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Arrhythmias in Adults with Congenital Heart Disease: What the Practicing Cardiologist Needs to Know

Published on Jul 1, 2019in Canadian Journal of Cardiology 5.59
· DOI :10.1016/j.cjca.2019.07.009
Paul Khairy55
Estimated H-index: 55
(MHI: Montreal Heart Institute)
Abstract The expanding population of adults with congenital heart disease (CHD) combined with the pervasiveness of arrhythmias has resulted in the rapid growth of a dedicated sector of cardiology at the intersection between two subspecialties: electrophysiology and adult CHD. Herein, practical considerations are offered regarding urgent referral for catheter ablation of atrial arrhythmias, anticoagulation, and primary prevention implantable cardioverter-defibrillators (ICDs). Patients with Ebstein anomaly and ventricular pre-excitation should be referred promptly due to the high prevalence of multiple accessory pathways and increased incidence of atrial tachyarrhythmias, which may be poorly tolerated. In patients with transposition of the great arteries and atrial switch surgery, atrial arrhythmias should be managed without delay since they could provoke ventricular arrhythmias and sudden death. Other settings in which atrial arrhythmias can be poorly tolerated include single ventricle physiology and Eisenmenger syndrome. Long-term anticoagulation is generally indicated in patients with sustained intra-atrial reentrant tachycardia or atrial fibrillation and a mechanical valve, moderate or severe systemic atrioventricular valve stenosis, traditional risk factors for stroke, and/or moderate or complex CHD. The only class I indication for a primary prevention ICD is a systemic left ventricular ejection fraction ≤35%, with biventricular physiology, and New York Heart Association class II or III symptoms. ICD therapy is reasonable in selected adults with tetralogy of Fallot and multiple risk factors for sudden death identified by observational studies. Indications for ICDs in patients with systemic right ventricles and univentricular hearts are less well established, underscoring the need for future research to inform risk stratification.
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Published on Aug 1, 2019in International Journal of Cardiology 3.47
Marie-A Chaix3
Estimated H-index: 3
(MHI: Montreal Heart Institute),
May Chergui1
Estimated H-index: 1
(UdeM: Université de Montréal)
+ 1 AuthorsPaul Khairy55
Estimated H-index: 55
(MHI: Montreal Heart Institute)
Abstract Background Sudden death is the leading cause of mortality in patients with transposition of the great arteries (TGA) and atrial switch surgery. Understanding underlying mechanisms could contribute to identifying high-risk patients and preventing such catastrophic deaths. Methods A total of 144 adults (≥18 years) with TGA and atrial switch surgery were followed at our adult congenital center since 1989. Four patients were excluded: two with double-outlet right ventricles and two with sub...
Published on Jun 1, 2019in Canadian Journal of Cardiology 5.59
François-Pierre Mongeon16
Estimated H-index: 16
(MHI: Montreal Heart Institute),
Laurent Macle (MHI: Montreal Heart Institute)+ 4 AuthorsPaul Khairy55
Estimated H-index: 55
(MHI: Montreal Heart Institute)
Abstract Non-vitamin K antagonist oral anticoagulants (NOACs) have several advantages over vitamin K antagonists (VKA) that render them an attractive option for adults with congenital heart disease (CHD). Efficacy and safety data specific to the adult CHD population are emerging. Herein, we synthesize the growing literature regarding NOACs in adults with CHD and attempt to identify subgroups for which it appears reasonable to extrapolate data from populations without CHD. Small observational stu...
Published on Mar 1, 2019in The Annals of Thoracic Surgery 3.92
Walid Ben Ali2
Estimated H-index: 2
(MHI: Montreal Heart Institute),
Ismail Bouhout9
Estimated H-index: 9
(MHI: Montreal Heart Institute)
+ 2 AuthorsNancy Poirier22
Estimated H-index: 22
(MHI: Montreal Heart Institute)
Background There is growing awareness of the long-term impact of a Fontan circulation on the associated morbidity and mortality. Comparative data on the incidence of supraventricular arrhythmia and sinus node dysfunction following extracardiac conduit (EC) and lateral tunnel (LT) Fontans are controversial. We performed a meta-analysis pooling all available long-term results comparing the EC and LT Fontan, with a special focus on arrhythmia. Methods We performed a systematic search of PubMed, Emb...
Published on Dec 1, 2017in International Journal of Cardiology 3.47
Benjamin M. Moore4
Estimated H-index: 4
(RPA: Royal Prince Alfred Hospital),
Christopher Yu2
Estimated H-index: 2
(Concord Hospital)
+ 2 AuthorsDavid S. Celermajer87
Estimated H-index: 87
(RPA: Royal Prince Alfred Hospital)
Abstract Background The life expectancy of adults with congenital heart disease (CHD) has significantly improved in recent decades, with non-cardiovascular causes of death now competing with traditional cardiovascular causes. The risk of sudden cardiac death (SCD), a devastating event, still remains elevated above that of the general population. Methods We reviewed 2935 patients in our adult CHD database (age≥16years, seen at least once in our centre) and documented all cases of SCD between 2000...
Published on Nov 1, 2016in International Journal of Cardiology 3.47
Paul Khairy55
Estimated H-index: 55
(MHI: Montreal Heart Institute),
Jamil Aboulhosn21
Estimated H-index: 21
(UCLA: University of California, Los Angeles)
+ 18 AuthorsSylvie Levesque20
Estimated H-index: 20
Abstract Background There is a paucity of data to guide decisions regarding thromboprophylaxis for atrial arrhythmias in congenital heart disease. Methods A retrospective multicenter cohort study enrolled patients with documented sustained atrial arrhythmias and congenital heart disease from 12 North American centers to quantify thromboembolic and bleeding rates associated with antiplatelet and anticoagulation therapy, and explore associated factors. A blinded committee adjudicated all qualifyin...
Published on May 7, 2016in European Heart Journal 23.24
Jim T. Vehmeijer4
Estimated H-index: 4
Tom F. Brouwer9
Estimated H-index: 9
+ 4 AuthorsJoris R. de Groot30
Estimated H-index: 30
Aims Sudden cardiac death is a major cause of mortality in adult congenital heart disease (ACHD) patients. The indications for implantable cardioverter-defibrillator (ICD) implantation in ACHD patients are still not well established. We aim to systematically review the literature on indications and outcome of ICD implantation in ACHD patients. Methods and results We performed a comprehensive search in EMBASE, MEDLINE, and Google Scholar to identify all studies on ICD implantation in ACHD patient...
Published on May 1, 2016in International Journal of Cardiology 3.47
Claudia C. Engelings1
Estimated H-index: 1
(MHH: Hannover Medical School),
Paul C. Helm4
Estimated H-index: 4
+ 7 AuthorsOktay Tutarel15
Estimated H-index: 15
(MHH: Hannover Medical School)
Abstract Background Due to the great advances in the care of patients with congenital heart disease (CHD), mortality has decreased significantly over the last decades. Nonetheless, mortality for adults with congenital heart disease (ACHD) is still higher than for the general population. An analysis regarding causes of death in a nationwide contemporary cohort of ACHD is lacking. Methods A well-characterized cohort of the German National Register for Congenital Heart Defects was screened for pati...
Published on Apr 1, 2016in American Journal of Cardiology 2.84
Toni Grönberg1
Estimated H-index: 1
(UTU: University of Turku),
Juha Hartikainen40
Estimated H-index: 40
+ 3 AuthorsK.E. Juhani Airaksinen44
Estimated H-index: 44
(TYKS: Turku University Hospital)
The efficacy of the anticoagulation in preventing thromboembolic complications (TEC) and the usefulness of the CHA 2 DS 2 VASc score for assessing stroke risk during cardioversion of acute atrial fibrillation (AF) are unclear. Thus, our objectives were to assess the ability of the CHA 2 DS 2 VASc score to predict TEC and to evaluate the efficacy of anticoagulation in the prevention of TEC in Finnish CardioVersion (FinCV) study. The FinCV is a retrospective, multicenter study of 3,143 patients, w...
Published on Nov 1, 2014in Europace 6.10
Wei Wei5
Estimated H-index: 5
(Guangdong General Hospital),
Xianzhang Zhan6
Estimated H-index: 6
(Guangdong General Hospital)
+ 5 AuthorsShulin Wu9
Estimated H-index: 9
(Guangdong General Hospital)
Aims The aim of the study was to describe the complex electrophysiological features of accessory pathways (APs) in adult Ebstein's anomaly (EA). Methods and results We performed a retrospective study of 17 consecutive adult EA cases with APs who underwent electrophysiological study and radiofrequency catheter ablation (RFCA) from November 2011 to May 2013. There were a total of 24 atrioventricular reentrant tachycardias (AVRTs) due to 23 APs, including 20 (87.0%) non-decremental conducting, 2 (8...
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