Long-term outcome of repaired tetralogy of Fallot: Survival, tachyarrhythmia, and impact of pulmonary valve replacement


      Pulmonary valve replacement (PVR) is recommended for severe pulmonary regurgitation in repaired tetralogy of Fallot (rTOF).


      The purpose of this study was to investigate the event rate and effectiveness of PVR.


      A retrospective study of tetralogy of Fallot patients who survived total repair from 1970 to 2020 was conducted.


      We identified 1744 rTOF patients; 86.6% with classic rTOF, 11.5% with pulmonary atresia, 0.8% with endocardial cushion defect, and 1.1% with absent pulmonary valve. Annual risks of tachyarrhythmia/sudden cardiac arrest (SCA) increased to 0.295% and 1.338% in patients aged 10–30 and 30–60 years, respectively, without sex predominance. PVR (223 surgical and 39 percutaneous) event rate was 34.7% ± 2.1% by 30 years after repair (annual risk: 1.57% between 10 and 30 years after repair). The second PVR rate was 9.9% ± 4.1% by 20 years after the first PVR. Tachyarrhythmia/SCA risk was higher in PVR patients than in No PVR patients and was reduced in PVR patients without tachyarrhythmia/SCA before PVR. However, survival in patients with ventricular tachyarrhythmia/SCA still was better after PVR. At PVR, 13% of patients had tachyarrhythmia/SCA, which was the major predictor of events after PVR. Before PVR, although the ventricular tachyarrhythmia/SCA risks included QRS duration >160 ms and New York Heart Association functional class III or IV, supraventricular tachyarrhythmia was associated with PVR age ≥28 years and N-terminal pro–brain natriuretic peptide >450 pg/mL.


      Tachyarrhythmia/SCA occurrence and the need for PVR increased with age during young adulthood. PVR reduced subsequent arrhythmias only in those patients without arrhythmias before PVR.


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        • Hoffman J.I.
        • Kaplan S.
        The incidence of congenital heart disease.
        J Am Coll Cardiol. 2002; 39: 1890-1900
        • Wu M.H.
        • Chen H.C.
        • Lu C.W.
        • Wang J.K.
        • Huang S.C.
        • Huang S.K.
        Prevalence of congenital heart disease at live birth in Taiwan.
        J Pediatr. 2010; 156: 782-785
        • Lillehei C.W.
        • Cohen M.
        • Warden H.E.
        • et al.
        Direct vision intracardiac surgical correction of the tetralogy of Fallot, pentalogy of Fallot, and pulmonary atresia defects; report of first ten cases.
        Ann Surg. 1955; 142: 418-442
        • Chiu S.N.
        • Wang J.K.
        • Chen H.C.
        • et al.
        Long-term survival and unnatural deaths of patients with repaired tetralogy of Fallot in an Asian cohort.
        Circ Cardiovasc Qual Outcomes. 2012; 5: 120-125
        • Blais S.
        • Marelli A.
        • Vanasse A.
        • et al.
        The 30-year outcomes of tetralogy of Fallot according to native anatomy and genetic conditions.
        Can J Cardiol. 2021; 37: 877-886
        • Hickey E.J.
        • Veldtman G.
        • Bradley T.J.
        • et al.
        Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades.
        Eur J Cardiothorac Surg. 2009; 35 (; discussion 164): 156-164
        • Wray J.
        • Frigiola A.
        • Bull C.
        Loss to specialist follow-up in congenital heart disease; out of sight, out of mind.
        Heart. 2013; 99: 485-490
        • Stout K.K.
        • Daniels C.J.
        • Aboulhosn J.A.
        • et al.
        2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
        J Am Coll Cardiol. 2019; 73: e81-e192
        • Mongeon F.P.
        • Ben Ali W.
        • Khairy P.
        • et al.
        Pulmonary valve replacement for pulmonary regurgitation in adults with tetralogy of Fallot: a meta-analysis—a report for the Writing Committee of the 2019 Update of the Canadian Cardiovascular Society Guidelines for the Management of Adults With Congenital Heart Disease.
        Can J Cardiol. 2019; 35: 1772-1783
        • Vonder Muhll I.F.
        Timing and results of pulmonary valve replacement for pulmonary regurgitation in repaired tetralogy of Fallot: a challenge for evidence-based medicine.
        Can J Cardiol. 2019; 35: 1620-1622
        • Harrild D.M.
        • Berul C.I.
        • Cecchin F.
        • et al.
        Pulmonary valve replacement in tetralogy of Fallot: impact on survival and ventricular tachycardia.
        Circulation. 2009; 119: 445-451
        • Bessière F.
        • Gardey K.
        • Bouzeman A.
        • et al.
        Impact of pulmonary valve replacement on ventricular arrhythmias in patients with tetralogy of Fallot and implantable cardioverter-defibrillator.
        JACC Clin Electrophysiol. 2021; 7: 1285-1293
        • Egbe A.C.
        • Crestanello J.
        • Dearani J.A.
        • et al.
        Long-term outcomes in adults with repaired tetralogy of Fallot and pulmonary atresia.
        Cardiol Young. 2019; 29: 1078-1081
        • Chelliah A.
        • Moon-Grady A.J.
        • Peyvandi S.
        • et al.
        Contemporary outcomes in tetralogy of Fallot with absent pulmonary valve after fetal diagnosis.
        J Am Heart Assoc. 2021; 10e019713
        • Kotani Y.
        • Chetan D.
        • Ono N.
        • et al.
        Late functional outcomes after repair of tetralogy of Fallot with atrioventricular septal defect: a double case-match control study.
        J Thorac Cardiovasc Surg. 2013; 145 (1484.e1471-1474): 1477-1484
        • Shuhaiber J.H.
        • Robinson B.
        • Gauvreau K.
        • et al.
        Outcome after repair of atrioventricular septal defect with tetralogy of Fallot.
        J Thorac Cardiovasc Surg. 2012; 143: 338-343
        • van Mil S.
        • Heung T.
        • Malecki S.
        • et al.
        Impact of a 22q11.2 microdeletion on adult all-cause mortality in tetralogy of Fallot patients.
        Can J Cardiol. 2020; 36: 1091-1097
        • Khairy P.
        • Aboulhosn J.
        • Gurvitz M.Z.
        • et al.
        Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.
        Circulation. 2010; 122: 868-875
        • Wu M.H.
        • Lu C.W.
        • Chen H.C.
        • Chiu S.N.
        • Kao F.Y.
        • Huang S.K.
        Arrhythmic burdens in patients with tetralogy of Fallot: a national database study.
        Heart Rhythm. 2015; 12: 604-609
        • Labombarda F.
        • Hamilton R.
        • Shohoudi A.
        • et al.
        Increasing prevalence of atrial fibrillation and permanent atrial arrhythmias in congenital heart disease.
        J Am Coll Cardiol. 2017; 70: 857-865
        • Go A.S.
        • Hylek E.M.
        • Phillips K.A.
        • et al.
        Prevalence of diagnosed atrial fibrillation in adults: national implications for rhythm management and stroke prevention: the AnTicoagulation and Risk Factors in Atrial Fibrillation (ATRIA) Study.
        JAMA. 2001; 285: 2370-2375
        • Kim Y.G.
        • Han K.D.
        • Choi J.I.
        • et al.
        Non-genetic risk factors for atrial fibrillation are equally important in both young and old age: A nationwide population-based study.
        Eur J Prev Cardiol. 2021; 28: 666-676
        • Beurskens N.E.G.
        • Hagdorn Q.A.J.
        • Gorter T.M.
        • et al.
        Risk of cardiac tachyarrhythmia in patients with repaired tetralogy of Fallot: a multicenter cardiac MRI based study.
        Int J Cardiovasc Imaging. 2019; 35: 143-151
        • Cohen M.I.
        • Khairy P.
        • Zeppenfeld K.
        • Van Hare G.F.
        • Lakkireddy D.R.
        • Triedman J.K.
        Preventing arrhythmic death in patients with tetralogy of Fallot: JACC review topic of the week.
        J Am Coll Cardiol. 2021; 77: 761-771
        • Geva T.
        • Mulder B.
        • Gauvreau K.
        • et al.
        Preoperative predictors of death and sustained ventricular tachycardia after pulmonary valve replacement in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort.
        Circulation. 2018; 138: 2106-2115
        • Hohendanner F.
        • Heinzel F.R.
        • Blaschke F.
        • et al.
        Pathophysiological and therapeutic implications in patients with atrial fibrillation and heart failure.
        Heart Fail Rev. 2018; 23: 27-36
        • Egbe A.C.
        • Najam M.
        • Banala K.
        • et al.
        Impact of atrial arrhythmia on survival in adults with tetralogy of Fallot.
        Am Heart J. 2019; 218: 1-7
        • Therrien J.
        • Siu S.C.
        • Harris L.
        • et al.
        Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot.
        Circulation. 2001; 103: 2489-2494
        • Chiu S.N.
        • Huang S.C.
        • Wang J.K.
        • et al.
        Implantable cardioverter defibrillator therapy in repaired tetralogy of Fallot after pulmonary valve replacement: implications for the mechanism of ventricular arrhythmia.
        Int J Cardiol. 2017; 249: 156-160
        • Bokma J.P.
        • Geva T.
        • Sleeper L.A.
        • et al.
        A propensity score-adjusted analysis of clinical outcomes after pulmonary valve replacement in tetralogy of Fallot.
        Heart. 2018; 104: 738-744