The Short QT Syndrome
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WPW: WOLFF-PARKINSON-WHITE Syndrome
WPW: WOLFF-PARKINSON-WHITE Syndrome What is Wolff-Parkinson-White Syndrome? Wolff-Parkinson-White Syndrome, or WPW, is named for three physicians who described a syndrome in 1930 in young people with episodes of heart racing and an abnormal pattern on their electrocardiogram (ECG or EKG). Over the next few decades, it was discovered that this ECG pattern and the heart racing was due to an extra electrical pathway in the heart. Thus, WPW is a syndrome associated with an abnormal heart rhythm, or “arrhythmia”. Most people with WPW do not have any other problems with their heart. Normally, the electrical impulses in the heart originate in the atria or top chambers of the heart and spread across the atria. The electrical impulses are then conducted to the ventricles (the pumping/bottom chambers of the heart) through a group of specialized cells called the atrioventricular node or AV node. This is usually the only electrical pathway between the atria and ventricles. In WPW, there is an additional pathway made up of a few extra cells left over from when the heart formed. The conduction of electricity through the heart causes the contractions which are the “heartbeat”. What is WPW Syndrome as opposed to a WPW ECG? A person has WPW Syndrome if they experience symptoms from abnormal conduction through the heart by the WPW pathway. Most commonly, the symptom is heart racing, or “palpitations”. The particular type of arrhythmia in WPW is called “supraventricular tachycardia” or SVT. “Tachycardia” means fast heart rate; “supraventricular” means the arrhythmia requires the cells above the ventricles to be part of the abnormal circuit. -
Non Commercial Use Only
Cardiogenetics 2017; volume 7:6304 Sudden death in a young patient with atrial fibrillation Case Report Correspondence: María Angeles Espinosa Castro, Inherited Cardiovascular Disease A 22-year-old man suffered a sudden Program, Cardiology Department, Gregorio María Tamargo, cardiac arrest without previous symptoms Marañón Hospital, Dr. Esquerdo, 46, 28007, María Ángeles Espinosa, while he was at rest, waiting for a subway Madrid, Spain. Víctor Gómez-Carrillo, Miriam Juárez, train. Cardiopulmonary resuscitation was Tel.: +34.91.586.82.90. immediately started using an Automated E-mail: [email protected] Francisco Fernández-Avilés, External Defibrillation that identified the Raquel Yotti Key words: KCNQ1; mutation; channelopa- presence of ventricular fibrillation and thy; sudden cardiac death; atrial fibrillation. Inherited Cardiovascular Disease delivered a shock. Return of spontaneous Program, Cardiology Department, circulation was achieved after three Contributions: MT, acquisition and interpreta- Gregorio Marañón Hospital, Madrid, attempts, being atrial fibrillation (AF) the tion of data for the work, ensuring that ques- Spain patient’s rhythm at this point (Figure 1). tions related to the accuracy or integrity of any He was admitted to our Cardiovascular part of the work is appropriately investigated Intensive Care Unit and therapeutic and resolved; MAE, conception of the work, hypothermia was performed over a period critical revision of the intellectual content, final approval of the version to be published, Abstract of 24 h. After completing hypothermia, ensuring that questions related to the accuracy rewarming, and another 24 h of controlled of any part of the work is appropriately inves- Sudden cardiac death (SCD) in young normothermia the patient awakened with no tigated and resolved; VG-C, acquisition and patients without structural heart disease is residual neurologic damage. -
Mitral Valve Prolapse, Arrhythmias, and Sudden Cardiac Death: the Role of Multimodality Imaging to Detect High-Risk Features
diagnostics Review Mitral Valve Prolapse, Arrhythmias, and Sudden Cardiac Death: The Role of Multimodality Imaging to Detect High-Risk Features Anna Giulia Pavon 1,2,*, Pierre Monney 1,2,3 and Juerg Schwitter 1,2,3 1 Cardiac MR Center (CRMC), Lausanne University Hospital (CHUV), 1100 Lausanne, Switzerland; [email protected] (P.M.); [email protected] (J.S.) 2 Cardiovascular Department, Division of Cardiology, Lausanne University Hospital (CHUV), 1100 Lausanne, Switzerland 3 Faculty of Biology and Medicine, University of Lausanne (UniL), 1100 Lausanne, Switzerland * Correspondence: [email protected]; Tel.: +41-775-566-983 Abstract: Mitral valve prolapse (MVP) was first described in the 1960s, and it is usually a benign condition. However, a subtype of patients are known to have a higher incidence of ventricular arrhythmias and sudden cardiac death, the so called “arrhythmic MVP.” In recent years, several studies have been published to identify the most important clinical features to distinguish the benign form from the potentially lethal one in order to personalize patient’s treatment and follow-up. In this review, we specifically focused on red flags for increased arrhythmic risk to whom the cardiologist must be aware of while performing a cardiovascular imaging evaluation in patients with MVP. Keywords: mitral valve prolapse; arrhythmias; cardiovascular magnetic resonance Citation: Pavon, A.G.; Monney, P.; Schwitter, J. Mitral Valve Prolapse, Arrhythmias, and Sudden Cardiac Death: The Role of Multimodality 1. Mitral Valve and Arrhythmias: A Long Story Short Imaging to Detect High-Risk Features. In the recent years, the scientific community has begun to pay increasing attention Diagnostics 2021, 11, 683. -
Long QT Syndrome: from Channels to Cardiac Arrhythmias
Long QT syndrome: from channels to cardiac arrhythmias Arthur J. Moss, Robert S. Kass J Clin Invest. 2005;115(8):2018-2024. https://doi.org/10.1172/JCI25537. Review Series Long QT syndrome, a rare genetic disorder associated with life-threatening arrhythmias, has provided a wealth of information about fundamental mechanisms underlying human cardiac electrophysiology that has come about because of truly collaborative interactions between clinical and basic scientists. Our understanding of the mechanisms that control the critical plateau and repolarization phases of the human ventricular action potential has been raised to new levels through these studies, which have clarified the manner in which both potassium and sodium channels regulate this critical period of electrical activity. Find the latest version: https://jci.me/25537/pdf Review series Long QT syndrome: from channels to cardiac arrhythmias Arthur J. Moss1 and Robert S. Kass2 1Heart Research Follow-up Program, Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York, USA. 2Department of Pharmacology, Columbia University Medical Center, New York, New York, USA. Long QT syndrome, a rare genetic disorder associated with life-threatening arrhythmias, has provided a wealth of information about fundamental mechanisms underlying human cardiac electrophysiology that has come about because of truly collaborative interactions between clinical and basic scientists. Our understanding of the mecha- nisms that control the critical plateau and repolarization phases of the human ventricular action potential has been raised to new levels through these studies, which have clarified the manner in which both potassium and sodium channels regulate this critical period of electrical activity. -
Effects of Nifekalant Injected Into the Pericardial Space on the Transmural Dispersion of Repolarization in Pig
Showa Univ J Med Sci 19(3), 123 135, September 2007 Original Effects of Nifekalant Injected into the Pericardial Space on the Transmural Dispersion of Repolarization in Pig Hiroyuki ITo, Taku ASANO, Youichi KOBAYASHI, Tatsuya ONUKI, Fumito MwosHI, Taka-aki MATSUYAMA, Yoshino MINOURA, Norikazu WATANABE, Mitsuharu KAWAMURA, Kaoru TANNO and Takashl KATAGIRI Abstract: Transmural dispersion of repolarization (TDR) has been implicated in the onset of ventricular arrhythmia. We investigated the effects of nifekalant (NIF) injected into the pericardial space on TDR and T wave in pig. We injected 50 or 100 mg of NIF into the pericardial space of 11 pigs, and measured the effective refractory period (ERP) between the endocardial and epicardial myocardial cells, as well as QT time and QT peak-end as an index of TDR and T waveforms, respectively. TDR decreased from 56•} 10 msec to 44•}8 msec (P < 0.01), 5.1 min after injection of 100 mg of NIF, although the QTc did not change. At a later time, QTc increased from 457 •} 44 msec before injection to 540•}49 msec (P < 0.01) and TDR recovered to the control level. When 50 mg of NIF was injected, the T wave amplitude decreased from 0.433 •}0.301 mV before injection to 0.107•}0.192 mV (P < 0.01) at 10 min after injection ; 100 mg of NIF caused the T wave amplitude to decrease more rapidly, reaching a negative value at 4.5 min after injection. Injecting NIF into the pericardial space altered ERP, QT, and T waveform, and also decreased TDR. -
Neurotransmitter Actions
Central University of South Bihar Panchanpur, Gaya, India E-Learning Resources Department of Biotechnology NB: These materials are taken/borrowed/modified/compiled from various resources like research articles and freely available internet websites, and are meant to be used solely for the teaching purpose in a public university, and for serving the needs of specified educational programmes. Dr. Jawaid Ahsan Assistant Professor Department of Biotechnology Central University of South Bihar (CUSB) Course Code: MSBTN2003E04 Course Name: Neuroscience Neurotransmitter Actions • Excitatory Action: – A neurotransmitter that puts a neuron closer to an action potential (facilitation) or causes an action potential • Inhibitory Action: – A neurotransmitter that moves a neuron further away from an action potential • Response of neuron: – Responds according to the sum of all the neurotransmitters received at one time Neurotransmitters • Acetylcholine • Monoamines – modified amino acids • Amino acids • Neuropeptides- short chains of amino acids • Depression: – Caused by the imbalances of neurotransmitters • Many drugs imitate neurotransmitters – Ex: Prozac, zoloft, alcohol, drugs, tobacco Release of Neurotransmitters • When an action potential reaches the end of an axon, Ca+ channels in the neuron open • Causes Ca+ to rush in – Cause the synaptic vesicles to fuse with the cell membrane – Release the neurotransmitters into the synaptic cleft • After binding, neurotransmitters will either: – Be destroyed in the synaptic cleft OR – Taken back in to surrounding neurons (reuptake) Excitable cells: Definition: Refers to the ability of some cells to be electrically excited resulting in the generation of action potentials. Neurons, muscle cells (skeletal, cardiac, and smooth), and some endocrine cells (e.g., insulin- releasing pancreatic β cells) are excitable cells. -
Basic Arrhythmia Review Guide-Advanced
BASIC ARRHYTHMIA REVIEW GUIDE ADVANCED The following study guide provides a review of information covered in the basic arrhythmia competency. Preparation with this guide will help to achieve success on the exam. Sample questions and websites are provided at the end of this guide. DESCRIPTION OF THE HEART The adult heart is a muscular organ weighing less than a pound and about the size of a clenched fist. It lies between the right and Left left lung in an area called the mediastinal cavity behind the sternum of the breast bone. Approximately two-thirds of the heart Atrium lies to the left of the sternum and one-third to the right of the sternum. Right HEART MUSCLES Atrium The heart is composed of three layers each with its own special function. The outermost layer is called the pericardium, essentially a sac around the heart. The middle and thickest layer of the heart is called the Left myocardium. This layer contains all the atrial and ventricular Ventricle muscle fibers needed for contraction as well as the blood supply Right and electrical conduction system. Ventricle The innermost layer of the heart is the endocardium and is composed of endothelium and connective tissue. Any disruption or injury to this endothelium can lead to infection, which in turn can cause valve damage, sepsis, or death. CHAMBERS A normal human heart contains four separate chambers: right atrium, left atrium, right ventricle, and left ventricle. The right and left sides of the heart are divided by a septum. The right atrium (RA) receives oxygen-poor (venous) blood from the body’s organs via the superior and inferior vena cava (SVC and IVC). -
Tachycardia (Fast Heart Rate)
Tachycardia (fast heart rate) Working together to improve the diagnosis, treatment and quality of life for all those aff ected by arrhythmias www.heartrhythmalliance.org Registered Charity No. 1107496 Glossary Atrium Top chambers of the heart that receive Contents blood from the body and from the lungs. The right atrium is where the heart’s natural pacemaker (sino The normal electrical atrial node) can be found system of the heart Arrhythmia An abnormal heart rhythm What are arrhythmias? Bradycardia A slow heart rate, normally less than 60 beats per minute How do I know what arrhythmia I have? Cardiac Arrest the abrupt loss of heart function, breathing and consciousness Types of arrhythmia Cardioversion a procedure used to return an abnormal What treatments are heartbeat to a normal rhythm available to me? Defi brillation a treatment for life-threatening cardiac arrhythmias. A defibrillator delivers a dose of electric current to the heart Important information This booklet is intended for use by people who wish to understand more about Tachycardia. The information within this booklet comes from research and previous patients’ experiences. The booklet off ers an explanation of Tachycardia and how it is treated. This booklet should be used in addition to the information given to you by doctors, nurses and physiologists. If you have any questions about any of the information given in this booklet, please ask your nurse, doctor or cardiac physiologist. 2 Heart attack A medical emergency in which the blood supply to the heart is blocked, causing serious damage or even death of heart muscle Tachycardia Fast heart rate, more than 100 beats per minute Ventricles The two lower chambers of the heart. -
Genetic Testing for Hereditary Cardiac Disease
Clinical Appropriateness Guidelines Genetic Testing for Hereditary Cardiac Disease EFFECTIVE MARCH 8, 2021 Appropriate.Safe.Affordable © 2019 AIM Specialty Health 2064-0319 Table of Contents Scope .......................................................................................................................................................... 3 Genetic Counseling Requirement ............................................................................................................... 3 Appropriate Use Criteria.............................................................................................................................. 3 Confirmation/Diagnostic Testing of Affected Individuals .............................................................................. 4 Testing of Asymptomatic Individuals .............................................................................................................. 4 Post-Mortem Testing ........................................................................................................................................ 4 Long QT ............................................................................................................................................................. 5 Dilated Cardiomyopathy .................................................................................................................................. 5 Tests Not Clinically Appropriate ..................................................................................................................... -
The Example of Short QT Syndrome Jules C
Hancox et al. Journal of Congenital Cardiology (2019) 3:3 Journal of https://doi.org/10.1186/s40949-019-0024-7 Congenital Cardiology REVIEW Open Access Learning from studying very rare cardiac conditions: the example of short QT syndrome Jules C. Hancox1,4* , Dominic G. Whittaker2,3, Henggui Zhang4 and Alan G. Stuart5,6 Abstract Background: Some congenital heart conditions are very rare. In a climate of limited resources, a viewpoint could be advanced that identifying diagnostic criteria for such conditions and, through empiricism, effective treatments should suffice and that extensive mechanistic research is unnecessary. Taking the rare but dangerous short QT syndrome (SQTS) as an example, this article makes the case for the imperative to study such rare conditions, highlighting that this yields substantial and sometimes unanticipated benefits. Genetic forms of SQTS are rare, but the condition may be under-diagnosed and carries a risk of sudden death. Genotyping of SQTS patients has led to identification of clear ion channel/transporter culprits in < 30% of cases, highlighting a role for as yet unidentified modulators of repolarization. For example, recent exome sequencing in SQTS has identified SLC4A3 as a novel modifier of ventricular repolarization. The need to distinguish “healthy” from “unhealthy” short QT intervals has led to a search for additional markers of arrhythmia risk. Some overlap may exist between SQTS, Brugada Syndrome, early repolarization and sinus bradycardia. Genotype-phenotype studies have led to identification of arrhythmia substrates and both realistic and theoretical pharmacological approaches for particular forms of SQTS. In turn this has increased understanding of underlying cardiac ion channels. -
An Overview on the Short Qt Interval in Childhood
Journal of Cardiology & Current Research An Overview on The Short Qt Interval in Childhood Overview on the Topic Editorial The QT interval on the electrocardiogram involves both the electrical depolarization (QRS complex) as the electrical Volume 2 Issue 2 - 2015 change in the length or voltage of the same could have serious Francisco R Breijo-Marquez* repolarization of the ventricles (T wave). Any significant Department of Clinical & Experimental Cardiology, USA alteration in the ECG in which such interval is shortened to its normalconsequences. length; Classically,therefore, it the is a short sign and QT notinterval a symptom. is defined So muchas an *Corresponding author: Francisco R Breijo-Marquez, Department of Clinical & Experimental Cardiology, East so that, there may be patients with such electrical disturbance Boston Hospital, School of Medicine, 02136 Boston, and without showing any symptoms throughout their lives. Massachusetts, USA, Email: The short QT syndrome (SQTS) is the set of symptoms Received: March 02, 2015 | Published: March 04, 2015 presented by a patient who has a shortening of the QT interval on the ECG. It is therefore the set of symptoms and signs, in this case the QT interval shortening. (Whenever we speak of “syndrome”, we mean to the set of symptoms (subjective appreciation) and signs (objective appreciation) observed in any disease. treatment of the same to date [2,3]. This syndrome is a cardiac channelopathy associated with a Currently, there is not any unanimity among different authors on what should be the limits of the length of the QT interval predispositionThe diagnostic to atrial hallmark fibrillation of the condition and sudden remains cardiac a short death. -
A 13-Year-Old Boy with a Short QT Interval
Olgu Sunumları Anadolu Kardiyol Derg 274 Case Reports 2012; 12: 272-5 3. Glicklich D, Figura I. Vancomycin and cardiac arrest. Ann Intern Med 1984; 101: 880. 4. Mayhew JF Deutsch S. Cardiac arrest following administration of vancomy- cin. Can Anaesth Soc J 1985; 32: 65-6. [CrossRef] 5. Dajee H, Laks H, Miller J, Oren R. Profound hypotension from rapid vancomy- cin administration during cardiac operation. J Thorac Cardiovasc Surg 1984; 87: 145-6. 6. Boussemart T, Cardona J, Berthier M, Chevrel J, Oriot D. Cardiac arrest asso- ciated with vancomycin in a neonate. Arch Dis Child Fetal Neonatal Ed 1995; 73: 123. [CrossRef] Yaz›şma Adresi/Address for Correspondence: Dr. Nurettin Yeral Mustafa Kemal Üniversitesi Tıp Fakültesi, Araştırma ve Uygulama Hastanesi, Kardiyoloji Anabilim Dalı, Hatay-Türkiye Tel: +90 326 263 85 34 Faks: +90 284 513 40 20 E-posta: [email protected] Çevrimici Yayın Tarihi/Available Online Date: 13.03.2012 Figure 1. ECG with short QT interval (QTc=300 ms) and tall peaked T waves ©Telif Hakk› 2012 AVES Yay›nc›l›k Ltd. Şti. - Makale metnine www.anakarder.com web ECG - electrocardiogram sayfas›ndan ulaş›labilir. nal uncle had died suddenly at the age of 28 years with a possible ©Copyright 2012 by AVES Yay›nc›l›k Ltd. - Available on-line at www.anakarder.com diagnosis of myocardial infarction but any record of ECG was not pres- doi:10.5152/akd.2012.074 ent and history of symptoms of arrhythmia had not been learned. Family screening with ECGs revealed that his mother, father and sister had T-waves that were morphologically identical to his, with tall, symmetric, peaked T-waves.