Day 1 :
Lankenau Institute for Medical Research
Time : 10:15 AM to 10:55 AM
Dr. Antzelevitch is Professor and Executive Director of Cardiovascular Research at the Lankenau Institute for Medical Research and Director of Research at Lankenau Heart Institute in Wynnewood, PA. Awards include the Distinguished Scientist Award from the North American Society of Pacing and Electrophysiology (Heart Rhythm Society), Carl J. Wiggers Award of the American Physiological Society, the Distinguished Scientist Award of the American College of Cardiology and the Distinguished Service Award of the Cardiac Electrophysiology Society. He has published over 500 original papers and reviews and six books. He is currently Associate Editor of the Heart Rhythm journal and Secretary-Treasurer of the Cardiac Electrophysiology Society
A prominent J wave is encountered in a number of life-threatening cardiac arrhythmia syndromes, including the Brugada (BrS) and early repolarization (ERS) syndromes. BrS and ERS differ with respect to the magnitude and lead location of abnormal J waves in the ECG and are thought to represent a continuous spectrum of phenotypic expression termed J wave syndromes. Both are associated with vulnerability to polymorphic ventricular tachycardia (VT) and ventricular fibrillation (VF) leading to sudden cardiac death (SCD) in young adults. J wave syndromes are characterized by J-point and ST-elevation in distinct ECG-leads. The region most affected by BrS is the anterior right ventricular outflow tract, accounting for why J-point and ST-segment elevation are generally limited to the right precordial leads. The region most affected in ERS is the inferior wall of the left ventricle, accounting for why the appearance of J waves or early repolarization in the inferior ECG leads is associated with the highest risk for development of arrhythmias and SCD. Despite two decades of intensive research, risk stratification and the approach to therapy of these two inherited cardiac arrhythmia syndromes are still undergoing rapid evolution. Considerable controversy exists as to risk stratification, approach to therapy as well as mechanisms underlying these two syndromes. My objective in this presentation is to provide an integrated review of the clinical characteristics, risk stratifiers, as well as the molecular, ionic, cellular and genetic mechanisms underlying these two interesting syndromes, which have captured the interest and attention of the cardiology community worldwide in recent years.
Université Pierre et Marie Curie
Time : 11:15 to 11:55
Guy H Fontaine has made 16 original contributions in the design and the use of the first cardiac pacemakers in the early 60s. He has serendipitously identified ARVD during his contributions to antiarrhythmic surgery in the early 70s. He has developed the technique of Fulguration to replace surgery in the early 80s. He has been one of the “216 individuals who have made a significant contribution to the study of cardiovascular disease since the 14th century”, one of the “500 greatest geniuses of the 21th century” (USA Books), one of the “100 life time of achievement” (UK Book). He has 900+ publications including 201 book chapters. Reviewer of 23 scientific journals both in basic and clinical science. He has served as a member of the Editorial Board of Circulation during 5 years after reviewing during decades papers for this Journal. He has given 11 master lectures of 90’ each in inland China in 2014. He has developed new techniques of hypothermia for neurologic brain protection in OHCA, stroke and spinal cord injury. He is the first to have resuscitated his wife at home with an external defibrillator (Schiller) still working after 30 years. He has also invented a high-tech device which can be considered as the ultimate in palliative care
ARVCs is covering a spectrum of mostly inherited cardiomyopathies of increasing interest because od a relatively small number of mututions have been identified in 60% of patients (Fressart Europace 2014). The mechanisms of EGS anomalies are btter understood as well as their long term prognosis leading to CHF (Hulot Circulation 2004)
Arrhythmogenic Right Ventricular Dysplasia (ARVD) is mostly due to PKP2 desmosomal mutation with increased RV size with apoptotic thinness of the free wall and segmental anomalies of contraction. This is also due to the presence of fat and interstitial fibrosis mostly observed in the RV free wall and LV apex. This disease is frequent in the general population 3.7% but become clinically apparent in a small number of cases (Fontaine AJC 2014). Clinical presentation is mostly ventricular arrhythmias which can lead to unexpected sudden cardiac death especially in young people and during endurance sports. Some of these patients seen at a late stage of the disease can be misclassified as IDCM. However, in some rare patients, the disease can stop completely its progression.
Brugada syndrome (BrS) has a unique ECG pattern of coved type observed only in lead V1. Structural changes are sometimes producing a Phenotype suggesting ARVD. However, these dkiseases are two different entities with some degree overlap both phenotypically and genotypically in a small number of cases.
Right Ventricular Outflow Tract Ventricular Tachycardia (ROVT VT) is generally benign but one personal case of SD with pathologic documentation demonstrated a localised infundibular anomaly suggesting localised ARVD.
Naxos disease has been identified in the Greek eponym disease is the homozygous form with associated palmoplantar keratosis. This led to the identification of the first mutation Plakoglobin leading to the discovery of multiple candidate genes and finally other mutations.
UHL’s anomaly is rare form which suggests major early apoptosis creating an arrhythmogenic substrate which proved importaznt to demonstrate the re-entrant mechanism of ventricular arrhythmias.
These cardiomyopathies can be affected by a genetically superimposed myocarditis which is frequently the determinant of prognosis (Lopez-Ayala HR 2016).
Experimental ablation of the disease has been demonstrated on the Zebra fish and the mouse opening new vistas for its treatment and prevention (Saffitz Science 2015).