Published Online
on January 2, 2008

A more recent version of this article appeared on January 22, 2008

This Article Full Text (PDF) CME: Take the course for this article: Circulation: January 22, 2008, Volume 117, Number 3 All Versions of this Article: 117/3/363    most recent CIRCULATIONAHA.107.726372v1 Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Khairy, P. Articles by Cecchin, F. Search for Related Content PubMed PubMed Citation Articles by Khairy, P. Articles by Cecchin, F. Pubmed/NCBI databases Medline Plus Health Information Pacemakers and Implantable Defibrillators Related Collections Clinical Studies Pediatric and congenital heart disease, including cardiovascular surgery Epidemiology Related Article

Submitted on July 6, 2007
Accepted on October 18, 2007

Implantable Cardioverter-Defibrillators in Tetralogy of Fallot

Paul Khairy MD, PhD^*, Louise Harris MD, Michael J. Landzberg MD, Sangeetha Viswanathan MRCPCH, Amanda Barlow MD, Michael A. Gatzoulis MD, Susan M. Fernandes MHP, PA-C, Luc Beauchesne MD, Judith Therrien MD, Philippe Chetaille MD, Elaine Gordon MD, Isabelle Vonder Muhll MD, and Frank Cecchin MD

From the Canadian Adult Congenital Heart (CACH) Network (P.K., L.H., A.B., L.B., J.T., E.G., I.V.M.), Canada; Alliance for Congenital heart Quebec Interinstitutional REsearch (ACQUIRE) (P.K., J.T., P.C.), Canada; Leeds General Infirmary (S.V.), Leeds, United Kingdom; Royal Brompton Hospital (M.A.G.), London, United Kingdom; and Children’s Hospital, (P.K., M.J.L., S.M.F., F.C.), Boston, Mass.

^* To whom correspondence should be addressed. E-mail: paul.khairy{at}umontreal.ca.

Background—Tetralogy of Fallot is the most common form of congenital heart disease in implantable cardioverter-defibrillator (ICD) recipients, yet little is known about the value of ICDs in this patient population.

Methods and Results—We conducted a multicenter cohort study in high-risk patients with Tetralogy of Fallot to determine actuarial rates of ICD discharges, identify risk factors, and characterize ICD-related complications. A total of 121 patients (median age 33.3 years; 59.5% male) were enrolled from 11 sites and followed up for a median of 3.7 years. ICDs were implanted for primary prevention in 68 patients (56.2%) and for secondary prevention in 53 (43.8%), defined by clinical sustained ventricular tachyarrhythmia or resuscitated sudden death. Overall, 37 patients (30.6%) received at least 1 appropriate and effective ICD discharge, with a median ventricular tachyarrhythmia rate of 213 bpm. Annual actuarial rates of appropriate ICD shocks were 7.7% and 9.8% in primary and secondary prevention, respectively (P=0.11). A higher left ventricular end-diastolic pressure (hazard ratio 1.3 per mm Hg, P=0.004) and nonsustained ventricular tachycardia (hazard ratio 3.7, P=0.023) independently predicted appropriate ICD shocks in primary prevention. Inappropriate shocks occurred in 5.8% of patients yearly. Additionally, 36 patients (29.8%) experienced complications, of which 6 (5.0%) were acute, 25 (20.7%) were late lead-related, and 7 (5.8%) were late generator-related complications. Nine patients died during follow-up, which corresponds to an actuarial annual mortality rate of 2.2%, which did not differ between the primary and secondary prevention groups.

Conclusions—Patients with tetralogy of Fallot and ICDs for primary and secondary prevention experience high rates of appropriate and effective shocks; however, inappropriate shocks and late lead-related complications are common.

Key words: tetralogy of Fallot • defibrillation • tachyarrhythmias • death, sudden • heart defects, congenital

Related Article:

Clinical Summaries
Circulation 2008 117: 331-332. [Extract] [Full Text]

This article has been cited by other articles:

				W. M. Book, B. Kogon, and M. E. McConnell Letter by Book et al Regarding Article, "Pulmonary Valve Replacement in Tetralogy of Fallot: Impact on Survival and Ventricular Tachycardia" Circulation, September 8, 2009; 120(10): e79 - e79. [Full Text] [PDF]

				D. M. Harrild, C. I. Berul, F. Cecchin, T. Geva, K. Gauvreau, E. P. Walsh, and F. Pigula Response to Letter Regarding Article, "Pulmonary Valve Replacement in Tetralogy of Fallot: Impact on Survival and Ventricular Tachycardia" Circulation, September 8, 2009; 120(10): e80 - e80. [Full Text] [PDF]

				E. M. Aliot, W. G. Stevenson, J. M. Almendral-Garrote, F. Bogun, C. H. Calkins, E. Delacretaz, P. D. Bella, G. Hindricks, P. Jais, M. E. Josephson, et al. EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias: Developed in a partnership with the European Heart Rhythm Association (EHRA), a Registered Branch of the European Society of Cardiology (ESC), and the Heart Rhythm Society (HRS); in collaboration with the American College of Cardiology (ACC) and the American Heart Association (AHA) Europace, June 1, 2009; 11(6): 771 - 817. [Full Text] [PDF]

				J. E. Deanfield, R. Yates, F. J. Meijboom, and B. J.M. Mulder CHAPTER 10 Congenital Heart Disease in Children and Adults ESC Textbook of Cardiovascular Medicine, January 1, 2009; 2(1): med-9780199566990-chapter - med-9780199566990-chapter. [Abstract] [Full Text] [PDF]

				T. P. Graham Jr The Year in Congenital Heart Disease J. Am. Coll. Cardiol., October 28, 2008; 52(18): 1492 - 1499. [Full Text] [PDF]

				M. J. Silka and Y. Bar-Cohen Should patients with congenital heart disease and a systemic ventricular ejection fraction less than 30% undergo prophylactic implantation of an ICD?: Patients With Congenital Heart Disease and a Systemic Ventricular Ejection Fraction Less Than 30% Should Undergo Prophylactic Implantation of an Implantable Cardioverter Defibrillator Circ Arrhythm Electrophysiol, October 1, 2008; 1(4): 298 - 306. [Full Text] [PDF]

				J. K. Triedman Should patients with congenital heart disease and a systemic ventricular ejection fraction less than 30% undergo prophylactic implantation of an ICD?: Implantable Cardioverter Defibrillator Implantation Guidelines Based Solely on Left Ventricular Ejection Fraction Do Not Apply to Adults With Congenital Heart Disease Circ Arrhythm Electrophysiol, October 1, 2008; 1(4): 307 - 316. [Full Text] [PDF]

				P. Khairy, L. Harris, M. J. Landzberg, S. M. Fernandes, A. Barlow, L.-A. Mercier, S. Viswanathan, P. Chetaille, E. Gordon, A. Dore, et al. Sudden Death and Defibrillators in Transposition of the Great Arteries With Intra-atrial Baffles: A Multicenter Study Circ Arrhythm Electrophysiol, October 1, 2008; 1(4): 250 - 257. [Abstract] [Full Text] [PDF]

				M. Tomaske, R. Pretre, M. Rahn, and U. Bauersfeld Epicardial and pleural lead ICD systems in children and adolescents maintain functionality over 5 years Europace, October 1, 2008; 10(10): 1152 - 1156. [Abstract] [Full Text] [PDF]

				K. K. Witte, C. B. Pepper, J. C. Cowan, J. D. Thomson, K. M. English, and M. E. Blackburn Implantable cardioverter-defibrillator therapy in adult patients with tetralogy of Fallot Europace, August 1, 2008; 10(8): 926 - 930. [Abstract] [Full Text] [PDF]

				P. Bordachar, X. Iriart, J. Chabaneix, F. Sacher, S. Lafitte, P. Jais, M. Haissaguerre, J. Clementy, P. Dos Santos, and J.-B. Thambo Presence of ventricular dyssynchrony and haemodynamic impact of right ventricular pacing in adults with repaired Tetralogy of Fallot and right bundle branch block Europace, August 1, 2008; 10(8): 967 - 971. [Abstract] [Full Text] [PDF]

				K. K. Witte, C. B. Pepper, J. D. Thomson, and K. M. English Letter by Witte et al Regarding Article, "Implantable Cardioverter-Defibrillators in Tetralogy of Fallot" Circulation, July 29, 2008; 118(5): e99 - e99. [Full Text] [PDF]

				P. Khairy, L. Harris, A. Barlow, L. Beauchesne, J. Therrien, E. Gordon, I. Vonder Muhll, P. Chetaille, M. A. Gatzoulis, M. J. Landzberg, et al. Response to Letter Regarding Article, "Implantable Cardioverter-Defibrillators in Tetralogy of Fallot" Circulation, July 29, 2008; 118(5): e100 - e100. [Full Text] [PDF]