The Jervell and Lange-Nielsen syndrome; atrial pacing combined with ß-blocker therapy, a favorable approach in young high-risk patients with long QT syndrome?

BackgroundPatients with Jervell and Lange-Nielsen syndrome (JLNS) exhibit severe phenotypes that are characterized by congenital deafness, very long QT intervals, and high risk of life-threatening arrhythmias. Current treatment strategies include high doses of beta-blocker medication, left cardiac sympathetic denervation, and ICD placement, which is challenging in young children.ObjectiveThe purpose of this study was to evaluate the safety and effect of pacing in addition to beta-blocker treatment in children with JLNS.MethodsAll genetically confirmed patients with JLNS born since 1999 in Norway were included in the study. Data on history of long QT syndrome–related symptoms, QT interval, and beta-blocker and pacemaker treatment were recorded.ResultsA total of 9 patients with QT intervals ranging from 510 to 660 ms were identified. Eight patients developed long QT syndrome–related symptoms, and 1 patient died before diagnosis. The survivors received beta-blocker medication. Seven patients also received a pacemaker; 1 had a ventricular lead and 6 had atrial leads. The patient with the ventricular lead died during follow-up. The 6 patients with atrial leads survived without events at a mean follow-up of 6.9 years after pacemaker implantation. Two patients received prophylactic upgrade to a 2-chamber ICD.ConclusionNo arrhythmic events occurred in 6 very young JLNS patients who received atrial pacing in combination with increased doses of beta-blockers during 7-year follow-up. If confirmed in additional patients, this treatment strategy may prevent life-threatening arrhythmias in this high-risk patient group and may act as a bridge to insertion of a 2-chamber ICD when left cardiac sympathetic denervation is not available

Nadolol decreases the incidence and severity of ventricular arrhythmias during exercise stress testing compared with β1-selective β-blockers in patients with catecholaminergic polymorphic ventricular tachycardia

BackgroundCatecholaminergic polymorphic ventricular tachycardia (CPVT) is an inheritable cardiac disease predisposing to malignant ventricular arrhythmias.ObjectiveWe aimed to explore the incidence and severity of ventricular arrhythmias in patients with CPVT before the initiation of β-blocker treatment, when treated with β1-selective β-blockers, and when treated with nadolol.MethodsIn this study, 34 patients with CPVT were included (mean age 34 ± 19 years; 15 (44%) women; 30 (88%) ryanodine receptor 2 variant positive). We performed 3 bicycle exercise stress tests in each patient: (1) before the initiation of β-blocker treatment, (2) after >6 weeks of treatment with β1-selective β-blockers and (3) after >6 weeks of treatment with nadolol. We recorded resting and maximum heart rates and the most severe ventricular arrhythmia occurring. Severity of arrhythmias was scored as 1 point for no arrhythmias or only single ventricular extrasystoles, 2 points for >10 ventricular extrasystoles per minute or bigeminy, 3 points for couplets, and 4 points for nonsustained ventricular tachycardia or sustained ventricular tachycardia.ResultsResting heart rate was similar during treatment with nadolol and β1-selective β-blockers (54 ± 10 beats/min vs 56 ± 14 beats/min; P = .50), while maximum heart rate was lower during treatment with nadolol compared with β1-selective β-blockers (122 ± 21 beats/min vs 139 ± 24 beats/min; P = .001). Arrhythmias during exercise stress testing were less severe during treatment with nadolol compared with during treatment with β1-selective β-blockers (arrhythmic score 1.6 ± 0.9 vs 2.5 ± 0.8; P < .001) and before the initiation of β-blocker treatment (arrhythmic score 1.6 ± 0.9 vs 2.7 ± 0.9; P = .001); however, no differences were observed during treatment with β1-selective β-blockers compared with before the initiation of β-blocker treatment (arrhythmic score 2.5 ± 0.8 vs 2.7 ± 0.9; P = .46).ConclusionThe incidence and severity of ventricular arrhythmias decreased during treatment with nadolol compared with during treatment with β1-selective β-blockers. β1-Selective β-blockers did not change the occurrence or severity of arrhythmias compared with no medication

Cardiac Mechanical Alterations and Genotype Specific Differences in Subjects With Long QT Syndrome

AbstractObjectivesThis study aimed to explore systolic and diastolic function and to investigate genotype-specific differences in subjects with long QT syndrome (LQTS).BackgroundLQTS is an arrhythmogenic cardiac ion channelopathy that traditionally has been considered a purely electrical disease. The most commonly affected ion channels are the slow potassium channel, IKs (KCNQ1 gene/LQT1), and the rapid potassium channel, IKr (KCNH2 gene/LQT2). Recent reports have indicated mechanical abnormalities in patients with LQTS.MethodsWe included 192 subjects with genotyped LQTS (139 LQT1, 53 LQT2). Healthy persons of similar age and sex as patients served as controls (n = 60). Using echocardiography, we assessed systolic function by left ventricular (LV) ejection fraction (EF), global longitudinal strain (GLS), and contraction duration (16 LV segments). Mechanical dispersion was calculated as standard deviation of contraction duration. Time difference between contraction duration and QT interval from electrocardiography (ECG) was defined as electromechanical time difference. We assessed diastolic function by transmitral filling velocities, early diastolic myocardial velocity (e′), and left atrial volume index (LAVI). Heart rate corrected QT interval (QTc) was assessed from 12-lead ECG.ResultsSystolic function by GLS was reduced in subjects with LQTS compared with healthy controls (−22.1 ± 2.1% vs. −23.0 ± 2.0%, p = 0.01), and GLS was worse in subjects with LQT2 compared with subjects with LQT1 (p = 0.01). Subjects with LQTS had longer contraction duration (426 ± 41 ms vs. 391 ± 36 ms, p < 0.001) and more dispersed contractions (33 ± 14 ms vs. 21 ± 7 ms, p < 0.001) compared with healthy controls. Diastolic function was also reduced in subjects with LQTS compared with healthy controls; e′ was lower (10.7 ± 2.7 cm/s vs. 12.5 ± 2.0 cm/s, p < 0.001), and LAVI was increased (30 ± 8 ml/m2 vs. 26 ± 5 ml/m2, p = 0.01), also when adjusted for age and other possible confounders.ConclusionsSubjects with LQTS had a consistent reduction in both systolic and diastolic function compared with healthy controls. Differences in myocardial function between subjects with LQT1 and subjects with LQT2 may indicate that mechanical alterations in LQTS are genotype specific

Role of risk stratification by SPECT, PET, and hybrid imaging in guiding management of stable patients with ischaemic heart disease: expert panel of the EANM cardiovascular committee and EACVI

Risk stratification has become increasingly important in the management of patients with suspected or known ischaemic heart disease (IHD). Recent guidelines recommend that these patients have their care driven by risk assessment. The purpose of this position statement is to summarize current evidence on the value of cardiac single-photon emission computed tomography, positron emission tomography, and hybrid imaging in risk stratifying asymptomatic or symptomatic patients with suspected IHD, patients with stable disease, patients after coronary revascularization, heart failure patients, and specific patient population. In addition, this position statement evaluates the impact of imaging results on clinical decision-making and thereby its role in patient management. The document represents the opinion of the European Association of Nuclear Medicine (EANM) Cardiovascular Committee and of the European Association of Cardiovascular Imaging (EACVI) and intends to stimulate future research in this fiel

EACVI survey on hypertrophic cardiomyopathy

Publisher Copyright: Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: [email protected]: The European Association of Cardiovascular Imaging (EACVI) Scientific Initiatives Committee performed a global survey to evaluate current practice for the assessment and management of patients with hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: A total of 213 centres from 38 different countries (87% European) responded to the survey. One hundred twenty-one (57%) centres followed HCM patients in a general cardiology outpatient clinic and 85 (40%) centres in a specialized HCM/cardiomyopathy clinic. While echocardiography was the primary imaging modality, cardiovascular magnetic resonance (CMR) has become an important complementary tool. Cardiac anatomy, left ventricular (LV) systolic, and diastolic function were assessed according to current European guidelines and recommendations. To evaluate LV obstruction, 49% of the centres performed bedside provocation manoeuvres in every patient and 55% of the centres used exercise stress echocardiography. The majority of centres used the 5-year risk assessment of sudden cardiac death (SCD) calculated with the HCM Risk-SCD score. However, 34% of the centres also used extensive non-infarct late gadolinium enhancement on CMR and 27% the presence of LV apical aneurysm to help select patients for primary prevention implantable cardioverter-defibrillator therapy. Ninety-nine percent of the responding centres performed regular imaging follow-up of HCM patients. CONCLUSION: Most centres followed European guidelines and recommendations for the diagnosis and management of patients with HCM. The importance of bedside provocation manoeuvres and exercise stress echocardiography to diagnose LV outflow obstruction requires emphasis. Additional risk markers for SCD are used in many centres and might indicate the need for an update of current European recommendations.publishersversionpublishe

Mechanical Dispersion Assessed by Myocardial Strain in Patients After Myocardial Infarction for Risk Prediction of Ventricular Arrhythmia

ObjectivesThe aim of this study was to investigate whether myocardial strain echocardiography can predict ventricular arrhythmias in patients after myocardial infarction (MI).BackgroundLeft ventricular (LV) ejection fraction (EF) is insufficient for selecting patients for implantable cardioverter-defibrillator (ICD) therapy after MI. Electrical dispersion in infarcted myocardium facilitates malignant arrhythmia. Myocardial strain by echocardiography can quantify detailed regional and global myocardial function and timing. We hypothesized that electrical abnormalities in patients after MI will lead to LV mechanical dispersion, which can be measured as regional heterogeneity of contraction by myocardial strain.MethodsWe prospectively included 85 post-MI patients, 44 meeting primary and 41 meeting secondary ICD prevention criteria. After 2.3 years (range 0.6 to 5.5 years) of follow-up, 47 patients had no and 38 patients had 1 or more recorded arrhythmias requiring appropriate ICD therapy. Longitudinal strain was measured by speckle tracking echocardiography. The SD of time to maximum myocardial shortening in a 16-segment LV model was calculated as a parameter of mechanical dispersion. Global strain was calculated as average strain in a 16-segment LV model.ResultsThe EF did not differ between ICD patients with and without arrhythmias occurring during follow-up (34 ± 11% vs. 35 ± 9%, p = 0.70). Mechanical dispersion was greater in ICD patients with recorded ventricular arrhythmias compared with those without (85 ± 29 ms vs. 56 ± 13 ms, p < 0.001). By Cox regression, mechanical dispersion was a strong and independent predictor of arrhythmias requiring ICD therapy (hazard ratio: 1.25 per 10-ms increase, 95% confidence interval: 1.1 to 1.4, p < 0.001). In patients with an EF >35%, global strain showed better LV function in those without recorded arrhythmias (−14.0% ± 4.0% vs. −12.0 ± 3.0%, p = 0.05), whereas the EF did not differ (44 ± 8% vs. 41 ± 5%, p = 0.23).ConclusionsMechanical dispersion was more pronounced in post-MI patients with recurrent arrhythmias. Global strain was a marker of arrhythmias in post-MI patients with relatively preserved ventricular function. These novel parameters assessed by myocardial strain may add important information about susceptibility for ventricular arrhythmias after MI

EuroEcho-imaging 2017:highlights

International audienc

EACVI survey on investigations and imaging modalities in chronic coronary syndromes

AIMS The European Association of Cardiovascular Imaging (EACVI) Scientific Initiatives Committee performed a global survey to evaluate current practice for the assessment and management of patients with suspected and confirmed chronic coronary syndromes. METHODS AND RESULTS One-hundred and ten imaging centres from 37 countries across the world responded to the survey. Most non-invasive investigations for coronary artery disease were widely available, except cardiovascular magnetic resonance (available 40% centres). Coronary computed tomography angiography (CCTA) and nuclear scans were reported by a multi-disciplinary team in only a quarter of centres. In the initial assessment of patients presenting with chest pain, only 32% of respondents indicated that they rely on pre-test probability for selecting the optimal imaging test while 31% proceed directly to CCTA. In patients with established coronary artery disease and recurrent chest pain, respondents opted for stress echocardiography (27%) and nuclear stress perfusion scans (26%). In asymptomatic patients with coronary artery disease and an obstructive (>70%) right coronary artery stenosis, 58% of respondents were happy to pursue medical therapy without further testing or intervention. This proportion fell to 29% with left anterior descending artery stenosis and 1% with left main stem obstruction. In asymptomatic patients with evidence of moderate-to-severe myocardial ischaemia (15%), only 18% of respondents would continue medical therapy without further investigation. CONCLUSION Despite guidelines recommendations pre-test probability is used to assess patients with suspected coronary artery in a minority of centres, one-third of centres moving directly to CCTA. Clinicians remain reticent to pursue a strategy of optimal medical therapy without further investigation or intervention in patients with controlled symptoms but obstructive coronary artery stenoses or myocardial ischaemia

Monitoring of Myocardial Involvement in Early Arrhythmogenic Right Ventricular Cardiomyopathy Across the Age Spectrum

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by fibrofatty replacement of primarily the right ventricular myocardium, a substrate for life-threatening ventricular arrhythmias (VAs). Repeated cardiac imaging of at-risk relatives is important for early disease detection. However, it is not known whether screening should be age-tailored. Objectives: The goal of this study was to assess the need for age-tailoring of follow-up protocols in early ARVC by evaluating myocardial disease progression in different age groups. Methods: We divided patients with early-stage ARVC and genotype-positive relatives without overt structural disease and VA at first evaluation into 3 groups: age 50 years without overt ARVC phenotype at first evaluation. Unlike recommended by current guidelines, our study suggests that follow-up of ARVC patients and relatives should not stop at older age