitario Gregorio Marañón, Madrid); Diego Garcia-Hamilton, MD (Department of Cardiology, CIBERCV, Hospital Universitario La Paz, Madrid); Rafael Peinado, PhD (Department of Cardiology, CIBERCV, Hospital Universitario La Paz, Madrid); Laura Dos, PhD (Department of Cardiology, Unitat Integrada de Cardiopaties Congènites de l'Adolescent i de l’Adult Vall d'Hebron-Sant Pau, CIBERCV, Barcelona); Antonia Pijuan-Domenech, PhD (Department of Cardiology, Unitat Integrada de Cardiopaties Congènites de l'Adolescent i de l’Adult Vall d'Hebron-Sant Pau, CIBERCV, Barcelona); Joaquín Rueda, PhD (Department of Cardiology, CIBERCV, Hospital Universitari i Politecnic LA FE, Valencia); Maria-Jose Rodriguez-Puras PhD (Department of Cardiology, Hospital Universitario Virgen del Rocío, Instituto de Biomedicina, CIBERCV, Sevilla); Rocio Garcia-Orta, PhD (Department of Cardiology, Hospital Virgen de las Nieves y Hospital San Cecilio, Granada); Efren Martinez-Quintana, PhD (Department of Cardiology, Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria); Raquel Prieto, MD (Department of Cardiology, CIBERCV, Hospital General Universitario Gregorio Marañón, Madrid); Tomas Datino, PhD (Department of Cardiology, CIBERCV, Hospital General Universitario Gregorio Marañón, Madrid); Jose Ruiz-Cantador, MD (Department of Cardiology, CIBERCV, Hospital Universitario La Paz, Madrid); Beatriz Bouzas-Zubeldia, MD (Department of Cardiology, Complejo Hospitalario Universitario de A Coruña, A Coruña); Isaac Martinez-Bendayan, MD (Department of Cardiology, Complejo Hospitalario Universitario de A Coruña, A Coruña); Blanca Gordon, MD (Department of Cardiology, Unitat Integrada de Cardiopaties Congènites de l'Adolescent i de l’Adult Vall d'Hebron-Sant Pau, CIBERCV, Barcelona); Javier Cantalapiedra, MD (Department of Cardiology, Unitat Integrada de Cardiopaties Congènites de l'Adolescent i de l’Adult Vall d'Hebron-Sant Pau, CIBERCV, Barcelona); Víctor Gonzalez-Fernandez, MD (Department of Cardiology, Unitat Integrada de Cardiopaties Congènites de l'Adolescent i de l’Adult Vall d'Hebron-Sant Pau, CIBERCV, Barcelona); Aleix Olivella, MD (Department of Cardiology, Unitat Integrada de Cardiopaties Congènites de l'Adolescent i de l’Adult Vall d'Hebron-Sant Pau, CIBERCV, Barcelona); Francisco Buendia, MD (Department of Cardiology, CIBERCV, Hospital Universitari i Politecnic LA FE, Valencia); Eduardo Moreno, MD (Department of Cardiology, Hospital Virgen de las Nieves y Hospital San Cecilio, Granada); Juan L Rodríguez-Hernandez, MD (Department of Cardiology, Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria); Juan Robledo-Carmona, MD (Department of Cardiology, Hospital Virgen de la Victoria, Málaga); Marta Lopez, MD (Department of Cardiology, Hospital Universitario Miguel Servet, Zaragoza); Antonio García-Honrubia, MD (Department of Cardiology, Hospital General Universitario de Elche); María Bastos-Fernandez, MD (Department of Cardiology, Complejo Hospitalario de Pontevedra); Gemma Lacuey, MD (Department of Cardiology, Complejo Hospitalario de Navarra, Pamplona); Nuria Mieza, MD (Department of Cardiology, Hospital Universitario Puerta del Mar, Cádiz); Luis F Valenzuela, MD (Department of Cardiology, Hospital Universitario Virgen del Rocío, Instituto de Biomedicina, CIBERCV, Sevilla); Joaquin Cano-Nieto, MD (Department of Cardiology, HRU Carlos Haya, Málaga); Iris de-la-Puerta, MD (Department of Cardiology, Hospital Universitari Arnau de Vilanova de Lleida); Marta Noris, MD (Department of Cardiology, Hospital Universitario Son Espases, Palma de Mallorca); Beatriz Garcia-Aranda, MD (Department of Cardiology, Hospital Universitario 12 de octubre, Madrid); Silvia Montserrat, MD (Department of Cardiology, CIBERCV, Hospital Clinic, Barcelona); Inmaculada Sánchez, MD (Department of Pediatric Cardiology, Hospital Universitario Ramón y Cajal, Madrid, Spain); Javier Bermejo, PhD (Department of Cardiology, CIBERCV, Hospital General Universitario Gregorio Marañón, Madrid); and Francisco Fernandez-Aviles, PhD (Department of Cardiology, CIBERCV, Hospital General Universitario Gregorio Marañón, Madrid).

    • Contributors JMO designed the study as the principal investigator. JMO, PG, AEG, PA, AA, DG-H, RP, LD-S, AP-D, JR, M-JR-P, RG-O and EM-Q identified the patients and collected the data. JMO and JB drafted the plans for the data analyses. JMO, PG and JB conducted data analysis and drafted the manuscript. All authors were involved in interpretation of the results and revision of the manuscript, and all approved the final version of the manuscript. JMO and PG are guarantors. The corresponding author (PG) attests that all the listed authors meet the authorship criteria and that no others meeting the criteria have been omitted.

    • Funding This study was supported by the Instituto de Salud Carlos III, Ministerio de Economía y Competividad, Spain (Exp PI14/02099 and PI17/01327) and cofinanced by FEDER (European Funds for Regional Development).

    • Competing interests None declared.

    • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

    • Patient consent for publication Not required.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Data availability statement No data are available. Data sharing: no additional data available.

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