PREVENTION-ACHD: PRospEctiVE study on implaNTable cardioverter-defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease; Rationale and Design

J T Vehmeijer, Z Koyak, A H Zwinderman, L Harris, R Peinado, E N Oechslin, C K Silversides, B J Bouma, W Budts, I C van Gelder, J M Oliver, B J M Mulder, J R de Groot, J T Vehmeijer, Z Koyak, A H Zwinderman, L Harris, R Peinado, E N Oechslin, C K Silversides, B J Bouma, W Budts, I C van Gelder, J M Oliver, B J M Mulder, J R de Groot

Abstract

Background: Many adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). An implantable cardioverter-defibrillator (ICD) may prevent SCD, but the evidence for primary prevention indications is still unsatisfactory.

Study design: PREVENTION-ACHD is a prospective study with which we aim to prospectively validate a new risk score model for primary prevention of SCD in ACHD patients, as well as the currently existing guideline recommendations. Patients are screened using a novel risk score to predict SCD as well as current ICD indications according to an international Consensus Statement. Patients are followed up for two years. The primary endpoint is the occurrence of SCD and sustained ventricular arrhythmias. The Study was registered at ClinicalTrials.gov (NCT03957824).

Conclusion: PREVENTION-ACHD is the first prospective study on SCD in ACHD patients. In the light of a growing and aging population of patients with more severe congenital heart defects, more robust clinical evidence on primary prevention of SCD is urgently needed.

Keywords: Primary prevention; Risk score; Risk stratification; Ventricular fibrillation; Ventricular tachycardia.

Conflict of interest statement

J.T. Vehmeijer, Z. Koyak, A.H. Zwinderman, L. Harris, R. Peinado, E.N. Oechslin, C.K. Silversides, B.J. Bouma, W. Budts, I.C. van Gelder, J.M. Oliver and B.J.M. Mulder declare that they have no competing interests. J.R. de Groot receives unrestricted research grants from Medtronic, Abbott Laboratories, and Atricure and is a consultant at Daiichi Sankyo and Atricure.

Figures

Fig. 1
Fig. 1
The risk score based on clinical risk factors represents the annual risk of sudden cardiac death. For each of the following seven risk factors one point is attributed to the model: 1 Coronary artery disease, 2 Heart failure symptoms (New York Heart Association class II/III), 3 Supraventricular tachycardia, 4 Impaired systemic ventricular function (ejection fraction <40%), 5 Impaired subpulmonary ventricular function (ejection fraction <40%), 6 QRS duration >120 ms, 7 QT dispersion >70 ms (ASD atrial septal defect, SCD sudden cardiac death, TGA transposition of the great arteries, *Seven risk factors not possible for Fontan patients, as these patients do not have a subpulmonary ventricle)
Fig. 2
Fig. 2
Flow chart of patient selection and follow-up (CHD congenital heart disease, ICD implantable cardioverter-defibrillator, SCD sudden cardiac death). (Consensus: PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease [11])

References

    1. Koyak Z, Harris L, de Groot JR, et al. Sudden cardiac death in adult congenital heart disease. Circulation. 2012;126(16):1944–1954. doi: 10.1161/CIRCULATIONAHA.112.104786.
    1. Nieminen HP, Jokinen EV, Sairanen HI. Causes of late deaths after pediatric cardiac surgery: a population-based study. J Am Coll Cardiol. 2007;50(13):1263–1271. doi: 10.1016/j.jacc.2007.05.040.
    1. Oechslin EN, Harrison DA, Connelly MS, et al. Mode of death in adults with congenital heart disease. Am J Cardiol. 2000;86(10):1111–1116. doi: 10.1016/S0002-9149(00)01169-3.
    1. Verheugt CL, Uiterwaal CS, van der Velde ET, et al. Mortality in adult congenital heart disease. Eur Heart J. 2010;31(10):1220–1229. doi: 10.1093/eurheartj/ehq032.
    1. van der Linde D, Konings EE, Slager MA, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol. 2011;58(21):2241–2247. doi: 10.1016/j.jacc.2011.08.025.
    1. Marelli AJ, Ionescu-Ittu R, Mackie AS, et al. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014;130(9):749–756. doi: 10.1161/CIRCULATIONAHA.113.008396.
    1. Bardy GH, Lee KL, Mark DB, et al. Amiodarone or an implantable cardioverter-defibrillator for congestive heart failure. N Engl J Med. 2005;352(3):225–237. doi: 10.1056/NEJMoa043399.
    1. Moss AJ, Zareba W, Hall WJ, et al. Prophylactic implantation of a defibrillator in patients with myocardial infarction and reduced ejection fraction. N Engl J Med. 2002;346(12):877–883. doi: 10.1056/NEJMoa013474.
    1. Priori SG, Blomstrom-Lundqvist C, Mazzanti A, et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC) Eur Heart J. 2015;36(41):2793–2867. doi: 10.1093/eurheartj/ehv316.
    1. Vehmeijer JT, Brouwer TF, Limpens J, et al. Implantable cardioverter-defibrillators in adults with congenital heart disease: a systematic review and meta-analysis. Eur Heart J. 2016;37(18):1439–1448. doi: 10.1093/eurheartj/ehv735.
    1. Khairy P, Van Hare GF, Balaji S, et al. PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease. Heart Rhythm. 2014;11(10):e102–65. doi: 10.1016/j.hrthm.2014.05.009.
    1. Hernandez-Madrid A, Paul T, Abrams D, et al. Arrhythmias in congenital heart disease: a position paper of the European Heart Rhythm Association (EHRA), Association for European Paediatric and Congenital Cardiology (AEPC), and the European Society of Cardiology (ESC) Working Group on Grown-up Congenital heart disease, endorsed by HRS, PACES, APHRS, and SOLAECE. Europace. 2018;20(11):1719–1753. doi: 10.1093/europace/eux380.
    1. Vehmeijer JT, Koyak Z, Budts W, et al. Prevention of sudden cardiac death in adults with congenital heart disease: do the guidelines fall short? Circ Arrhythm Electrophysiol. 2017 doi: 10.1161/CIRCEP.116.005093.
    1. Moons KG, Kengne AP, Grobbee DE, et al. Risk prediction models: II. External validation, model updating, and impact assessment. Heart. 2012;98(9):691–698. doi: 10.1136/heartjnl-2011-301247.
    1. Altman DG, Vergouwe Y, Royston P, Moons KGM. Prognosis and prognostic research: validating a prognostic model. BMJ. 2009;338:b605. doi: 10.1136/bmj.b605.
    1. van der Velde ET, Vriend JW, Mannens MM, et al. CONCOR, an initiative towards a national registry and DNA-bank of patients with congenital heart disease in the Netherlands: rationale, design, and first results. Eur J Epidemiol. 2005;20(6):549–557. doi: 10.1007/s10654-005-4264-9.
    1. Gallego P, Gonzalez AE, Sanchez-Recalde A, et al. Incidence and predictors of sudden cardiac arrest in adults with congenital heart defects repaired before adult life. Am J Cardiol. 2012;110(1):109–117. doi: 10.1016/j.amjcard.2012.02.057.
    1. Wilkoff BL, Williamson BD, Stern RS, et al. Strategic programming of detection and therapy parameters in implantable cardioverter-defibrillators reduces shocks in primary prevention patients: results from the PREPARE (Primary Prevention Parameters Evaluation) study. J Am Coll Cardiol. 2008;52(7):541–550. doi: 10.1016/j.jacc.2008.05.011.

Source: PubMed

Sponsorzy i współpracownicy

Warunki medyczne

Interwencje lekowe

3
Subskrybuj