Anne Fournier

CHU Sainte-Justine · Department of Cardiology

Background The univentricular heart with a predominant right ventricle morphology (uRV) has been associated with a higher rate of adverse cardiovascular events. It remains to be determined whether the specific type of uRV influences outcomes. Methods and Results A North American multicenter retrospective cohort study was conducted by the Alliance...

Background There is a paucity of data on long-term outcomes after Fontan palliation in patients with a dominant morphological univentricular right (uRV) vs left (uLV) ventricle. Objectives The purpose of this study was to compare the incidence of atrial arrhythmias, thromboembolic events, cardiac transplantation, and death following Fontan palliat...

There is a growing number of youth with healthcare needs such as disabilities or chronic health conditions who require lifelong care. In Canada, transfer to the adult healthcare system typically occurs at age 18 and is set by policy regardless of whether youth and their families are ready. When the transition to adult services is suboptimal, youth...

SIGNIFICANCE The integrated nature of this project will allow us to co-create with patients, families, and clinicians a set of general recommendations, tailored to local contexts, that can serve as a model for the transition care path of patients with DMD. The period of transition from pediatric to adult care is particularly challenging for adolesc...

Background Transition from paediatric to adult care is challenging for youths with a chronic condition. Most transition programmes place high value in autonomy and independence. We undertook a qualitative study to: (1) identify the needs and aspirations of youths and (2) better understand the well-being and flourishing of youths. Methods Semi-stru...

Hypertrophic cardiomyopathy (HCM), a common cardiomyopathy in children, is an important cause of morbidity and mortality. Early recognition and appropriate management are important. An electrocardiogram (ECG) is often used as a screening tool in children to detect heart disease. The ECG patterns in children with HCM are not well described.ECGs coll...

Arrhythmias are a common complication associated with tetralogy of Fallot (ToF), one of the most prevalent forms of congenital heart disease. As illustrated by this case-based review, various forms of arrhythmias can be encountered across the lifespan of patients with ToF, from infancy to older adulthood. These include atrioventricular block, junct...

Background The role of multidisciplinary clinics for psychosocial care is increasingly recognized for those living with inherited cardiac conditions (ICC). In Canada, access to healthcare providers differ between clinics. Little is known about the relationship between access to specialty care and a patient's ability to cope with, and manage their c...

Background Non-invasive assessment of Fontan Associated Liver Disease (FALD) is of interest, but studies have yielded inconsistent results about the correlation of severity of disease with laboratory values and imaging. Transient elastography (TE) is a non-invasive imaging modality used commonly for liver stiffness measurement (LSM) and in Fontan p...

Inherited arrhythmia syndromes are rare genetic conditions that predispose seemingly healthy individuals to sudden cardiac arrest and death. The Hearts in Rhythm Organization is a multidisciplinary Canadian network of clinicians, researchers, patients, and families that aims to improve care for patients and families with inherited cardiac condition...

Background Diagnosis of congenital long‐QT syndrome (LQTS) is complicated by phenotypic ambiguity, with a frequent normal‐to‐borderline resting QT interval. A 3‐step algorithm based on exercise response of the corrected QT interval (QTc) was previously developed to diagnose patients with LQTS and predict subtype. This study evaluated the 3‐step alg...

Splice-site variants in cardiac genes may predispose carriers to potentially lethal arrhythmias. To investigate, we screened 1315 probands and first-degree relatives enrolled in the Canadian Hearts in Rhythm Organization (HiRO) registry. 10% (134/1315) of patients in the HiRO registry carry variants within 10 base-pairs of the intron-exon boundary...

Background Vitamin K antagonists (VKAs) are frequently prescribed to patients with congenital heart disease (CHD) for atrial arrhythmias or Fontan palliation but there is a paucity of data regarding time spent in the therapeutic range (TTR). We sought to determine the TTR in patients with CHD and atrial arrhythmias or Fontan palliation prescribed V...

Twenty-four-hour ambulatory blood pressure monitoring (ABPM) is widely accepted as a more accurate method for measurement of blood pressure (BP) compared to a single office-based measurement of BP. However, it is unclear how physicians interpret ABPM and make management decisions. This study’s goal is to investigate variation in ABPM interpretation...

Aims Genetic testing is recommended in specific inherited heart diseases but its role remains unclear and it is not currently recommended in unexplained cardiac arrest (UCA). We sought to assess the yield and clinical utility of genetic testing in UCA using whole-exome sequencing (WES). Methods and results Survivors of UCA requiring external defib...

ABSTRACT Background: Several medication choices are available for acute and prophylactic treatment of refractory supraventricular tachycardia (SVT) in infants. There are almost no controlled trials, and medication choices are not necessarily evidence based. Our objective was to report the effectiveness of management strategies for infant SVT. Metho...

BACKGROUND Following an unexplained cardiac arrest, patients and family members typically undergo a comprehensive series of investigations to identify the underlying cause of arrest, with a diagnosis identified in more than half of cardiac arrest survivors. However, the long-term arrhythmic outcomes among cardiac arrest survivors remains unclear....

BACKGROUND The etiology and mechanism of supraventricular tachycardia (SVT) are well understood, however there is no consensus on the acute treatment and medications for prevention of recurrent SVT in infants, and a number of medication choices are available. There are almost no controlled trials and medication choices are not necessarily evidence-...

Research teams developing biobanks and/or genomic databases must develop policies for the disclosure and reporting of potentially actionable genomic results to research participants. Currently, a broad range of approaches to the return of results exist, with some studies opting for non-disclosure of research results while others follow clinical gui...

Aims : The term idiopathic ventricular fibrillation (IVF) describes survivors of unexplained cardiac arrest (UCA) without a specific diagnosis after clinical and genetic testing. Previous reports have described a subset of IVF individuals with ventricular arrhythmia initiated by short-coupled trigger premature ventricular contractions (PVCs) for w...

Background - Following an unexplained cardiac arrest, clinical genetic testing is increasingly becoming standard of care. Periodic review of variant classification is required, as re-interpretation can change the diagnosis, prognosis, and management of patients and their relatives. Methods - This study aimed to develop and validate a standardized a...

Purpose of review: Although rare, sudden cardiac death (SCD) in the young is a tragic event, having a dramatic impact upon all involved. The psychosocial burden associated with SCD can leave friends, families, and entire communities bereft. With only limited evidence to describe the volatile emotional reactions associated with a young SCD, there is...

The SARS-Coronavirus-2 (COVID-19) related pandemic has resulted in profound health, financial and societal impacts. Organized sporting events, from the recreational to Olympic levels, have been cancelled to both mitigate the spread of COVID-19 and protect athletes and highly active individuals from potential acute and long-term infection associated...

The N-terminal proBNP (NT-proBNP), produced by cardiomyocytes, has several reported utilities and associations in acute Kawasaki disease (KD). The objective of this study is to examine the relationship between serum values of NT-proBNP at time of KD diagnosis with cardiac systolic, diastolic function and electrocardiographic changes, at onset of th...

The COVID-19 pandemic has raised ethical questions for the cardiovascular leader and practitioner. Attention has been redirected from a system that focuses on individual patient benefit towards one that focuses on protecting society as a whole. Challenging resource allocation questions highlight the need for a clearly articulated ethics framework t...

Cardiac rehabilitation programs across Canada have suspended in-person services as a result of large-scale physical distancing recommendations designed to flatten the COVID-19 pandemic curve. Virtual cardiac rehabilitation (VCR) offers an alternate mechanism of care delivery, capable of providing similar patient outcomes and safety profiles compare...

Valvular heart disease is observed in approximately 2% of the general population1. Although the initial observation is often localized (for example, to the aortic or mitral valve), disease manifestations are regularly observed in the other valves and patients frequently require surgery. Despite the high frequency of heart valve disease, only a hand...

Hypertension Canada’s 2020 guidelines for the prevention, diagnosis, risk assessment, and treatment of hypertension in adults and children provide comprehensive, evidence-based guidance for health care professionals and patients. Hypertension Canada develops the guidelines using rigourous methodology, carefully mitigating the risk of bias in our pr...

Background The Hearts in Rhythm Organization (HiRO) is a team of Canadian inherited heart rhythm and cardiomyopathy experts, genetic counsellors, nurses, researchers, patients and families dedicated to the detection of inherited arrhythmias and cardiomyopathies, provision of best therapies and protection from the tragedy of sudden cardiac arrest....

Background: Patients with bicuspid aortic valve (BAV) have a higher risk of developing aortic valve dysfunction and progressive proximal aorta dilatation, which can lead to aortic dissection. To this day, identification of children at risk of developing severe aortic dilatation during their pediatric follow-up is still challenging because most stu...

Background: Unexplained cardiac arrest (UCA) is rare in children. Despite investigations, the etiology in up to half remains unknown. Objectives: We assessed the management and outcomes of pediatric UCA survivors through the Canadian Pediatric Heart Rhythm Network METHODS: A retrospective case series of children (aged 1-19 years) presenting with...

Obesity is associated with additional left ventricular hypertrophy (LVH) in adults with hypertrophic cardiomyopathy (HCM). It is not known whether obesity can lead to further LVH in children with HCM. Echocardiographic LV dimensions were determined in 504 children with HCM. Measurements of interventricular septal thickness (IVST) and posterior wall...

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 consis...

Dramatic increases in survival to adulthood for persons born with congenital heart disease (CHD) have led rise to a corresponding need to provide age-appropriate and developmentally appropriate care across the lifespan. Health care transition is a multidimensional process that ideally begins in early adolescence in the pediatric setting and continu...

Prevention of sudden cardiac arrest/death (SCA/D) among athletes is a universal goal, although the optimal strategy for its achievement is controversial, with the inclusion of the 12-lead electrocardiogram (ECG) at the center of the debate. The ECG exhibits superior sensitivity over history and physical examination to detect conditions associated w...

Background: Coronary artery aneurysms (CAA) are a serious complication of Kawasaki disease. Treatment with intravenous immunoglobulin (IVIg) within 10 days of fever onset reduces the risk of CAA from 25% to <5%. Corticosteroids and infliximab are often used in high-risk patients or those with CAA at diagnosis, but there are no data on their longer...

Background: Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM). Objective: The purpose of this study was to determine predictors of LAE in children with HCM. Methods: A retrospective data collection was performed on 446 children and teenagers 2...

Sudden cardiac death (SCD), especially in a young seemingly healthy individual, is a tragic and highly publicized event, which is often followed by a strong emotional reaction from the public and medical community.” Although rare, SCD in the young is devastating to families and communities, underpinning our society's desire to avoid any circumstanc...

Introduction Kawasaki disease (KD) can be associated with concomitant viral or bacterial infections. Children with persistent or recurrent fever 36 hours after the end of intravenous immunoglobulin (IVIG) are considered to be resistant to treatment and are at increased risk for coronary complications. Although concomitant infection does not affect...

Background: Coronary artery (CA) aneurysms are a serious complication of Kawasaki disease (KD). Conventional imaging techniques often described segments with regressed aneurysms as normal, whereas studies have shown significant endothelial dysfunction. Methods: KD patients with aneurysms scheduled for routine coronary angiography underwent optic...

Hypertension Canada provides annually-updated, evidence-based guidelines for the diagnosis, assessment, prevention, and treatment of hypertension in adults and children. This year, the adult and pediatric guidelines are combined in one document. The new 2018 pregnancy-specific hypertension guidelines are published separately. For 2018, 5 new guidel...

Background Atrial arrhythmias are the most common complication encountered in the growing and aging population with congenital heart disease (CHD). We assessed the types and patterns of atrial arrhythmias, associated factors, and age-related trends. Methods A multicenter cohort study enrolled 482 patients with CHD and atrial arrhythmias, age 32.0...

La saturométrie est un moyen simple, sécuritaire, non invasif et démontré d’améliorer le dépistage de la cardiopathie congénitale grave chez les nouveau-nés. Pourtant, ce test n’est pas encore utilisé systématiquement au Canada. Le présent point de pratique fait ressortir l’information essentielle et les recommandations en matière de dépistage. Sel...

Pulse oximetry screening is safe, noninvasive, easy to perform and proven to enhance detection of critical congenital heart disease in newborns. However, this test has yet to be adopted as routine practice in Canada. The present practice point highlights essential details and recommendations for screening, which research has shown to be highly spec...

Background QT interval variations in response to exercise-induced increases in heart rate (HR) have been reported in children and adults in the diagnosis of long QT syndrome. A quick standing challenge has been proposed as an alternative provocative test in adults, with no pediatric data yet available. Methods A standing test was performed in 100...

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 congenita...

Hypertension Canada provides annually-updated, evidence-based guidelines for the diagnosis, assessment, prevention, and treatment of hypertension. This year, we introduce 10 new guidelines. Three previous guidelines have been revised and 5 have been removed. Previous age and frailty distinctions have been removed as considerations for when to initi...

Following the 2016 guidelines for blood pressure measurement, diagnosis and investigation of pediatric hypertension, we now present evidence-based guidelines for the prevention and treatment of hypertension in children. These guidelines were developed by Hypertension Canada’s Guideline Committee pediatric subgroup after thorough evaluation of the a...

Aims: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an ion channelopathy characterized by ventricular arrhythmia during exertion or stress. Mutations in RYR2-coded Ryanodine Receptor-2 (RyR2) and CASQ2-coded Calsequestrin-2 (CASQ2) genes underlie CPVT1 and CPVT2, respectively. However, prognostic markers are scarce. We sought to...

Objectives: Since postnatal identification of accelerated idioventricular rhythm (AIVR) is essentially based on specific electrocardiographic patterns, its prenatal diagnosis is challenging and its genuine incidence undetermined. Therefore the objectives of this study are: 1) To evaluate the performance of specific ultrasonographic approaches in i...

We report a 5-year-old patient with severe pulmonary hypertension and a large secundum atrial septal defect who benefited from a percutaneous closure of the defect with an Occlutech® custom-made fenestrated device. Whereas the closure is technically identical to standard atrial defect closure, the immediate and midterm beneficial results are presen...

Pediatric syncope is a common problem peaking in adolescence, for which there is little data or evidence-based consensus on investigation and management. This document offers guidance for practical evaluation/management of pediatric patients (age<19 years) with syncope encountered in the acute or primary care setting. The writing committee utilized...