Publications by authors named "François-Pierre Mongeon"

67 Publications

Perinatal and cardiac outcomes of women with hypertrophic cardiomyopathy.

J Matern Fetal Neonatal Med 2021 Oct 15:1-6. Epub 2021 Oct 15.

Department of Obstetrics and Gynecology, Maternal Fetal Medicine Division, University Hospital Centre Sainte-Justine, Montreal, Canada.

Rationale: Pregnancy causes important physiologic stress for women with hypertrophic cardiomyopathy. Data regarding the impact of this condition on obstetrical outcomes is missing.

Objectives: Our objective was to report obstetrical and cardiac outcomes in pregnant women with hypertrophic cardiomyopathy and to assess the possible adverse effects of left ventricular outflow tract obstruction in pregnancy.

Study Design: This was a retrospective cohort study of pregnant women diagnosed with HCM and followed at single tertiary center between 1995 and 2019. Demographic, medical and surgical data, echocardiographic parameters, and pregnancy outcomes were abstracted through extensive chart review. Patients were divided into 2 groups: obstructive (maximal left ventricular outflow tract gradient over 30 mmHg) versus non-obstructive hypertrophic cardiomyopathy. Outcomes between groups were compared with -test, Mann-Whitney and Fisher's exact tests when appropriate.

Results: Eighteen women with 27 pregnancies were included. The study population was formed of 18 women with a total of 27 pregnancies that reached at least 20 weeks of gestation: 12 pregnancies in women with obstructive hypertrophic cardiomyopathy and 15 pregnancies in women with non-obstructive hypertrophic cardiomyopathy. Among the non-obstructive hypertrophic cardiomyopathy, 5 of them had been treated for their obstruction. One patient with obstructive hypertrophic cardiomyopathy had a medical termination of pregnancy for uncontrolled arrhythmia at 21 weeks. There were no maternal deaths. Left ventricular outflow tract obstruction was associated with increased cardiac events including arrhythmias and heart failure (5/12 versus 0/15;  = .006). Preterm birth occurred in more than 50% of cases, resulting from induced delivery for a maternal (40%) or fetal reason (60%). Most deliveries were late preterm between 34 and 36 6/7 weeks. In both groups, birthweight was mainly distributed below the 50th percentile (89%) and 35% of neonates were born small for gestational age defined as a birthweight below the 10th percentile. Most severe cases of small for gestational age (birthweight under the 5th percentile) were found in patients with treated obstructive hypertrophic cardiomyopathy.

Conclusion: Hypertrophic cardiomyopathy is associated with prematurity and small for gestational age. Left ventricular outflow tract obstruction is associated with adverse cardiac events including arrythmias or heart failure. Treated obstructive cardiomyopathy constitutes a sub-group of patients at high risk of severe small for gestational age and deserves a close surveillance. Therefore, fetal growth surveillance with ultrasound, early in the third trimester and doppler studies to assess the utero-placental perfusion in the second and third trimesters are warranted in all patients with hypertrophic cardiomyopathy regardless of the severity of their condition.
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http://dx.doi.org/10.1080/14767058.2021.1990883DOI Listing
October 2021

Wall stresses of early remodeled pulmonary autografts.

J Thorac Cardiovasc Surg 2021 Aug 31. Epub 2021 Aug 31.

Division of Cardiothoracic Surgery, Department of Surgery, University of California San Francisco Medical Center and San Francisco VA Medical Center, San Francisco, Calif. Electronic address:

Objective: The Ross procedure is an excellent option for children or young adults who need aortic valve replacement because it can restore survival to that of the normal aged-matched population. However, autograft remodeling can lead to aneurysmal formation and reoperation, and the biomechanics of this process is unknown. This study investigated postoperative autograft remodeling after the Ross procedure by examining patient-specific autograft wall stresses.

Methods: Patients who have undergone the Ross procedure who had intraoperative pulmonary root and aortic specimens collected were recruited. Patient-specific models (n = 16) were developed using patient-specific material property and their corresponding geometry from cine magnetic resonance imaging at 1-year follow-up. Autograft ± Dacron for aneurysm repair and ascending aortic geometries were reconstructed to develop patient-specific finite element models, which incorporated material properties and wall thickness experimentally measured from biaxial stretching. A multiplicative approach was used to account for prestress geometry from in vivo magnetic resonance imaging. Pressure loading to systemic pressure (120/80) was performed using LS-DYNA software (LSTC Inc, Livermore, Calif).

Results: At systole, first principal stresses were 809 kPa (25%-75% interquartile range, 691-1219 kPa), 567 kPa (485-675 kPa), 637 kPa (555-755 kPa), and 382 kPa (334-413 kPa) at the autograft sinotubular junction, sinuses, annulus, and ascending aorta, respectively. Second principal stresses were 360 kPa (310-426 kPa), 355 kPa (320-394 kPa), 272 kPa (252-319 kPa), and 184 kPa (147-222 kPa) at the autograft sinotubular junction, sinuses, annulus, and ascending aorta, respectively. Mean autograft diameters were 29.9 ± 2.7 mm, 38.3 ± 5.3 mm, and 26.6 ± 4.0 mm at the sinotubular junction, sinuses, and annulus, respectively.

Conclusions: Peak first principal stresses were mainly located at the sinotubular junction, particularly when Dacron reinforcement was used. Patient-specific simulations lay the foundation for predicting autograft dilatation in the future after understanding biomechanical behavior during long-term follow-up.
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http://dx.doi.org/10.1016/j.jtcvs.2021.08.058DOI Listing
August 2021

Pulmonary Vein Stenosis After Atrial Fibrillation Ablation: Insights From the ADVICE Trial.

Can J Cardiol 2020 12 3;36(12):1965-1974. Epub 2020 Nov 3.

Montreal Health Innovations Coordinating Center (MHICC), Montreal, Quebec, Canada.

Background: Pulmonary vein (PV) stenosis is a complication of atrial fibrillation (AF) ablation. The incidence of PV stenosis after routine post-ablation imaging remains unclear and is limited to single-centre studies. Our objective was to determine the incidence and predictors of PV stenosis following circumferential radiofrequency ablation in the multicentre Adenosine Following Pulmonary Vein Isolation to Target Dormant Conduction Elimination (ADVICE) trial.

Methods: Patients with symptomatic AF underwent circumferential radiofrequency ablation in one of 13 trial centres. Computed tomographic (CTA) or magnetic resonance (MRA) angiography was performed before ablation and 90 days after ablation. Two blinded reviewers measured PV diameters and areas. PVs with stenosis were classified as severe (> 70%), moderate (50%-70%), or mild (< 50%). Predictors of PV stenosis were identified by means of multivariable logistic regression.

Results: A total of 197 patients (median age 59.5 years, 29.4% women) were included in this substudy. PV stenosis was identified in 41 patients (20.8%) and 47 (8.2%) of 573 ablated PVs. PV stenosis was classified as mild in 42 PVs (7.3%) and moderate in 5 PVs (0.9%). No PVs had severe stenosis. Both cross-sectional area and diameter yielded similar classifications for severity of PV stenosis. Diabetes was associated with a statistically significant increased risk of PV stenosis (OR 4.91, 95% CI 1.45-16.66).

Conclusions: In the first systematic multicentre evaluation of post-ablation PV stenosis, no patient acquired severe PV stenosis. Although the results are encouraging for the safety of AF ablation, 20.8% of patients had mild or moderate PV stenosis, in which the long-term effects are unknown.
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http://dx.doi.org/10.1016/j.cjca.2020.10.013DOI Listing
December 2020

A Novel Recurrent Genetic Variant Is Associated With a Dysplasia-Associated Arterial Disease Exhibiting Dissections and Fibromuscular Dysplasia.

Arterioscler Thromb Vasc Biol 2020 11 17;40(11):2686-2699. Epub 2020 Sep 17.

Division of Cardiovascular Medicine, Department of Internal Medicine (H.L.H., Y.W., M.-L.Y., K.L.H., J.L., A.E.K., S.K.G.), University of Michigan Medical School, Ann Arbor.

Objective: While rare variants in the gene have been associated with classical Ehlers-Danlos syndrome and rarely with arterial dissections, recurrent variants in underlying a systemic arteriopathy have not been described. Monogenic forms of multifocal fibromuscular dysplasia (mFMD) have not been previously defined. Approach and Results: We studied 4 independent probands with the pathogenic variant c.1540G>A, p.(Gly514Ser) who presented with arterial aneurysms, dissections, tortuosity, and mFMD affecting multiple arteries. Arterial medial fibroplasia and smooth muscle cell disorganization were confirmed histologically. The c.1540G>A variant is predicted to be pathogenic in silico and absent in gnomAD. The c.1540G>A variant is on a shared 160.1 kb haplotype with 0.4% frequency in Europeans. Furthermore, exome sequencing data from a cohort of 264 individuals with mFMD were examined for variants. In this mFMD cohort, c.1540G>A and 6 additional relatively rare variants predicted to be deleterious in silico were identified and were associated with arterial dissections (=0.005).

Conclusions: c.1540G>A is the first recurring variant recognized to be associated with arterial dissections and mFMD. This variant presents with a phenotype reminiscent of vascular Ehlers-Danlos syndrome. A shared haplotype among probands supports the existence of a common founder. Relatively rare genetic variants predicted to be deleterious by in silico analysis were identified in ≈2.7% of mFMD cases, and as they were enriched in patients with arterial dissections, may act as disease modifiers. Molecular testing for should be considered in patients with a phenotype overlapping with vascular Ehlers-Danlos syndrome and mFMD.
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http://dx.doi.org/10.1161/ATVBAHA.119.313885DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7953329PMC
November 2020

Myocardial Vascular Function Assessed by Dynamic Oxygenation-sensitive Cardiac Magnetic Resonance Imaging Long-term Following Cardiac Transplantation.

Transplantation 2021 06;105(6):1347-1355

Research Center, Montreal Heart Institute and Université de Montréal, Montreal, QC, Canada.

Background: Coronary vascular function is related to adverse outcomes following cardiac transplantation (CTx) in patients with or without cardiac allograft vasculopathy (CAV). The noninvasive assessment of the myocardial vascular response using oxygenation-sensitive cardiac magnetic resonance (OS-CMR has not been investigated in stable long-term CTx recipients).

Methods: CTx patients were prospectively recruited to complete a CMR study with a breathing maneuver of hyperventilation followed by a voluntary apnea. Changes in OS-sensitive signal intensity reflecting the myocardial oxygenation response were monitored and expressed as % change in response to these breathing maneuvers. Myocardial injury was further investigated with T2-weighted imaging, native and postcontrast T1 measurements, extracellular volume measurements, and late gadolinium enhancement.

Results: Forty-six CTx patients with (n = 23) and without (n = 23) CAV, along with 25 healthy controls (HC), were enrolled. The OS response was significantly attenuated in CTx compared with HC at the 30-second time-point into the breath-hold (2.63% ± 4.16% versus 6.40% ± 5.96%; P = 0.010). Compared with HC, OS response was lower in CTx without CAV (2.62% ± 4.60%; P < 0.05), while this response was further attenuated in patients with severe CAV (grades 2-3, -2.24% ± 3.65%). An inverse correlation was observed between OS-CMR, ventricular volumes, and diffuse fibrosis measured by extracellular volume mapping.

Conclusions: In heart transplant patients, myocardial oxygenation is impaired even in the absence of CAV suggesting microvascular dysfunction. These abnormalities can be identified by oxygenation-sensitive CMR using simple breathing maneuvers.
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http://dx.doi.org/10.1097/TP.0000000000003419DOI Listing
June 2021

Autograft remodeling after the Ross procedure by cardiovascular magnetic resonance imaging: Aortic stenosis versus insufficiency.

J Thorac Cardiovasc Surg 2020 Jun 27. Epub 2020 Jun 27.

Divison of Noninvasive Cardiology, Department of Specialized Medicine, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada. Electronic address:

Background: Studies suggest that patients undergoing the Ross procedure for aortic insufficiency are at greater risk of autograft dilatation than those with aortic stenosis. By using a tailored Ross technique to mitigate autograft dilatation in patients with aortic insufficiency, we aimed to compare the biomechanical and morphologic remodeling of the autograft at 1 year between patients with aortic insufficiency and patients with aortic stenosis.

Methods: A total of 210 patients underwent a Ross procedure (2011-2016). Of those, 86 patients (mean age 43 ± 13 years; 32% were female) completed preoperative and postoperative cardiovascular magnetic resonance imaging. A total of 71 studies were suitable for analysis: 41 patients with aortic stenosis and 30 patients with aortic insufficiency. Nine healthy adults were used as controls. Autograft root dimensions, individual sinus volumes, and distensibility were measured using cardiovascular magnetic resonance.

Results: At 1 year, there was no difference in autograft root dimensions between patients with aortic stenosis (mean annulus 25.1 ± 3.1 mm and sinus diameters 35 ± 4.1 mm) and patients with aortic insufficiency (26.6 ± 3 mm and 37.1 ± 3.5 mm; P = .12 and .06, respectively). Relative sinus of Valsalva volumes were symmetrical in the aortic stenosis (right 34.8% ± 4%, left 33.7% ± 3.5%, noncoronary 31.4% ± 3.2%) and aortic insufficiency groups (34.8% ± 3.9%, 33.8% ± 2.8%, 31.3% ± 3.7%, P = .85, .92, and .82), and similar to those of healthy adults. Aortic root distensibility was reduced in both groups compared with healthy adults (P = .003), but was similar between aortic stenosis (3.12 ± 1.58 × 10 mm Hg) and aortic insufficiency (3.04 ± 1.15 × 10 mm Hg; P = .9).

Conclusions: Using a tailored technique, there were no differences in the morphologic or biomechanical remodeling of the autograft root 1 year after the Ross procedure between patients with aortic stenosis and patients with aortic insufficiency. However, autograft roots are stiffer than native aortic roots.
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http://dx.doi.org/10.1016/j.jtcvs.2020.03.185DOI Listing
June 2020

Right Ventricular Basal Aneurysm as a Substrate for Ventricular Tachycardia in Tetralogy of Fallot.

JACC Clin Electrophysiol 2020 06;6(6):743-744

Montreal Heart Institute, Montreal, Quebec, Canada. Electronic address:

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http://dx.doi.org/10.1016/j.jacep.2020.03.007DOI Listing
June 2020

T mapping performance and measurement repeatability: results from the multi-national T mapping standardization phantom program (T1MES).

J Cardiovasc Magn Reson 2020 05 7;22(1):31. Epub 2020 May 7.

The Prince Charles Hospital, Griffith University and University of Queensland, Brisbane, Australia.

Background: The T Mapping and Extracellular volume (ECV) Standardization (T1MES) program explored T mapping quality assurance using a purpose-developed phantom with Food and Drug Administration (FDA) and Conformité Européenne (CE) regulatory clearance. We report T measurement repeatability across centers describing sequence, magnet, and vendor performance.

Methods: Phantoms batch-manufactured in August 2015 underwent 2 years of structural imaging, B and B, and "reference" slow T testing. Temperature dependency was evaluated by the United States National Institute of Standards and Technology and by the German Physikalisch-Technische Bundesanstalt. Center-specific T mapping repeatability (maximum one scan per week to minimum one per quarter year) was assessed over mean 358 (maximum 1161) days on 34 1.5 T and 22 3 T magnets using multiple T mapping sequences. Image and temperature data were analyzed semi-automatically. Repeatability of serial T was evaluated in terms of coefficient of variation (CoV), and linear mixed models were constructed to study the interplay of some of the known sources of T variation.

Results: Over 2 years, phantom gel integrity remained intact (no rips/tears), B and B homogenous, and "reference" T stable compared to baseline (% change at 1.5 T, 1.95 ± 1.39%; 3 T, 2.22 ± 1.44%). Per degrees Celsius, 1.5 T, T (MOLLI 5s(3s)3s) increased by 11.4 ms in long native blood tubes and decreased by 1.2 ms in short post-contrast myocardium tubes. Agreement of estimated T times with "reference" T was similar across Siemens and Philips CMR systems at both field strengths (adjusted R ranges for both field strengths, 0.99-1.00). Over 1 year, many 1.5 T and 3 T sequences/magnets were repeatable with mean CoVs < 1 and 2% respectively. Repeatability was narrower for 1.5 T over 3 T. Within T1MES repeatability for native T was narrow for several sequences, for example, at 1.5 T, Siemens MOLLI 5s(3s)3s prototype number 448B (mean CoV = 0.27%) and Philips modified Look-Locker inversion recovery (MOLLI) 3s(3s)5s (CoV 0.54%), and at 3 T, Philips MOLLI 3b(3s)5b (CoV 0.33%) and Siemens shortened MOLLI (ShMOLLI) prototype 780C (CoV 0.69%). After adjusting for temperature and field strength, it was found that the T mapping sequence and scanner software version (both P < 0.001 at 1.5 T and 3 T), and to a lesser extent the scanner model (P = 0.011, 1.5 T only), had the greatest influence on T across multiple centers.

Conclusion: The T1MES CE/FDA approved phantom is a robust quality assurance device. In a multi-center setting, T mapping had performance differences between field strengths, sequences, scanner software versions, and manufacturers. However, several specific combinations of field strength, sequence, and scanner are highly repeatable, and thus, have potential to provide standardized assessment of T times for clinical use, although temperature correction is required for native T tubes at least.
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http://dx.doi.org/10.1186/s12968-020-00613-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7204222PMC
May 2020

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 12 29;35(12):1772-1783. Epub 2019 Aug 29.

McGill Adult Unit for Congenital Heart Disease (MAUDE Unit), McGill University, Montréal, Québec, Canada.

Background: There is no systematic evidence review of the long-term results of surgical pulmonary valve replacement (PVR) dedicated to adults with repaired tetralogy of Fallot (rTOF) and pulmonary regurgitation.

Methods: Our primary objective was to determine whether PVR reduced long-term mortality in adults with rTOF compared with conservative therapy. Secondary objectives were to determine the postoperative incidence rate of death, the changes in functional capacity and in right ventricular (RV) volumes and ejection fraction after PVR, and the postoperative incidence rate of sustained ventricular arrhythmias. A systematic search of multiple databases for studies was conducted without limits.

Results: No eligible randomized controlled trial or cohort study compared outcomes of PVR and conservative therapy in adults with rTOF. We selected 10 cohort studies (total 657 patients) reporting secondary outcomes. After PVR, the pooled incidence rate of death was 1% per year (95% confidence interval [CI] 0-1% per year) and the pooled incidence rate of sustained ventricular arrhythmias was 1% per year (95% CI 1%-2% per year). PVR improved symptoms (odds ratio for postoperative New York Heart Association functional class > II 0.08, 95% CI 0.03-0.24). Indexed RV end-diastolic (-61.29 mL/m, -43.64 to -78.94 mL/m) and end-systolic (-37.20 mL/m, -25.58 to -48.82 mL/m) volumes decreased after PVR, but RV ejection fraction did not change (0.19%, -2.36% to 2.74%). The effect of PVR on RV volumes remained constant regardless of functional status.

Conclusion: Studies comparing PVR and conservative therapy exclusively in adults with rTOF are lacking. After PVR, the incidence rates of death and ventricular tachycardia are both 1 per 100 patient-years. Pooled analyses demonstrated an improved functional status and a reduction in RV volumes.
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http://dx.doi.org/10.1016/j.cjca.2019.08.031DOI Listing
December 2019

Hyperventilation-induced heart rate response as a potential marker for cardiovascular disease.

Sci Rep 2019 11 29;9(1):17887. Epub 2019 Nov 29.

Department of Anaesthesiology and Pain Medicine, Bern University Hospital, Inselspital, University of Bern, Bern, Switzerland.

An increase of heart rate to physical or mental stress reflects the ability of the autonomous nervous system and the heart to respond adequately. Hyperventilation is a user-controlled breathing maneuver that has a significant impact on coronary function and hemodynamics. Thus, we aimed to investigate if the heart rate response to hyperventilation (HRR) can provide clinically useful information. A pooled analysis of the HRR after 60 s of hyperventilation was conducted in 282 participants including healthy controls; patients with heart failure (HF); coronary artery disease (CAD); a combination of both; or patients suspected of CAD but with a normal angiogram. Hyperventilation significantly increased heart rate in all groups, although healthy controls aged 55 years and older (15 ± 9 bpm) had a larger HRR than each of the disease groups (HF: 6 ± 6, CAD: 8 ± 8, CAD+/HF+: 6 ± 4, and CAD-/HF-: 8 ± 6 bpm, p < 0.001). No significant differences were found between disease groups. The HRR may serve as an easily measurable additional marker of cardiovascular health. Future studies should test its diagnostic potential as a simple, inexpensive pre-screening test to improve patient selection for other diagnostic exams.
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http://dx.doi.org/10.1038/s41598-019-54375-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6884614PMC
November 2019

Non-Vitamin K Antagonist Oral Anticoagulants in Adult Congenital Heart Disease.

Can J Cardiol 2019 Dec 26;35(12):1686-1697. Epub 2019 Jun 26.

Adult Congenital Heart Center, Department of Medicine, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada; Division of electrophysiology, Department of medicine, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada.

Non-vitamin K antagonist (VKA) oral anticoagulants (NOACs) have several advantages over VKAs 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 studies suggest that NOACs are safe and effective in selected adults with CHD. NOACs are contraindicated in patients with a mechanical valve, in those with mitral or tricuspid valve stenosis with enlarged and diseased atria, with or without a mitral or tricuspid bioprosthesis, and after recent cardiac surgery (< 3 months). There is currently insufficient evidence to recommend NOACs in patients with a Fontan circulation or cyanotic CHD. Growing literature supports the use of NOACs in patients without CHD who have various forms of valvular heart disease. Therefore, when an indication for oral anticoagulation is established, it appears reasonable to consider a NOAC instead of a VKA in adults with CHD lesions analogous to isolated mitral regurgitation, tricuspid regurgitation, or aortic regurgitation or stenosis. The NOAC agent selected and the prescribed dose should be tailored according to bleeding risk, body weight, renal function, and comedications, especially antiepileptic drugs. The decision to initiate a NOAC should be shared between the patient and care provider. Large-scale research studies are required to further assess safety and efficacy in selected patient subgroups.
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http://dx.doi.org/10.1016/j.cjca.2019.06.022DOI Listing
December 2019

Thromboembolic Risk After Atriopulmonary, Lateral Tunnel, and Extracardiac Conduit Fontan Surgery.

J Am Coll Cardiol 2019 08;74(8):1071-1081

Montreal Health Innovations Coordinating Center, Montreal, Quebec, Canada; Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada. Electronic address:

Background: Thromboembolic events contribute greatly to morbidity and mortality following Fontan surgery for univentricular hearts.

Objectives: This study sought to evaluate the effect of type of Fontan surgery on thromboembolic risk.

Methods: A North American multicenter retrospective cohort study enrolled 522 patients with Fontan palliation consisting of an atriopulmonary connection (APC) (21.4%), lateral tunnel (LT) (41.8%), or extracardiac conduit (EC) (36.8%). Thromboembolic complications and new-onset atrial arrhythmia were reviewed and classified by a blinded adjudicating committee. Thromboembolic risk across surgical techniques was assessed by multivariable competing-risk survival regression.

Results: Over a median follow-up of 11.6 years, 10- and 20-year freedom from Fontan conversion, transplantation, or death was 94.7% and 78.9%, respectively. New-onset atrial arrhythmias occurred in 4.4, 1.2, and 1.0 cases per 100 person-years with APC, LT, and EC, respectively. APC was associated with a 2.82-fold higher risk of developing atrial arrhythmias (p < 0.001), with no difference between LT and EC (p = 0.95). A total of 71 thromboembolic events, 32 systemic and 39 venous, occurred in 12.8% of subjects, for an overall incidence of 1.1%/year. In multivariable analyses, EC was independently associated with a lower risk of systemic (hazard ratio [HR]: 0.20 vs. LT; 95% confidence interval [CI]: 0.04 to 0.97) and combined (HR: 0.34 vs. LT; 95% CI: 0.13 to 0.91) thromboembolic events. A lower incidence of combined thromboembolic events was also observed with antiplatelet agents (HR: 0.54; 95% CI: 0.32 to 0.92) but not anticoagulation (p = 0.53).

Conclusions: The EC Fontan was independently associated with a lower thromboembolic risk after controlling for time-varying effects of atrial arrhythmias and thromboprophylaxis.
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http://dx.doi.org/10.1016/j.jacc.2019.06.051DOI Listing
August 2019

Lead Extraction With Baffle Stenting in Adults With Transposition of the Great Arteries.

JACC Clin Electrophysiol 2019 06 27;5(6):671-680. Epub 2019 Mar 27.

Department of Medicine, Electrophysiology Service, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada; Department of Medicine, Adult Congenital Heart Disease Center, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada. Electronic address:

Objectives: This study sought to assess the feasibility, safety, and outcomes of a stepwise combined percutaneous approach that includes transvenous lead extraction (TLE) followed by baffle stenting and device reimplantation in patients with D-transposition of the great arteries (D-TGA) and atrial baffle dysfunction.

Background: Management of baffle leak or stenosis in patients with D-TGA and atrial switch surgery is challenging in the presence of transvenous cardiac implantable electronic devices. Baffle complications hinder device-related interventions and addressing baffle dysfunction often requires TLE.

Methods: All consecutive patients with D-TGA and TLE followed by a percutaneous baffle intervention at the Montreal Heart Institute between 2009 and 2018 were enrolled.

Results: Ten patients, median 38.6 years of age (range 15.2 to 50.6 years), 5 males (50.0%) were included. Procedures were performed for a device-related indication in 5 patients (50.0%) and for baffle dysfunction in 5 patients (50.0%). A total of 19 leads (17 pacing, 2 defibrillation) were targeted, with a median time from implantation of 8.7 (range 4.3 to 22.1) years. A laser sheath was most frequently required for successful TLE, which was achieved in all patients. Immediate baffle stenting was performed in 9 patients (90.0%) and immediate device reimplantation in 6 (60.0%). During a median follow-up of 3.0 (range 0.1 to 8.2) years, the only complication was subpulmonary atrioventricular valve damage requiring surgery in 1 patient, 8 months after the procedure.

Conclusions: A combined approach with TLE followed by baffle stenting and reimplantation appears to be safe and feasible in D-TGA patients with atrial switch, baffle dysfunction, and transvenous leads.
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http://dx.doi.org/10.1016/j.jacep.2019.01.023DOI Listing
June 2019

Variability in the Echocardiographic Evaluation of the Systemic Right Ventricle.

Can J Cardiol 2019 02 30;35(2):178-184. Epub 2018 Nov 30.

Adult Congenital Heart Center, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada. Electronic address:

Background: Echocardiographic evaluation of the systemic right ventricle (sRV) remains challenging in patients with transposition of the great arteries (TGA) corrected by an atrial switch (AS) and with congenitally corrected TGA (ccTGA). The aim of this study was to determine the interobserver and intraobserver variability of echocardiographic parameters for sRV size and systolic function.

Methods: Six independent observers retrospectively interpreted 44 previously acquired echocardiograms (25 patients with TGA/AS and 19 patients with ccTGA). Quantitative parameters included inlet and longitudinal diameters, systolic and diastolic areas, fractional area change (FAC), and wall thickness. sRV dilatation and systolic function were qualitatively graded as normal, mild, moderate, or severe. sRV hypertrophy was graded as present or absent. Intraclass correlation coefficients (ICCs) and Kappa statistics were computed to assess interobserver variability. Images from 10 patients (5 TGA/AS and 5 ccTGA) were reinterpreted at a 1-month interval, and ICC and Kendall tau b statistics were computed to assess intraobserver variability.

Results: Interobserver and intraobserver agreement were good to excellent for sRV diameters, areas and FAC (ICC, 0.49-0.97), except for the sRV wall thickness (ICC < 0) and the FAC for 1 observer. Interobserver agreement was poor for the qualitative assessment of sRV size and systolic function (Kappa < 0.25), but with a good to excellent intraobserver agreement.

Conclusions: These findings suggest that overall appreciation of sRV size and systolic function relies on variable interpretation of measurements by observers. Readers experienced in CHD and with clear thresholds for quantitative parameters, along with a validated algorithm, are required to guide the evaluation of sRV.
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http://dx.doi.org/10.1016/j.cjca.2018.11.021DOI Listing
February 2019

Myocardial Partition Coefficient of Gadolinium: A Pilot Study in Patients With Acute Myocarditis, Chronic Myocardial Infarction, and in Healthy Volunteers.

Can J Cardiol 2019 01 16;35(1):51-60. Epub 2018 Oct 16.

Philippa & Marvin Carsley CMR Center, Montréal Heart Institute, Université de Montréal, Montréal, Québec, Canada. Electronic address:

Background: The tissue-blood partition coefficient (PC) of gadolinium, derived from T1 measurements, reflects myocardial connective tissue fraction and tissue injury, increasing in proportion with edema or fibrosis. We determined the myocardial PC of gadolinium in patients with acute myocarditis, chronic myocardial infarction (MI), and healthy volunteers. We hypothesized that the characteristics of the injured myocardium in patients with MI and myocarditis may differ and that the PC will be higher in chronically injured myocardium (MI) compared with acutely injured myocardium (myocarditis).

Methods: We performed late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) imaging and T1 mapping before and after administration of gadolinium (0.1 mmol/kg Gd-BOPTA) at 3 Tesla in 10 healthy volunteers (47.1 ± 12.4 years), 18 patients with chronic MI (62.5 ± 8.1 years), and 16 patients with acute myocarditis (42.5 ± 13.9 years).

Results: In patients with chronic MI and focal scar by LGE, the whole left ventricular myocardial PC (0.45 ± 0.05) was higher compared with patients with MI without focal scar (0.39 ± 0.03, P = 0.02) but not significantly different from whole myocardial PC in volunteers (0.40 ± 0.05) or patients with myocarditis (0.41 ± 0.05). The PC in myocarditis scars was lower than in chronic MI scars (0.60 ± 0.12 vs 0.77 ± 0.16, P = 0.016). The relationships of PC and scar burden, expressed as % LGE, were similar and significant for the 2 groups (P = 0.042).

Conclusion: The tissue-blood partition coefficient of Gd-BOPTA is elevated in areas of acute and chronic myocardial injury and may serve as a marker for disease activity and density of scars, which was found to be higher in chronic MI than in acute myocarditis.
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http://dx.doi.org/10.1016/j.cjca.2018.10.005DOI Listing
January 2019

Comparing CMR Mapping Methods and Myocardial Patterns Toward Heart Failure Outcomes in Nonischemic Dilated Cardiomyopathy.

JACC Cardiovasc Imaging 2019 08 15;12(8 Pt 2):1659-1669. Epub 2018 Nov 15.

Noninvasive Cardiovascular Imaging Program, Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts; Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address:

Objectives: In patients with nonischemic dilated cardiomyopathy (NIDCM), native T1, partition coefficient (λ), and extracellular volume fraction (ECV) mapping may offer prognostic values beyond late gadolinium enhancement (LGE), by scaling the range of myocardial changes.

Background: In patients with NIDCM, LGE is seen in 30% of patients and it indicates adverse prognosis.

Methods: The study mapped 6 anatomical locations using all 4 cardiac magnetic resonance (CMR) tissue-characterizing methods and associated with outcome. The authors performed T1 mapping of the myocardium and the blood pool, before and serially after contrast injection, using a Look-Locker cine gradient-echo technique to obtain T1 and the corresponding reciprocal R1 values. λ values were derived from the slopes of the least-squares regression lines for myocardial versus blood R1, then adjusted to serum hematocrit to yield ECV.

Results: Consecutive 240 NIDCM patients (49 ± 16 years of age; 38% women) underwent CMR for cardiac function, LGE, native T1, λ, and ECV. After a median of 3.8 years, 36 (15%) experienced major adverse cardiac events (MACE), including 22 heart failure hospitalizations and 14 deaths. Nonischemic LGE was detected in 34%, whereas ECV was elevated (≥1 location) in 58%. Comparing the 4 methods, mean ECV and λ both demonstrated strong association with MACE (both p < 0.001). In contrast to native T1 and LGE, ECV values from all 6 locations were associated with MACE and death, with the anteroseptum being the most significant (p < 0.0001). The number of abnormal ECV locations correlated linearly with annual MACE rates (p = 0.0003). Mean ECV was the only predictor to enter a prognostic model that contained age, sex, New York Heart Association functional class, and left ventricular ejection fraction. For every 10% increase, mean ECV portended to a 2.8-fold adjusted increase risk to MACE (p < 0.001).

Conclusions: In this study of patients with NIDCM, mapping the myocardial extent of abnormality using ECV offers prognostication toward heart failure outcomes incremental to LGE or native T1 mapping.
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http://dx.doi.org/10.1016/j.jcmg.2018.08.021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6506397PMC
August 2019

Cryoablation for Perinodal Arrhythmia Substrates in Patients With Congenital Heart Disease and Displaced Atrioventricular Conduction Systems.

JACC Clin Electrophysiol 2018 10 25;4(10):1328-1337. Epub 2018 Jul 25.

Adult Congenital Heart Center, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada. Electronic address:

Objectives: The purpose of this study was to assess the safety and efficacy of cryoablation for perinodal substrates in patients with congenital heart disease (CHD) and a displaced atrioventricular (AV) conduction system or an AV conduction system location that was difficult to predict.

Background: Catheter ablation for perinodal arrhythmias in patients with CHD may incur higher risks due to unconventional or difficult to predict locations of the AV conduction system. Cryoablation carries theoretical advantages for such patients but has not been studied in this setting.

Methods: A total of 35 patients with CHD underwent cryoablation for perinodal substrates at the Montreal Heart Institute between 2006 and 2016. Ten of these patients, age 33 ± 13 years, 60% male, had AV conduction systems that were displaced or of uncertain location and underwent cryoablation (6-mm electrode-tip catheter) for 12 perinodal arrhythmias: AV nodal re-entrant tachycardia (n = 4), non-automatic focal atrial tachycardia (n = 4), septal intra-atrial re-entrant tachycardia (n = 3), and para-Hisian automatic focal atrial tachycardia (n = 1). Four patients had single-ventricle physiology and had undergone Fontan palliation (3 atriopulmonary and 1 intracardiac total cavopulmonary connection), 4 underwent repair of AV septal defects, 1 had congenitally corrected transposition of the great arteries (TGA), and 1 had TGA with a Mustard baffle.

Results: Cryoablation was acutely successful in 9 of 12 targeted arrhythmias (75%) with no procedural complication. Crossover to radiofrequency ablation successfully eliminated the remaining 3 arrhythmias at sites deemed safe by cryoablation, with no complication. Over a follow-up period of 26 (interquartile range: 15 to 64) months, 1 of 9 successfully cryoablated arrhythmias recurred.

Conclusions: Cryoablation is feasible, safe, and moderately effective for perinodal arrhythmia substrates in patients with various forms of CHD associated with AV conduction systems that are displaced or in locations that cannot be reliably predicted.
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http://dx.doi.org/10.1016/j.jacep.2018.05.026DOI Listing
October 2018

Pregnancy in adults with repaired/unrepaired atrial septal defect.

J Thorac Dis 2018 Sep;10(Suppl 24):S2945-S2952

Adult Congenital Heart Centre, Montreal Heart Institute, CHUS Ste-Justine, Université de Montréal, Montreal, Canada.

Atrial septal defect (ASD) is the most common form of congenital heart disease. Left-to-right shunting leads to right ventricular (RV) volume overload with excessive pulmonary blood flow. Complications include exercise intolerance, pulmonary vascular disease, RV dysfunction, paradoxical thromboemboli, and atrial arrhythmias. Women with coexisting severe pulmonary hypertension should be counselled against pregnancy due to high incidence of maternal and fetal morbidity and mortality. In the absence of pulmonary hypertension, pregnancy is generally well tolerated in the setting of an ASD. Nevertheless, hemodynamic changes throughout gestation may increase the risk for complications, particularly in those with unrepaired ASDs. Arrhythmias are the most common cardiac event and occur in 4-5%, followed by paradoxical emboli in 2-5%. Obstetrical and neonatal complications include preeclampsia, a higher incidence of infants born small for gestational age, and higher fetal/perinatal mortality. Although there is no definitive evidence demonstrating superiority of an aggressive approach to ASD closure prior to pregnancy, it is currently common practice to electively close asymptomatic but large and/or hemodynamically significant ASDs prior to childbearing. Cardiology follow up during pregnancy should be adapted to clinical circumstances and includes transthoracic echocardiography during the second trimester and arrhythmia monitoring in the event of symptoms.
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http://dx.doi.org/10.21037/jtd.2017.10.130DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6174140PMC
September 2018

Obstetric and cardiac outcomes in women with Marfan syndrome and an aortic root diameter ≤ 45mm.

Eur J Obstet Gynecol Reprod Biol 2018 Nov 11;230:68-72. Epub 2018 Sep 11.

Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, Sainte-Justine University Hospital, Qc,Canada; Université de Montréal, QC, Canada. Electronic address:

Objective: To assess obstetric and aortic outcomes in women with Marfan Syndrome according to aortic root diameter, in view of recommendations for caesarean delivery when the aortic root diameter is ≥40 mm in the 2010 American guidelines versus >45 mm in the 2011 European guidelines.

Study Design: In this retrospective cohort study conducted at Sainte-Justine Mother and Child Tertiary Hospital, 27 pregnancies in 20 women with Marfan Syndrome as defined by the international criteria, were followed prospectively between 1994 and 2017, after excluding women with prior aortic surgery. Obstetric and aortic outcomes were compared in 2 groups according to aortic root diameter: < 40 mm (21 pregnancies) and 40-45 mm (6 pregnancies).

Results: 21/27 women had a vaginal delivery. The caesarean section rate was 23.8% and 16.7% in women with diameter <40 mm and 40-45 mm respectively (p-value = 1), and perinatal outcome was similar across groups. Two women with a prepregnancy aortic root diameter <40 mm developed an acute type B dissection during the third trimester. Both had a family history of aortic dissection.

Conclusions: Vaginal delivery with rigorous pain control and avoidance of Valsalva maneuver may be safely considered in women with Marfan Syndrome and an aortic root diameter ≤45 mm. The risk of type B aortic dissection during pregnancy is hard to predict. Other factors such as family history of dissection and descending aorta size may play an important role, and this may modify our counselling.
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http://dx.doi.org/10.1016/j.ejogrb.2018.09.012DOI Listing
November 2018

Impact of maternal pulmonary insufficiency on fetal growth in pregnancy.

J Matern Fetal Neonatal Med 2020 Apr 19;33(7):1100-1106. Epub 2018 Sep 19.

Maternal-Fetal Medicine Division, Obstetrics & Gynecology Department, Sainte-Justine University Hospital, Montreal, Canada.

It is known that fetal growth is usually proportional to left-sided cardiac output (CO), which parallels the right-sided CO and that congenital right-sided lesions are usually associated with better perinatal outcomes than left-sided lesions. Our objective was to document whether newborns from mothers with severe residual pulmonary valve insufficiency (PI) after surgical tetralogy of Fallot (TOF) or pulmonary valve stenosis (PS) correction have lower birth weight (BW) than newborns from mothers with absent, mild, or moderate PI. This is a retrospective cohort study of women affected with repaired TOF and corrected PS with varied severity of residual PI. Exclusion criteria were: left ventricular dysfunction, left-sided valvular heart disease, other right-sided structural heart disease, chronic hypertension, substance addiction, and incomplete follow-up. Pregnancies were divided into three groups: absent or mild PI, moderate PI, and severe PI. A generalized linear model with normal dependent variable distribution was built and the parameter estimation made with Generalized Estimation Equations (GEE) to take into account repeated mother in data. Variables such as gestational age at birth, maternal age, smoking, and body mass index were tested with bivariate analyses to assess their effect on BW. Only gestational age remained in the adjusted model. A total of 45 patients were included (33 TOF and 12 PS) and 97 pregnancies were reported: 22 miscarriages (22.7%) (15 TOF, 7 PS) and 75 successful pregnancies (57 TOF, 18 PS). The patients were divided into three groups: 1) absent or mild PI, 2) moderate PI, and 3) severe PI groups, which comprised, respectively, 29 (15 TOF, 4 PS), 20 (10 TOF, 1 PS), and 26 successful pregnancies (8 TOF, 7 PS). Using three levels of PI (absent or mild, moderate, and severe), the unadjusted model showed a significant effect of level of PI on BW ( = .0118), as well as the adjusted model ( = .0263) with gestational age as a covariate. The estimated mean newborn's BW was 3055.8 g in the severe PI group, 3151.0 g in the moderate PI group, and 3376.4 g in the absent or mild group when adjusted for gestational age. Hence, we estimated that the mean newborn's BW is 321 g lower in the severe PI group compared with absent or mild PI group ((CI: 572.3; -68.9),  = .0087). Pregnancy is usually well tolerated in repaired TOF and corrected PS. Severe PI either from repaired TOF or PS is at higher risk of lower newborn's BW. Special attention must be paid to the severity of PI. Fetal growth surveillance in the third trimester is warranted.
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http://dx.doi.org/10.1080/14767058.2018.1514492DOI Listing
April 2020

Consensus recommendations for echocardiography in adults with congenital heart defects from the International Society of Adult Congenital Heart Disease (ISACHD).

Int J Cardiol 2018 12 11;272:77-83. Epub 2018 Jul 11.

Cincinnati Children's Hospital, Cincinnati, USA; University Health Network, Peter Munk Cardiac Centre, Toronto Congenital Cardiac Centre for Adults, and University of Toronto, Canada.

The population of adults with congenital heart disease (ACHD) is increasing constantly due to medical, surgical and interventional successes and the input from advanced cardiovascular imaging. ACHD patients are at continuing risk of residua and sequelae related to their CHD contributing to significant morbidity and mortality. Consequently, lifelong expert surveillance is recommended for most patients. Healthcare providers are still working out how best to achieve this objective, how to train enough experts to provide high quality care, and how to organize the delivery of care. Echocardiography is crucial to clinical surveillance providing a comprehensive assessment of cardiac morphology, physiology, pathophysiology, and function. Thus it contributes significantly to the overall clinical management of ACHD patients. The International Society for Adult Congenital Heart Disease (ISACHD; www.isachd.org) is the leading organization of professionals worldwide dedicated to the pursuit of excellence in the care of ACHD patients. Recognizing the critical role of imaging in the diagnosis and management of ACHD, ISACHD established a task force to provide guidance on echocardiographic studies and reporting. The rationale is that standardization of echocardiographic imaging and reporting carries the potential to improve the overall quality of these exams around the world and facilitate collaborative multicenter research. The standardized ACHD protocols provided by the ISACHD task force (found in the appendices) include specific recommendations for data acquisition and reporting for each of the major adult congenital heart lesions. These protocols give a comprehensive and structured approach in the evaluation of ACHD patients and help to ensure excellent patient care.
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http://dx.doi.org/10.1016/j.ijcard.2018.07.058DOI Listing
December 2018

Transvenous Lead Extraction in Adults With Congenital Heart Disease: Insights From a 20-Year Single-Center Experience.

Circ Arrhythm Electrophysiol 2018 02;11(2):e005409

From the Department of Medicine (J.-B.G., M.-A.C., P.P., M.D., B.T., N.C.P., A.D., F.M., F.-P.M., A.W.A., R.I., P.K., B.M.), Adult Congenital Heart Disease Center (M.-A.C., N.C.P., A.D., F.M., F.-P.M., A.W.A., R.I., P.K., B.M.), and Coordination Center (A.S.), Montreal Heart Institute, Université de Montréal, Canada.

Background: Safety and feasibility data on transvenous lead extraction (TLE) in the challenging population of adults with congenital heart disease (A-CHD) are limited. Herein, we report the results of TLE in A-CHD during a 20-year period.

Methods And Results: All consecutive TLE procedures in A-CHD were included in a monocentric prospective registry from 1996. A total of 121 leads were extracted in 49 A-CHD (median age, 38 years; 51% men) during 71 TLE procedures. Twenty-four (49%) patients had transposition of the great arteries. Main indications for extraction were infection in 34 (48%) and lead failure in 22 (31%). A laser sheath was required for 56 (46%) leads and a femoral approach for 10 (8%). Complete TLE was achieved for 111 leads (92%). In multivariable analysis, lead duration (odds ratio, 1.02; 95% confidence interval, 1.00-1.04; <0.01) and number of previous cardiac surgeries (odds ratio, 2.65; 95% confidence interval, 1.52-4.67; <0.01) were predictive of TLE failure. No perioperative death or pericardial effusion was observed. Subpulmonary atrioventricular valve regurgitation increased in 8 patients (5 with transposition of the great arteries) and was independently associated with an implantable cardioverter defibrillator lead (odds ratio, 9.69; 95% confidence interval, 1.31-71.64; =0.03) and valvular vegetation (odds ratio, 7.29; 95% confidence interval, 1.32-40.51; =0.02). After a median of 54 (19-134) months of follow-up after the first TLE, 3 deaths occurred independently from lead management.

Conclusions: Despite complex anatomic issues, TLE can be achieved successfully in most A-CHD using advanced extraction techniques. Subpulmonary atrioventricular valve regurgitation is a prevalent complication, particularly in patients with transposition of the great arteries.
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http://dx.doi.org/10.1161/CIRCEP.117.005409DOI Listing
February 2018

Magnetic-guided catheter ablation of twin AV nodal reentrant tachycardia in a patient with left atrial isomerism, interrupted inferior vena cana, and Kawashima-Fontan procedure.

Clin Case Rep 2017 12 13;5(12):2105-2110. Epub 2017 Nov 13.

Montreal Heart Institute Université de Montréal Montreal Quebec Canada.

Twin AV nodal reentrant tachycardia most commonly occurs in patients with complex congenital heart disease who have two distinct AV nodes, His bundles, and non-preexcited QRS morphologies. Catheter ablation of the weaker AV node may be hindered by anatomical complexities. In such cases, remote magnetic guidance offers a potentially effective solution.
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http://dx.doi.org/10.1002/ccr3.1263DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5715600PMC
December 2017

Late Onset Postcapillary Pulmonary Hypertension in Patients With Transposition of the Great Arteries and Mustard or Senning Baffles.

J Am Heart Assoc 2017 Oct 12;6(10). Epub 2017 Oct 12.

Adult Congenital Heart Centre, Montreal Heart Institute, Université de Montréal, Quebec, Canada

Background: There is a paucity of data regarding late-onset pulmonary hypertension (PH) in patients with transposition of the great arteries and atrial switch surgery.

Methods And Results: A retrospective cohort study was conducted on 140 adults with transposition of the great arteries and atrial switch surgery, age 37.3±7.8, 37.1% female, in order to assess the prevalence and characteristics of late-onset PH and explore associated factors. Patients were followed for a median of 32.3 years after atrial switch surgery and 10.0 years after their first referral visit. PH was detected in 18 of 33 (54.5%) patients who had invasive hemodynamic studies. Average age at diagnosis of PH was 33.9±8.1 years. PH was postcapillary in all, with a mean pulmonary artery pressure of 36±12 mm Hg and mean pulmonary capillary wedge pressure of 28±8 mm Hg. PH was diagnosed in 13 of 17 (76.5%) patients who had cardiac catheterization for heart failure or decreased exercise tolerance. In multivariable analyses, systemic hypertension (odds ratio 9.4, 95% confidence interval 2.2-39.4, =0.002) and heart failure or New York Heart Association class III or IV symptoms (odds ratio 49.8, 95% confidence interval 8.6-289.0, <0.001) were independently associated with PH. Patients with PH were more likely to develop cardiovascular comorbidities including atrial (=0.001) and ventricular (=0.008) arrhythmias, require hospitalizations for heart failure (<0.001), and undergo tricuspid valve surgery (<0.001). Mortality was significantly higher in patients with PH (hazard ratio 9.4, 95% confidence interval 2.1-43.0], <0.001).

Conclusions: Late-onset postcapillary PH is highly prevalent in adults with transposition of the great arteries and atrial switch surgery and is associated with an adverse prognosis.
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http://dx.doi.org/10.1161/JAHA.117.006481DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5721856PMC
October 2017

Risk markers for excess mortality in adults with congenital heart disease: does one size fit all?

J Thorac Dis 2017 Jul;9(7):1772-1776

Montreal Heart Institute Adult Congenital Center, Université de Montréal, Montreal, Quebec, Canada.

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http://dx.doi.org/10.21037/jtd.2017.06.71DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5542967PMC
July 2017

Increasing Prevalence of Atrial Fibrillation and Permanent Atrial Arrhythmias in Congenital Heart Disease.

J Am Coll Cardiol 2017 Aug;70(7):857-865

Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada; Montreal Health Innovations Coordinating Center (MHICC), Montreal, Quebec, Canada. Electronic address:

Background: Atrial arrhythmias are the most common complication encountered in the growing and aging population with congenital heart disease.

Objectives: This study sought to assess the types and patterns of atrial arrhythmias, associated factors, and age-related trends.

Methods: A multicenter cohort study enrolled 482 patients with congenital heart disease and atrial arrhythmias, age 32.0 ± 18.0 years, 45.2% female, from 12 North American centers. Qualifying arrhythmias were classified by a blinded adjudicating committee.

Results: The most common presenting arrhythmia was intra-atrial re-entrant tachycardia (IART) (61.6%), followed by atrial fibrillation (28.8%), and focal atrial tachycardia (9.5%). The proportion of arrhythmias due to IART increased with congenital heart disease complexity from 47.2% to 62.1% to 67.0% in patients with simple, moderate, and complex defects, respectively (p = 0.0013). Atrial fibrillation increased with age to surpass IART as the most common arrhythmia in those ≥50 years of age (51.2% vs. 44.2%; p < 0.0001). Older age (odds ratio [OR]: 1.024 per year; 95% confidence interval [CI]: 1.010 to 1.039; p = 0.001) and hypertension (OR: 2.00; 95% CI: 1.08 to 3.71; p = 0.029) were independently associated with atrial fibrillation. During a mean follow-up of 11.3 ± 9.4 years, the predominant arrhythmia pattern was paroxysmal in 62.3%, persistent in 28.2%, and permanent in 9.5%. Permanent atrial arrhythmias increased with age from 3.1% to 22.6% in patients <20 years to ≥50 years, respectively (p < 0.0001).

Conclusions: IART is the most common presenting atrial arrhythmia in patients with congenital heart disease, with a predominantly paroxysmal pattern. However, atrial fibrillation increases in prevalence and atrial arrhythmias progressively become permanent as the population ages.
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http://dx.doi.org/10.1016/j.jacc.2017.06.034DOI Listing
August 2017

Shone Complex: An Under-recognized Congenital Heart Disease With Substantial Morbidity in Adulthood.

Can J Cardiol 2017 02 29;33(2):253-259. Epub 2016 Sep 29.

Adult Congenital Heart Center, Montreal Heart Institute, Université de Montréal, Montréal, Québec, Canada. Electronic address:

Background: Shone complex consists of a constellation of left-sided, usually obstructive, cardiac lesions, including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and aortic coarctation. Incomplete Shone complex consists of a mitral valve anomaly associated with lesions involving the subaortic region, aortic valve, or thoracic aorta. There is a paucity of data regarding long-term outcomes in adults with Shone complex.

Methods: We reviewed records of adults with complete or incomplete Shone complex followed at the Montreal Heart Institute between 1982 and 2014.

Results: Among 4189 adults with congenital heart disease, 28 (0.67%) patients (mean age, 35 ± 11 years; 50% women) had complete or incomplete Shone complex and were followed for a median of 8 years. Only 39% were previously diagnosed as having Shone complex. The most common defects were congenital mitral stenosis (93%), aortic coarctation (75%), and bicuspid aortic valve (71%). Heart transplantation was required in 2 patients (7.1%) at age 22 and 28 years, respectively. Overall, 48% had cardiovascular hospitalizations during adulthood, predominantly for arrhythmias or heart failure. Freedom from cardiovascular intervention was 55%, 18%, and 8% at 10, 20, and 30 years of age, respectively. Although aortic coarctation was the most common indication for initial intervention (61%), adult interventions occurred predominantly for aortic valve/left ventricular outflow tract (60%) and mitral valve (33%) lesions.

Conclusions: Shone complex is an under-recognized entity associated with relatively low mortality in adulthood but substantial morbidity related to arrhythmias, heart failure, and interventions. Increased awareness of this condition and associated complications may allow for more tailored follow-up.
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http://dx.doi.org/10.1016/j.cjca.2016.09.005DOI Listing
February 2017

Thromboprophylaxis for atrial arrhythmias in congenital heart disease: A multicenter study.

Int J Cardiol 2016 Nov 13;223:729-735. Epub 2016 Aug 13.

Co-PI, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

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 qualifying arrhythmias and outcomes.

Results: A total of 482 patients, 45.2% female, age 32.0±18.0years, were followed for 11.3±9.4years since the qualifying arrhythmia. Antiplatelet therapy was administered to 37.8%, anticoagulation to 54.4%, and neither to 7.9%. Congenital heart disease complexity was simple, moderate, and severe in 18.5%, 34.4%, and 47.1%, respectively. Freedom from thromboembolic events was 84.7±2.7% at 15years, with no difference between anticoagulation versus antiplatelet therapy (P=0.97). Congenital heart disease complexity was independently associated with thromboembolic events, with rates of 0.00%, 0.93%, and 1.95%/year in those with simple, moderate, and severe forms (P<0.001). CHADS and CHADS-VASc scores were not predictive of thromboembolic risk. Annualized bleeding rates with antiplatelet and anticoagulation therapy were 0.66% and 1.82% (P=0.039). In multivariable analyses, anticoagulation [hazard ratio (HR) 4.76, 95% CI (1.05-21.58), P=0.043] and HAS-BLED score [HR 3.15, 95% CI (1.02, 9.78), P=0.047] were independently associated with major bleeds.

Conclusion: Current management of atrial arrhythmias in congenital heart disease is associated with a modest rate of thromboembolic events, which is predicted by disease complexity but not CHADS/CHADS-VASc scores. HAS-BLED score is applicable to the congenital population in predicting major bleeds.
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http://dx.doi.org/10.1016/j.ijcard.2016.08.223DOI Listing
November 2016
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