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A Review of Long QT Syndrome: Everything a Hospitalist Should Know Nandita Sharma, MD,A Daniel Cortez, MD,B,C Kristin Disori, MD,D Jason R

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A Review of Long QT Syndrome: Everything a Hospitalist Should Know Nandita Sharma, MD,A Daniel Cortez, MD,B,C Kristin Disori, MD,D Jason R REVIEW ARTICLE A Review of Long QT Syndrome: Everything a Hospitalist Should Know Nandita Sharma, MD,a Daniel Cortez, MD,b,c Kristin Disori, MD,d Jason R. Imundo, MD,a Michael Beck, MDd ABSTRACT In this article, we will review various aspects of long QT syndrome (LQTS) necessary for hospitalists who care for children, adolescents, and young adults who have known LQTS and also review presenting features that should make one consider LQTS as a cause of hospitalization. Pediatric hospitalists care for patients who have suffered near-drowning, unexplained motor vehicular accidents, brief resolved unexpected events, sudden infant death syndrome, recurrent miscarriages, syncope, or seizures. These common conditions can be clinical clues in patients harboring 1 of 16 LQTS genetic mutations. LQTS is commonly caused by a channelopathy that can cause sudden cardiac death. Over the years, guidelines on management and recommendations for sports participation have evolved with our understanding of the disease and the burden of arrhythmias manifested in the pediatric age group. This review will include the genetic causes of LQTS, clinical features, and important historical information to obtain when these presentations are encountered. We will review medical and surgical treatments available to patients with LQTS and long-term care recommendations and prognosis for those diagnosed with LQTS. a Divisions of Pediatric www.hospitalpediatrics.org d Cardiology and Pediatric DOI:https://doi.org/10.1542/hpeds.2019-0139 Hospital Medicine, Copyright © 2020 by the American Academy of Pediatrics Department of Pediatrics, Penn State Children’s Address correspondence to Michael Beck, MD, Division of Pediatric Hospital Medicine, Department of Pediatrics, Penn State Children’s Hospital, Hershey, Hospital, 500 University Dr, Hershey, PA 17033-0850. E-mail: [email protected] Pennsylvania; bDivision of Adult Electrophysiology, HOSPITAL PEDIATRICS (ISSN Numbers: Print, 2154-1663; Online, 2154-1671). Department of FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. Cardiology, Penn State Medical Center, Hershey, FUNDING: No external funding. Pennsylvania; and POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose. cDepartment of Cardiology, University of Dr Sharma conceptualized and drafted the initial manuscript; Drs Cortez, Disori, Imundo, and Beck reviewed and revised the Lund, Lund, Sweden manuscript; and all authors approved the final manuscript as submitted. HOSPITAL PEDIATRICS Volume 10, Issue 4, April 2020 1 Downloaded from www.aappublications.org/news by guest on September 29, 2021 Long QT syndrome (LQTS) is more than a BACKGROUND effect of drugs or electrolyte abnormalities, prolongation of the QT segment of the Although a less common etiology of LQTS which can interact with the gene related to electrocardiogram (ECG). As the name compared with acquired causes, congenital LQTS2 (hERG).10,11 “syndrome” implies, a patient should have a LQTS is most commonly caused by an prolonged QTc, plus other ECG abnormalities underlying cardiac channelopathy. LQTS is GENETICS per Schwartz criteria (reviewed later), plus estimated to affect 1 in 2000 people and is Congenital LQTS results from mutations fi responsible for ∼4000 deaths per year in speci c clinical and family history. Failure to involving 1 of the many genes coding for ion the United States.1,2,3 Its clinical include these elements in medical decision- channels that facilitate the transport of manifestations are estimated to be less making to commonly encountered clinical potassium, sodium, and calcium. These ion scenarios frequently seen by pediatric common (1 in 5000) because most mutation carriers remain asymptomatic.4 channels play critical roles in maintaining hospitalists can lead to missed the heart’s normal rhythm. Despite having In 1957, Jervell and Lange-Nielsen5 were the opportunities to diagnosis this treatable at least 16 genetically distinct types of LQTS, and potentially fatal disease. The diagnosis first to document this syndrome in Norway at least 75% of the mutations are found in typically receives consideration when a in a family with children who were deaf and 3 genes: KCNQ1 (potassium channel), KCNH2 screening ECG demonstrates QT segment had QT prolongation, syncope, and death at a young age. This association came to be (potassium channel), and SCN5A (sodium prolongation or polymorphic ventricular known as Jervell and Lange-Nielsen channel) causing long QT (LQT) type 1, LQT2, tachycardia known as torsade-de-pointes 1 syndrome and has an autosomal recessive and LQT3, respectively. (Tdp). This rhythm can degenerate into pattern of inheritance. More commonly, Often Romano-Ward syndrome is used ventricular fibrillation and cause sudden LQTS is inherited in an autosomal dominant interchangeably with LQTS, but it is cardiac death and present as syncope and/ pattern and is associated with normal becoming increasingly common to or collapse. LQTS can result from congenital hearing, described as Romano-Ward reference the subtype according to the or acquired causes. Congenital causes syndrome in 1964.6,7 Although initially underlying genetic mutation (LQT1 to LQT16). occur less frequently than acquired and are described in 1957, much of the research The Jervell and Lange-Nielsen syndrome is due to the presence of genetic mutation(s) contributing to a better understanding caused by mutations in either KCNQ1/LQT1 that affect 1 of the several channels involved (90%) or KCNE1/LQT5 (10%). These genes of the disease has occurred only in with ventricular repolarization. Acquired encode components of the potassium the past 2 decades. As of 2016, causes can be secondary to electrolyte channel, which are critical for function of .500 different mutations have been abnormalities like hypocalcemia, both inner ear and cardiac conduction.9 identified in the 16 genes currently known hypokalemia, hypomagnesemia, drug side LQT1 is the most common of the 3 subtypes 1,8,9 effect, or drug–drug interaction. Both to cause LQTS. and has the highest incidence of cardiac causes of LQTS and familiarization of its Acquired causes of LQTS are far more events or symptomatic patients at 63%, protean manifestations are important for prevalent then congenital causes of LQTS. followed by 46% in LQT2 and 18% in LQT3. any hospitalist to understand. Most acquired causes result from adverse However, the likelihood of dying during a TABLE 1 Distinguishing Features of LQTS For the 3 Most Common Genetic Mutations Genotype LQT1 LQT2 LQT3 Genetics KCNQ1 KCNH2 SCNA5 Frequency, % 35–45 30–35 8–10 Function Loss Loss Gain Ion current affected ↓ IKs ↓ IKr ↑ INa Incidence of cardiac events22,% 63 46 18 Likelihood of dying during a 4420 cardiac event22,% Triggers21 Swimming, exercise, Loud noises, emotion, Sleep adrenergic stimuli peripartum period ECG Broad-based T wave Low-amplitude bifid T wave Long isoelectric ST segment Response to b-blockade21,33 111 11 1 Response to mexilitine21 1111 Exercise restriction 111 11 Uncertain Exercise-triggered events22,% 68 29 4 1, low likelihood; 11, moderate likelihood; 111, high likelihood. 2 SHARMA et al Downloaded from www.aappublications.org/news by guest on September 29, 2021 LQT3 event is 5 times more compared with TABLE 2 Differentiating Characteristics of Presenting Symptoms of LQTS and Vasovagal others.12 (Table 1) Syncope LQTS Vasovagal Syncope CLINICAL PRESENTATION Sudden onset syncope Aura first, followed by dizziness or nausea Now that we have reviewed the genetics of During exercise During cooldown after exercise LQTS, hospitalists need to be made aware of Swimming, exercise, emotionally, loud noise Typically orthostatic or emotionally induced what information to seek when obtaining a induced family history on patients who they take May present as convulsive event May present as convulsive event care of and the conditions that mimic LQTS. LQTS has a variety of clinical manifestations, many of which can find their way to syncope. In patients who experience unexplained sudden cardiac death in the hospitalist services. Patients with LQTS vasovagal syncope, typically an aura occurs young, 10% to 15% of sudden infant death typically present with syncope, fainting before syncope. In contrast, arrhythmia- syndrome (SIDS), and 9% of miscarried spells, brief resolved unexplained events, mediated syncope may have sudden loss of fetuses may be attributable to mutations in sudden cardiac death, near-drowning consciousness or occur after a brief either LQTS or catecholaminergic events, aborted cardiac arrest, recurrent prodrome of palpitations.15 Syncope that polymorphic ventricular tachycardia – syncopal episodes, drop attacks, narcolepsy, occurs during exertion (not during the susceptibility genes.16 20 or epilepsy.13 Although the cardiac events cooldown phase of exercise) should always may occur at any age from infancy through Family History be concerning and should alert the middle age, they are most common from the We would like to emphasize to hospitalists hospitalist to consider LQTS as an etiology preteen years through the 20s. Of the the importance of obtaining a family history and thus consider involving a pediatric individuals who do become symptomatic, that covers 3 generations when called to cardiologist. 50% experience their first cardiac event by evaluate patients who present with any of the age of 12 years and 90% present by the Because seizures
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