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 conditions, focused on those that confer predisposition to arrhythmia and sudden cardiac arrest and/or death. The field is rapidly evolving as research discoveries increase.
View Article and Find Full Text PDFBackground: Significant atrioventricular valve regurgitation (AVVR) is prevalent in Fontan adults. Two-dimensional speckle-tracking echocardiography allows for evaluation of subclinical myocardial dysfunction and offers technical benefits. We aimed to evaluate the association of AVVR with echocardiographic parameters and adverse outcomes.
View Article and Find Full Text PDFBackground: Clinical utility of cardiac biomarker testing during pregnancy in women with preexisting cardiac disease is not well known. We studied the levels and temporal trends of NT-proBNP (N-terminal pro-B-type natriuretic peptide) and hs-cTnI (high-sensitivity cardiac troponin I) throughout pregnancy in women with preexisting cardiac disease and sought to assess the association between NT-proBNP and hs-cTnI and pregnancy outcomes.
Methods: Three hundred seven pregnant women with preexisting cardiac disease were prospectively recruited.
Background: The aim of our study was to characterize echocardiographic changes during pregnancy in women with known LVOT obstruction or AS compared to the healthy pregnancy controls, and to assess the relationship with pregnancy outcomes.
Methods: We retrospectively studied 34 pregnant patients with congenital LVOT obstruction or AS with healthy age-matched pregnant controls. Patients with other significant valvular lesions, structural heart disease (LVEF < 40%), or prior valve surgery were excluded.
Background: Anorexia Nervosa (AN) is an eating disorder characterized by low body weight, distorted body image, and an intense fear of gaining weight. Electrocardiogram (ECG) changes, particularly in the QT interval, have been implicated in AN-associated sudden death but not well defined.
Objectives: To characterize QT interval changes during exercise in anorexia nervosa.
Purpose: Anorexia nervosa is a complex psychiatric condition with increased mortality. The electrocardiogram (ECG) may show repolarization changes which may associate with an increased risk of sudden death. Up to 80% of patients may be prescribed psychopharmacotherapies which alter the ECG, potentially compounding arrhythmic risk.
View Article and Find Full Text PDFBackground: Inherited heart rhythm disorders (IHRDs) are complex and uncommon arrhythmogenic conditions that can lead to sudden unexpected death in seemingly healthy individuals. Multidisciplinary programs can assist in the diagnostic testing of potentially affected individuals and their family members.
Methods: Patients evaluated in a specialized adult and pediatric IHRD clinic between April 2013 and February 2015 were characterized.
Circ Arrhythm Electrophysiol
September 2016
Background: Unexplained cardiac arrest (UCA) may be explained by inherited arrhythmia syndromes. The Cardiac Arrest Survivors With Preserved Ejection Fraction Registry prospectively assessed first-degree relatives of UCA or sudden unexplained death victims to screen for cardiac abnormalities.
Methods And Results: Around 398 first-degree family members (186 UCA, 212 sudden unexplained death victims' relatives; mean age, 44±17 years) underwent extensive cardiac workup, including ECG, signal averaged ECG, exercise testing, cardiac imaging, Holter-monitoring, and selective provocative drug testing with epinephrine or procainamide.
Background: The Cardiac Arrest Survivors with Preserved Ejection Fraction Registry (CASPER) enrolls patients with apparently unexplained cardiac arrest and no evident cardiac disease to identify the pathogenesis of cardiac arrest through systematic clinical testing. Exercise testing, drug provocation, advanced cardiac imaging, and genetic testing may be useful when a cause is not apparent.
Methods And Results: The first 200 survivors of unexplained cardiac arrest from 14 centers across Canada were evaluated to determine the results of investigation and follow-up (age, 48.