Arrhythmias, Channelopathies

Members of CVRI Theme ‘Arrhythmias & Channelopathies’ are involved in various translational and clinical research projects in cardiac electrophysiology. Specific areas of interest include the mechanisms and treatment of atrial fibrillation, inherited arrhythmia syndromes associated with sudden cardiac death, and sudden unexplained death in epilepsy (SUDEP).

Drs. Jeffrey Kim, Santiago Valdes, Christina Miyake and Caridad de la Uz comprise the team of pediatric electrophysiologists at Texas Children’s Hospital who are involved in a variety of clinical projects that encompass broad aspects of electrophysiology. The group has been studying the efficacy and use of antiarrhythmic medications including 1) digoxin, beta-blockers in supraventricular tachycardia, 2) flecainide in congenital heart disease, 3) use of sotalol in children and 4) resuscitation medications during cardiac arrest. They are actively studying the use of implantable cardioverter defibrillators (ICDs) in children with congenital heart disease, and arrhythmias in heterotaxy syndrome and congenital complete AV block. Lastly, the group is working as a team with Dr. Seema Lalani (Genetics), Dr. Yuxin Fan (Welsh Laboratory), and Dr. Xander Wehrens to study the genetic basis of several disorders including Wolff-Parkinson-White Syndrome, Catecholaminergic Polymorphic Ventricular Tachycardia, long QT syndrome, and left ventricular non-compaction.

Numerous basic research projects in cardiac electrophysiology and ion channel research synergize with our clinical and translational research projects. Specific projects include studies on the mechanistic basis of atrial fibrillation, inherited arrhythmia syndromes, arrhythmias associated with structural heart disease and neurological disorders, and basic ion channel research.

Cardiac arrhythmia mechanisms are studied in tissue samples and cardiac myocytes isolated from patients and large animal models. Moreover, genetic mouse models are used to study arrhythmias at the whole animal level, in isolated hearts using optical fluorescence mapping, in single cardiac myocytes and monolayers, and at the single channel level. These model systems are used to answer basic mechanistic and therapeutic questions relevant to clinic electrophysiology. For example, recent studies have demonstrated the importance of abnormal calcium cycling in the pathogenesis of atrial fibrillation initiation and progression. Novel molecular pathways involved in atrial identify development were discovered and linked to arrhythmia mechanisms. In addition, ongoing programs focus on the identification of new arrhythmia pathways associated with myotonic dystrophy, muscular dystrophy, Rett syndrome, and epilepsy.

Several CVRI investigators (led by Dr. Noebels) are part of a national network focused on sudden unexpected death in epilepsy, and their labs focus on the complex interplay between neuronal regulation and cardiac electrophysiology. Moreover, several labs pursue active research programs on the development of novel ion channel-modifying agents and anti-arrhythmic drugs. Finally, the CVRI has established new training opportunities for cardiology and pediatric cardiology fellows to participate in (basic) electrophysiology research at Baylor College of Medicine.

Anne Anderson, M.D. - Cellular mechanisms of epileptogenesis in immature brain

John Belmont, M.D. - CV genetics, cardiovascular malformations and connective tissue disorders

Robert Bryan, Ph.D. - Cerebrovascular circulation, EDHF in cerebral arteries, traumatic brain injury, K channels

Thomas Cooper, M.D. - Alternative splicing in cardiac development and disease

William Craigen, M.D. - Genetic disorders, metabolic disorders, mitochondrial function

Yuxin Fan, M.D., Ph.D. - Diagnostic tests for acquired and genetic cardiac diseases, viral myocarditis

Alica Goldman, M.D., Ph.D. - Genetic basis of epilepsy and channelopathies

Susan Hamilton, Ph.D. - Calcium handling, metabolic regulation ion channels

Craig Hartley, Ph.D. - Ultrasonic instrumentation for CV research, coronary blood flow

Frank Horrigan, Ph.D. - Mechanisms of ion channel gating, Ca-activated K channels

Jeffrey Jacot, Ph.D. - Regenerative therapies for congenital heart disease

Milan Jamrich, Ph.D. - Pattern formation and regeneration in mice and Xenopus

Kathryn Jones, DVM, Ph.D. - Chagas disease, vaccine development, animal models of heart disease

Seema Lalani, M.D. -* CHARGE syndrome, genetics of cardiovascular malformations

Hon-Chiu Leung, Ph.D. - Mass spectrometry

Na Li, Ph.D. - Atrial fibrillation mechanisms, microRNAs and arrhythmias, calcium handling

Sean Marrelli, Ph.D. - Regulation of cerebral blood flow, temperature-sensitive ion channels, KCa/TRP channels

James Martin, M.D., Ph.D. - Hippo, Wnt, Bmp signaling in development, regeneration, heart disease

Olga Medina-Martinez, Ph.D. - Molecular & cellular biology

Christina Miyake, MS, M.D. - Inherited cardiac arrhythmia syndromes

Jeffrey Neul, M.D., Ph.D. - Rett syndrome, neuronal regulation of cardiac function

Jeff Noebels, M.D. - Sudden unexpected death in epilepsy, channelopathies

Daniel Penny, M.D., Ph.D., MHA - Cardiovascular hemodynamics in children with congenital heart disease

Corey Reynolds, Ph.D. - Animal models of heart disease, CV imaging

Anilkumar Reddy, Ph.D. - Doppler and physiological monitoring of small animals, hypertrophy, cardiac remodeling

Christian Schaaf, M.D. - Rett syndrome, cholinergic regulation cardiac function

George Taffet, M.D. - Age-related changes in heart and vasculature, calcium handling, diastolic heart failure

Ming-Jer Tsai, Ph.D. - COUP-TFs and co-regulators in development and disease

Sophia Tsai, Ph.D. - COUP-TF and vein identity, angiogenesis, diabetes and atrial fibrillation

Chandrasekhar Yallampalli, DVM, Ph.D. - Regulation of lipid and lipoprotein metabolism