Publications Translational Cardiology / Electrophysiology
Beneficial normalization of cardiac repolarization by carnitine in transgenic SQT1 rabbit models. Cardiovasc. Res. 2024 DOI PubMed
Interactions between KCNQ1 and KCNH2 may modulate the long QT type 1 phenotype. Cardiovasc. Res. 120(7):673-674, 2024 DOI PubMed
KCNQ1 suppression-replacement gene therapy in transgenic rabbits with type 1 long QT syndrome. Eur Heart J 2024 DOI PubMed
State of Gene Therapy for Monogenic Cardiovascular Diseases. Mayo Clinic Proceedings 99(4):610-629, 2024 DOI PubMed
Therapeutic efficacy of mexiletine for LQT2: evidence in hiPSC-derived cardiomyocytes, transgenic rabbits, and patients. Circulation 2024 DOI PubMed
Whole-heart computational modelling provides further mechanistic insights into ST-elevation in Brugada syndrome. Int J Cardiol Heart Vasc. 51:101373, 2024 DOI PubMed
25 years of basic and translational science in EP Europace: novel insights into arrhythmia mechanisms and therapeutic strategies. Europace 25(8):euad210, 2023 DOI
Differential remodelling of late INa in paroxysmal and persistent atrial fibrillation: another piece in the complex picture of electrical remodeling in AF. Can J Cardiol 39(3):289-291, 2023 DOI
Gene- and variant-specific efficacy of serum/glucocorticoid regulated kinase 1-inhibition in long QT syndrome type 1 and 2. EP Europace 2023 DOI
Injectable Contraceptive, Depo-Provera, Produces Erratic Beating Patterns in Patient-Specific Re-Engineered Heart Cells with Type 2 Long QT Syndrome. Heart Rhythm S1547-5271(23)00218-7, 2023 DOI
Mechano-electrical interactions in wild-type and drug-induced LQTS rabbits. J. Physiol. 2023 DOI
Novel Insights Into the Prevalence of TNNI3K-Mediated Dilated Cardiomyopathy and Putative Disease Mechanisms. Circ Genom Precis Med 16(4):337-339, 2023 DOI
Simultaneous assessment of mechanical and electrical function in Langendorff-perfused ex-vivo mouse heart. Front. Cardiovasc. Med. 10:1293032, 2023 DOI PubMed
A computational model of rabbit geometry and ECG: Optimizing ventricular activation sequence and APD distribution. PLoS ONE 17(6):e0270559, 2022 DOI pdf
Catheter Ablation of Short-coupled Variant of Torsade de Pointes at the Moderator Band: A New Treatment Option for a Fatal Arrhythmia. Clin Res Cardiol 111(5):502-510, 2022 DOI PubMed pdf
Deep learning-enabled assessment of cardiac allograft rejection from endomyocardial biopsies. Nature Medicine 28(3):575-582, 2022 DOI
Docosahexaenoic acid normalizes QT interval in long QT type 2 transgenic rabbit models in a genotype-specific fashion. Europace 24(3):511, 2022 DOI PubMed
Effects of Short Term Adiponectin Receptor Agonism on Cardiac Function and Energetics in Diabetic db/db Mice. J Lipid Atheroscler 11(2):161-177, 2022 DOI pdf
Electromechanical reciprocity and arrhythmogenesis in long-QT syndrome and beyond. Eur Heart J ehac135, 2022 DOI
Genome-wide association meta-analysis identifies novel Brugada syndrome susceptibility loci and highlights a new mechanism of sodium channel regulation in disease susceptibility. Nat Genet. 54(3):232-239, 2022 DOI
Genotype-specific ECG-based risk stratification approaches in patients with long-QT syndrome. Front. Cardiovasc. Med. 9:916036, 2022 DOI PubMed pdf
Oxytocin exerts harmful cardiac repolarization prolonging effects - particularly in context of drug-induced LQTS. Int J Cardiol Heart Vasc. 40:101001, 2022 DOI pdf
Transgenic rabbit models for cardiac disease research. Br J Pharmacol 179(5):938-957, 2022 DOI PubMed
Usage of the wearable cardioverter-defibrillator during pregnancy. Int J Cardiol Heart Vasc. 41:101066, 2022 DOI
Arrhythmic risk during pregnancy and postpartum in patients with long-QT syndrome. Herzschrittmacherther Elektrophysiol 32(2), 180-185, 2021 DOI
Enhancing rare variant interpretation in inherited arrhythmias through quantitative analysis of consortium disease cohorts and population controls. Genet Med 23(1):47-58, 2021 DOI pdf
ESC Working Group on Cardiac Cellular Electrophysiology Position Paper: Relevance, Opportunities and Limitations of Experimental Models for Cardiac Electrophysiology Research. EP Europace 23(11):1795-1814, 2021 DOI pdf
Familial dilated cardiomyopathy associated with a novel heterozygous RYR2 early truncating variant. Cardiol J 28(1):173-175, 2021 DOI PubMed pdf
Familial early repolarization syndrome in two siblings: clinical and molecular characterization by whole-exome sequencing. EP Europace 23(5):775-780, 2021 DOI
Inflammation/Immune Response in Arrhythmogenic Cardiomyopathy: Partners in Crime?. Circulation 144(20), S. 1646-1655, 2021 DOI PubMed
Myocardial Histopathology Studies in Brugada Syndrome Decedents - Structural Features of a Presumed Electrical Disease. Am Coll Cardiol 78(15):1522-1524, 2021 DOI PubMed
Sex differences in idiopathic VF - Evidence for sex-specific differences in pro-arrhythmic triggers. Heart Rhythm 18(10):1655-1656, 2021 DOI
Sex-Related Differences in Inherited Arrhythmia Syndromes: Implications for Clinical Practice. Circulation 143(7):739-752, 2021 DOI PubMed
Animals in cardiovascular research: important role of rabbit models in cardiac electrophysiology. Eur Heart J 41(21):2036, 2020 DOI
Heritable arrhythmias associated with abnormal function of the potassium channels. Cardiovasc. Res. 116(9):1542-1556, 2020 DOI PubMed
Late INa blocker GS967 Suppress Polymorphic VT in a Transgenic Rabbit Model of Long QT Type 2. Circ. Arrhythm. Electrophysiol. 13(8):e006875, 2020 DOI PubMed
Mechanism-based QT/APD-shortening therapy for potassium-channel based long-QT syndromes?. Acta Cardiol 229(4):e13481, 2020 DOI PubMed
QT Interval and Its Prolongation - What Does It Mean?. Dtsch Med Wochenschr 145(8): 536-542, 2020 DOI
Transethnic genome-wide association study provides insights in the genetic architecture and heritability of long QT syndrome. Circulation 142(4):324-338, 2020 DOI PubMed pdf
Transgenic LQT2, LQT5 and LQT2-5 rabbit models with decreased repolarization reserve for prediction of drug-induced ventricular arrhythmias. Br J Pharmacol 177(16):3744-3759, 2020 DOI PubMed
Transgenic rabbit models in pro-arrhythmia research. Front. Pharmacol. 11:853, 2020 DOI PubMed
Why do we need genetics in cardiac rhythmology?. Herzschrittmacherther Elektrophysiol 31(4): 394-400, 2020 DOI PubMed
Comment: Postpartum hormones oxytocin and prolactin cause pro-arrhythmic prolongation of cardiac repolarization in long QT syndrome type 2 - Authors' reply. EP Europace 21(7):1141-1142, 2019 DOI PubMed
Dysregulation of the Mitochondrial Proteome Occurs in Mice Lacking Adiponectin Receptor 1. Front. Endocrinology 10:872, 2019 DOI PubMed
Electro-mechanical (dys-)function in long QT syndrome type 1. Int J Cardiol 274:144-151, 2019 DOI PubMed pdf
Perspectives from
K.H. Haugaa, M.K. Stokke. Cardiac electrical and mechanical alterations - united in the long QT syndrome. Int J Cardiol
2019 DOI
German Cardiac Society Working Group on Cellular Electrophysiology state-of-the-art paper: impact of molecular mechanisms on clinical arrhythmia management. Clin Res Cardiol 108(6):577-599, 2019 DOI PubMed
Impaired SIRT3 activity mediates cardiac dysfunction in endotoxemia by calpain-dependent disruption of ATP synthesis. J. Mol. Cell. Cardiol. 133:138-147, 2019 DOI PubMed
Long-term follow-up of implantable cardioverter-defibrillators in Short QT syndrome. Clin Res Cardiol 108(10):1140-1146, 2019 DOI PubMed
Mechanisms of sex differences in atrial fibrillation: role of hormones and differences in electrophysiology, structure, function, and remodelling. Europace 21:366–376, 2019 DOI PubMed
Postpartum hormones oxytocin and prolactin cause pro-arrhythmic prolongation of cardiac repolarization in long QT syndrome type 2. EP Europace 21(7):1126-1138, 2019 DOI PubMed
Sex-differences in Short QT Syndrome: a systematic literature review and pooled analysis. Eur J Prev Cardiol 24:2047487319850953, 2019 DOI PubMed
The role of Nav1.8 in cardiac electrophysiology - a matter of the heart or the nerve?. Cardiovascular Drugs and Therapy 33(6):645-647, 2019 DOI PubMed
Transgenic short-QT syndrome 1 rabbits mimic the human disease phenotype with QT/action potential duration shortening in the atria and ventricles and increased ventricular tachycardia/ventricular fibrillation inducibility. Eur Heart J 7;40(10):842-853, 2019 DOI PubMed
Perspectives from
A.O. Verkerk , C.A. Remme. Down the Rabbit Hole: Deciphering the Short QT Syndrome. Eur Heart J
40(10):842-853, 2019 DOI
A novel LMNA nonsense mutation causes two distinct phenotypes of cardiomyopathy with high risk of sudden cardiac death in a large five-generation family. Europace 20:2003-2013, 2018 DOI PubMed
Another step towards a mechanism-based, subtype-specific therapy in long QT syndrome. Int J Cardiol 263:67-68, 2018 DOI PubMed
Electro-mechanical heterogeneity in the heart: A key to long QT syndrome?. Herzschrittmacherther Elektrophysiol 2018 DOI PubMed
Fascicular parasystole with recurrent syncope. Eur Heart J 2(1):1-4, 2018 DOI
Transgenic animal models of cardiac channelopathies – benefits and limitations. Channelopathies in Heart Disease. 2018
Atrial fibrillation: Therapeutic potential of atrial K+ channel blockers. Pharmacol Ther 176:13-21, 2017 DOI PubMed
Gene expression analysis to identify mechanisms underlying heart failure susceptibility in mice and humans. Basic Res Cardiol 113(1):8, 2017 DOI PubMed
How can we avoid sport-related sudden cardiac death in children with congenital channelopathies? Recommendations for sportive activities. 42(2):162-170, 2017 DOI PubMed
Interregional electro-mechanical heterogeneity in the rabbit myocardium. Prog Biophys Mol Biol 14(9):1388-1397, 2017 DOI PubMed
Magnetic tesonance tissue phase mapping (TPM) demonstrates altered left ventricular diastolic function in children with chronic kidney disease. Pediatr Radiol 47: 169-177, 2017 DOI PubMed
Phase contrast MRI reveals regional, transmural, and base-to-apex dispersion of mechanical dysfunction in LQTS patients. Heart Rhythm <span class="bold">14(9):1388-1397, 2017 DOI PubMed pdf
Perspectives from
E. Arbelo. Another piece of the puzzle: Assessment of mechanical dispersion by magnetic resonance in long QT syndrome. Heart Rhythm
14(9):1398-99, 2017 DOI
Possible pathomechanisms for sensorineural hearing loss and deafness in patients with propionic acidemia. 2017 DOI PubMed
A novel transgenic rabbit model with reduced repolarization reserve: long QT syndrome caused by a dominant-negative mutation of KCNE1 gene. Br J Pharmacol 173(12):2046-61, 2016 DOI PubMed
Another jigsaw piece in the complex picture of hormonal regulation of cardiac repolarization. Eur Heart J 37(7):651-3, 2016 DOI PubMed
Electro-mechanical dysfunction in long QT syndrome: role for arrhythmogenic risk prediction and modulation by sex and sex hormones. Prog Biophys Mol Biol 120(1-3):255-69, 2016 DOI PubMed
Follow the white rabbit: Experimental and computational models of the rabbit heart provide insights into cardiac (patho-) physiology. Prog Biophys Mol Biol 121(2):75-6, 2016 DOI PubMed
It is difficult to make predictions, especially about the future:* thoughts about forecasting cardiotoxicity of pharmacological interventions. Prog Biophys Mol Biol 121(1):1-2, 2016 DOI PubMed
Mechanisms of acquired long-QT syndrome in patients with propionic acidemia. Heart Rhythm 13(6):1335-45, 2016 DOI PubMed
Normalization of QT Interval duration in a long QT syndrome patient during pregnancy and the postpartum period due to sex hormone effects on cardiac repolarization. Heart Rhythm Case Reports 2016 DOI PubMed
Preserved recovery of cardiac function following ischemia-reperfusion in mice lacking SIRT3. Can J Physiol Pharmacol 94(1):72-80, 2016 DOI PubMed
The potential impact of new generation transgenic methods on creating rabbit models of cardiac diseases. Prog Biophys Mol Biol 121(2):123-30, 2016 DOI PubMed
Transgenic rabbit models to investigate the cardiac ion channel diseases long QT syndrome. Prog Biophys Mol Biol 121(2):142-56, 2016 DOI PubMed
Brugada syndrome − diagnosis, risk stratification and therapy. Dtsch Arztebl Int 112:394–401, 2015 DOI PubMed
Early repolarization: a risk factor in Brugada syndrome. Am Coll Cardiol 66(2):205-6, 2015 DOI PubMed
Myocardial mitochondrial dysfunction in mice lacking adiponectin receptor 1. Basic Res Cardiol 110(4):495, 2015 DOI PubMed
SIRT3 deficiency impairs mitochondrial and contractile function in the heart. Basic Res Cardiol 110(4):493, 2015 DOI PubMed
High-dose flecainide with low-dose beta-blocker therapy in catecholaminergic polymorphic ventricular tachycardia: a case report and review of the literature. J Cardiol Cases 174(2):e29-31, 2014 DOI pdf
How do sex hormones modify arrhythmogenesis in LQTS? - Sex hormone effects on arrhythmogenic substrate and on triggered activity. Heart Rhythm 11(11):2107-2115, 2014 DOI PubMed
Hyperphosphorylation of RyRs underlies triggered activity in transgenic rabbit model of LQT2 syndrome. Circ. Res. 115(11):919-28, 2014 DOI PubMed
Pronounced effects of E4031 and erythromycin on APD, spatial APD dispersion and triangulation in transgenic long-QT type 1 rabbits. PLoS ONE 9(9):e107210, 2014 DOI PubMed pdf
Remote ICD-Monitoring in detection and follow-up of idiopathic ventricular fibrillation: Implications for the clinical follow-up of IVF patients. Int J Cardiol 174(2): e29-31, 2014 DOI PubMed
Differential effects of beta-adrenoceptor blockers Carvedilol and Metoprolol on SQT1- and SQT2-causing mutant channels. J. Cardiovasc. Electrophysiol. 24(10):1163-71, 2013 DOI PubMed
Risk stratification in long QT syndrome: Are we finally getting closer to a mutation-specific assessment of an individual patient's arrhythmogenic risk?. Heart Rhythm 10(5):726-7, 2013 DOI PubMed
Sex differences and sex hormone effects in long-QT syndrome – what can we learn from rabbit models?. Clin Res Cardiol Suppl 8(1):14-19, 2013 DOI
Spatial Correlation of Action Potential Duration and Diastolic Dysfunction in Transgenic and Drug-induced LQT2 Rabbits. Heart Rhythm 10(10):1533-1541, 2013 DOI PubMed pdf
Perspectives from
P. Kohl. From ion channel to organismic phenotype: An example of integrative translational research into cardiac electromechanics. Heart Rhythm
10(10):1542-3, 2020 DOI
A quantitative comparison of regional myocardial motion in mice, rabbits and humans using in-vivo phase contrast CMR. J Cardiovasc Magn 14:87, 2012 DOI PubMed pdf
Electromechanical and structural alterations in the aging rabbit heart and aorta. AJP Heart Physiol 2012 DOI PubMed pdf
Estradiol Promotes Sudden Cardiac Death in Transgenic Long-QT Type 2 Rabbits while Progesterone is Protective. Heart Rhythm 9(5):823-32, 2012 DOI PubMed pdf
Heart Rhythm outstanding basic science article 2012
Perspectives from
A.J. Moss. Sex Hormones and Ventricular Tachyarrhythmias in LQTS: New Insights Regarding Antiarrhythmic Therapy . Heart Rhythm
9(5):833-834, 2012 DOI
On the emerging role of rabbit as human disease model and the instrumental role of novel transgenic tools. Transgenic Res 21:699-713, 2012 DOI PubMed
Sex hormones and cardiac arrest in long QT syndrome: Does progesterone represent a potential new antiarrhythmic therapy? . Heart Rhythm 9(7):1150-2, 2012 DOI PubMed
Nicorandil normalizes prolonged repolarisation in the first transgenic rabbit model with Long-QT syndrome 1 both in vitro and in vivo. Eur J Pharmacol 2011 DOI PubMed pdf
Pharmacological activation of Kv11.1 in transgenic long QT-1 rabbits. J. Cardiovasc Pharmacol 57(2):223-30, 2011 DOI PubMed
Electrophysiological Studies of Transgenic Long QT Type 1 and Type 2 Rabbits Reveal Genotype-Differences in Ventricular Refractoriness and His-Purkinje Conduction. AJP Heart Physiol 299(3):H643-55, 2010 DOI PubMed pdf
Enhanced complement resistance of drug-selected P-glycoprotein positive multidrug resistant ovarian carcinoma cells. Clin Exp Immunol 155(2): 239-48, 2009 DOI PubMed pdf
In vivo effect of the selective elimination of Ito,f due to a dominant negative Kv4.2 loss-of-function mutation on atrial refractoriness and atrial fibrillation in mice. Circ J 73(3):461-7, 2009 DOI PubMed pdf
Origin of complex behavior of spatially discordant alternans in transgenic rabbit model of LQT2. J. Physiol. 587(Pt 19):4661-80, 2009 DOI PubMed pdf
Mechanisms of Cardiac Arrhythmias and Sudden Death in Transgenic Rabbits with Long QT Syndrome. Clin Invest 118(6):2246-2259, 2008 DOI PubMed pdf
Pharmacogenomics of anesthetic drugs in transgenic LQT1 and LQT2 rabbits reveal genotype-specific differential effects on cardiac repolarization. AJP Heart Physiol 295:H2264-72, 2008 DOI PubMed
Possible gender-related differences in the risk-to-benefit ratio of thrombolysis for acute submassive pulmonary embolism. Am J Cardiol 99(1):103-7, 2007 DOI