The incidence of atrial arrhythmias following Fontan repairs varies by the type of surgery and duration of follow-up. The incidence of late atrial tachycardia has been reduced from 60% to less than 20% by surgical modifications. Late ventricular tachycardia is reported in 3% to 12% of patients. Aggressive efforts to eliminate tachycardia and improve hemodynamics may improve clinical status.
FontanFBaudetE. Surgical repair of tricuspid atresia. Thorax. 1971;26(3):240–248.
2.
GhaiAHarrisLHarrisonDAWebbGDSiuSC. Outcomes of late atrial tachyarrhythmias in adults after the Fontan operation. J Am Coll Cardiol. 2001;37(2):585–592.
3.
GiannakoulasGDimopoulosKYukselSAtrial tachyarrhythmias late after Fontan operation are related to increase in mortality and hospitalization. Int J Cardiol. doi:10.1016/j.ijcard.2010.12.049.
4.
van den BoschAERoos-HesselinkJWVan DomburgRLong-term outcome and quality of life in adult patients after the Fontan operation. Am J Cardiol. 2004;93(9):1141–1145.
5.
FriedmanRA. Sinus and atrioventricular conduction disorders. In: DealBWolffGSGelbandH, eds. Current Concepts in Diagnosis and Treatment of Arrhythmias in Infants and Children. Armonk, NY: Futura Publishing Company;1998: 89–90.
6.
DillerGDimopoulosKOkonkoDHeart rate response during exercise predicts survival in adults with congenital heart disease. J Am Coll Cardiol. 2006;48(6):1250–1256.
7.
de GrootNMLukacPBlomNALong-term outcome of postoperative supraventricular tachycardias in patients with univentricular heart: a European multicenter study. Circ Arrhythm Electrophysiol. 2009;2(3):242–248.
8.
SeslarSPAlexanderMEBerulCICecchinFWalshEPTriedmanJK. Ablation of nonautomatic focal atrial tachycardia in children and adults with congenital heart disease. J Cardiovasc Electrophysiol. 2006;17(4):359–365.
9.
BaeEJNohCIChoiJYTwin AV node and induced supraventricular tachycardia in Fontan palliation patients. Pacing Clin Electrophysiol. 2005;28(2):126–134.
10.
OnoMBoethigDGoerlerHLangeMWesthoff-BleckMBreymannT. Clinical outcome of patients 20 years after Fontan operation—effect of fenestration on late morbidity. Eur J Cardiothorac Surg. 2006;30(6):923–929.
11.
BlaufoxADSleeperLABradleyDJFunctional status, heart rate, and rhythm abnormalities in 521 Fontan patients 6 to 18 years of age. J Thorac Cardiovasc Surg. 2008;136(1):100–107.
12.
StammCFriehsIMayerJEJrLong-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg. 2001;121(1):28–41.
13.
LeeJRKwakJKimKCComparison of lateral tunnel and extracardiac conduit Fontan procedure. Interact Cardiovasc Thorac Surg. 2007;6(3):328–330.
14.
BrownJWRuzmetovMDeschnerBWRodefeldMDTurrentineMW. Lateral tunnel Fontan in the current era: is it still a good option?Ann Thorac Surg. 2010;89(2):556–562.
15.
CohenMIVetterVLWernovskyGEpicardial pacemaker implantation and follow-up in patients with a single ventricle after the Fontan operation. J Throac Cardiovasc Surg. 2001;121(4):804–811.
16.
AzakieAMcCrindleBWVan ArsdellGExtracardiac conduit versus lateral tunnel cavopulmonary connections at a single institution: impact on outcomes. J Thorac Cardiovasc Surg. 2001;122(6):1219–1228.
17.
KimSJKimWHLimHGLeeJY. Outcome of 200 patients after an extracardiac Fontan procedure. J Thorac Cardiovasc Surg. 2008;136(1):108–116.
18.
GiannicoSHammadFAmodeoAClinical outcome of 193 extracardiac Fontan patients: the first 15 years. J Am Coll Cardiol. 2006;47(10):2065–2073.
19.
FujitaSTakahashiKTakeuchiDManagement of late atrial tachyarrhythmia long after Fontan operation. J Cardiol. 2009;53(3):410–416.
20.
WeipertJNoebauerCSchreiberCOccurrence and management of atrial arrhythmia after long-term Fontan circulation. J Thorac Cardiovasc Surg. 2004;127(2):457–464.
21.
NürnbergJHOvroutskiSAlexi-MeskishviliVEwertPHetzerRLangePE. New onset arrhythmias after the extracardiac conduit Fontan operation compared with the intraatrial lateral tunnel procedure: early and midterm results. Ann Thorac Surg. 2004;78(6):1979–1988.
22.
Robbers-VisserDMiedemaMNijveldAResults of staged total cavopulmonary connection for functionally univentricular hearts; comparison of intra-atrial lateral tunnel and extracardiac conduit. Eur J Cardiothorac Surg. 2010;37(4):934–941.
RodefeldMDBrombergBISchuesslerRBBoineauJPCoxJLHuddlestonCB. Atrial flutter after lateral tunnel construction in the modified Fontan operation: a canine model. J Thorac Cardiovasc Surg. 1996;111(3):514–526.
25.
CollinsKKRheeEKDeluccaJMModification to the Fontan procedure for the prophylaxis of intra-atrial reentrant tachycardia: short-term results of a prospective randomized blinded trial. J Thorac Cardiovasc Surg. 2004;127(3):721–729.
26.
GandhiSKBrombergBIRodefeldMDLateral tunnel suture line variation reduces atrial flutter after the modified Fontan operation. Ann Thorac Surg. 1996;61(5):1299–1309.
27.
GiannicoSCornoAMarinoBTotal extracardiac right heart bypass. Circulation. 1992;86(5 suppl): I110–I117.
28.
WarnesCASomervilleJ. Tricuspid atresia in adolescents and adults: current state and late complications. Br Heart J. 1986;56(6):535–543.
29.
WarnesCASomervilleJ. Tricuspid atresia with transposition of the great arteries in adolescents and adults: current state and late complications. Br Heart J. 1987;57(6):543–547.
30.
Sanchez-QuintanaDClimentVHoSYAndersonRH. Myoarchitecture and connective tissue in hearts with tricuspid atresia. Heart. 1999;81(2):182–191.
StephensonEALuMBerulCIArrhythmias in a contemporary Fontan cohort: prevalence and clinical associations in a multicenter cross-sectional study. J Am Coll Cardiol. 2010;56(11):890–896.
33.
NakamuraYYagiharaTKagisakiKHaginoIKobayashiJ. Ventricular performance in long-term survivors after Fontan operation. Ann Thorac Surg. 2011(1);91:172–180.
34.
KhairyPFernandesSMMayerJEJrLong-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008;117(1):85–92.
35.
TsaoSDealBJBackerCLWardKFranklinWHMavroudisC. Device management of arrhythmias after Fontan conversion. J Thorac Cardiovasc Surg. 2009;138(4):937–940.
36.
DubinAMJanousekJRheeEResynchronization therapy in pediatric and congenital heart disease patients: an international multicenter study. J Am Coll Cardiol. 2005;46(12):2277–2283.
37.
CecchinFFranginiPABrownDWCardiac resynchronization therapy (and multisite pacing) in pediatrics and congenital heart disease: five years experience in a single institution. J Cardiovasc Electrophysiol. 2009;20(1):58–65.
38.
BachaEAZimmermanFJMor-AviVVentricular resynchronization by multisite pacing improves myocardial performance in the postoperative single-ventricle patient. Ann Thorac Surg. 2004;78(5):1678–1683.
39.
DealBJMavroudisCBackerCL. Arrhythmia management in the Fontan patient. Pediatr Cardiol, 2007;28(6):448–456.
40.
SilversidesCKSalehianOOechslinECanadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: complex congenital cardiac lesions. Can J Cardiol, 2010;26(3):e98–e117.
41.
ThorneSABarnesICullinanPSomervilleJ. Amiodarone-associated thyroid dysfunction: risk factors in adults with congenital heart disease. Circulation. 1999;100(2):149–154.
42.
TriedmanJKAlexanderMELoveBAInfluence of patient factors and ablative technologies on outcomes of radiofrequency ablation of intra-atrial re-entrant tachycardia in patients with congenital heart disease. J Am Coll Cardiol. 2002;39(11):1827–1835.
43.
YapSCHarrisLSilversidesCKDownarEChauhanVS. Outcome of intra-atrial re-entrant tachycardia catheter ablation in adults with congenital heart disease: negative impact of age and complex atrial surgery. J Am Coll Cardiol. 2010;56(19):1589–1596.
44.
KannankerilPJAndersonMERottmanJNWathenMSFishFA. Frequency of late recurrence of intra-atrial reentry tachycardia after radiofrequency catheter ablation in patients with congenital heart disease. Am J Cardiol. 2003;92(7):879–881.
45.
MavroudisCDealBJBackerCLJ. Maxwell Chamberlain Memorial Paper for congenital heart surgery: 111 Fontan conversions with arrhythmia surgery: surgical lessons and outcomes. Ann Thorac Surg. 2007;84(5):1457–1466.
46.
DealBJMavroudisCBackerCLJohnsrudeCLRocchiniAP. Impact of arrhythmia circuit cryoablation during Fontan conversion for refractory atrial tachycardia. Am J Cardiol. 1999;83(4):563–568.
47.
MavroudisCBackerCLDealBJJohnsrudeCStrasburgerJ. Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation. J Thorac Cardiovasc Surg. 2001;122(5):863–871.
48.
DealBJMavroudisCBackerCLBuckSHJohnsrudeC. Comparison of anatomic isthmus block with the modified right atrial maze procedure for late atrial tachycardia in Fontan patients. Circulation. 2002;160(5):575–579.
49.
MavroudisCBackerCLDealBJ. Late reoperations for Fontan patients: state of the art invited review. Eur J Cardiothorac Surg. 2008;34(5):1034–1040.
50.
GiardiniAHagerAPace NapoleoneCPicchioFM. Natural history of exercise capacity after the Fontan operation: a longitudinal study. Ann Thorac Surg. 2008;85(3):818–821.
51.
AndersonPASleeperLAMahonyLContemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study. J Am Coll Cardiol. 2008;52(2):85–98.
52.
FernandesSMMcElhinneyDBKhairyPGrahamDALandzbergMJRhodesJ. Serial cardiopulmonary exercise testing in patients with previous Fontan surgery. Pediatr Cardiol. 2010;31(2):175–180.
53.
DillerGPGiardiniADimopoulosKPredictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients. Eur Heart J. 2010;31(24):3073–3083.
54.
PrakashATravisonTGFogelMARelation of size of secondary ventricles to exercise performance in children after Fontan operation. Am J Cardiol. 2010;106(11):1652–1656.
