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Long QT Syndrome 6

Romano-Ward Syndrome


Presentation

  • Patients with this syndrome may present with syncopal or near-syncopal events that may be mistaken for seizures. Milder presentations may include palpitations or lightheadedness.[ahcmedia.com]
  • Children, who present with LQTS and seizures, generally have a precipitating event causing seizures, and they respond well to drug therapy.[springermedizin.de]
  • The initial case was in a patient who presented with seizures, ventricular fibrillation, and syncope. After a trial of conventional modalities of treatment including left stellate ganglionectomy, primidone (Mysoline) therapy was initiated.[annals.org]
  • Among patients enrolled in The International Long-QT registry, the average age of presentation is 21 15 years. Presentation most often is with a syncopal episode. Males often present during pre-adolescence while females present later [9].[geneticheartdisease.org]
  • Given the patient's presentation and persistently prolonged QT interval an implantable cardioverter defibrillator was implanted. Genetic testing and family counseling were performed.[innovationsincrm.com]
Chills
  • Matron GSH came with chief complains of Fever since 20-25 days Intermittent ,high grade asso. with chills n rigors Taking some oral medication from pvt.[slideshare.net]
Splenectomy
  • A 3-year-old had a splenectomy for which he received a single dose of pneumococcal vaccine, because asplenics have increased risk of fatal pneumococcal infection.[ahcmedia.com]
Sepsis
  • The decedent returned to the hospital on Sept. 19, 2000, with a fever and disorientation; he died of pneumococcal sepsis shortly later.[ahcmedia.com]
Hypoxemia
  • 37 C because hypothermia could theoretically prolong the QT interval through prolonged recovery of inactivated sodium channels. 39 No matter what anesthetic technique is used, light or insufficient anesthesia, hypertension, bradycardia, tachycardia, hypoxemia[anesthesiology.pubs.asahq.org]
Severe Clinical Course
  • Even though the clinical diagnosis of J-LN is rather straightforward, it is important to genotype all these patients because it has been shown by Schwartz et al. [ 3 ] that the smaller group with KCNE1 mutations has a markedly less severe clinical course[ojrd.biomedcentral.com]
Small Teeth
  • LQTS8 Also known as Timothy syndrome (TS), patients show severe QT prolongation on their ECGs, which is combined with syndactyly, baldness at birth, and small teeth in 100% of cases and less penetrant cardiac structural malformations, mental retardation[frontiersin.org]
Phenylketonuria
  • Issues in newborn screening for phenylketonuria. Am Fam Physician. 1999;60:1462-1466. 12. Leslie LK, Rodday AM, Saunders TS, et al. Cardiac screening prior to stimulant treatment of ADHD: a survey of US-based pediatricians.[uspharmacist.com]

Workup

  • The patient tolerated the surgery and post-operative period without complication and was scheduled for an outpatient workup and management for his prolonged QT interval.[the-hospitalist.org]
Hypercapnia
  • […] could theoretically prolong the QT interval through prolonged recovery of inactivated sodium channels. 39 No matter what anesthetic technique is used, light or insufficient anesthesia, hypertension, bradycardia, tachycardia, hypoxemia, and hypocapnia or hypercapnia[anesthesiology.pubs.asahq.org]
Prolonged QT Interval
  • Get Update Overview Congenital long QT syndrome is electrocardiographically characterized by a prolonged QT interval and polymorphic ventricular arrhythmias (torsade de pointes).[diseaseinfosearch.org]
  • QT interval and congenital deafness is seen. [18] Other rare forms include Anderson-Tawil syndrome (LQT7), which combines a prolonged QT interval with periodic paralysis and abnormalities of the face and skeleton; and Timothy syndrome (LQT8) which associates[en.wikipedia.org]
  • Figure 2: ECG post procainamide administration showing markedly prolonged QT interval. Figure 3: Pause dependent torsades (TdP) after administration of procainamide.[innovationsincrm.com]
  • QT interval) Syndrome - see also Disease Jervell-Lange-Nielsen I45.81 long QT I45.81 QT interval prolongation I45.81 Romano-Ward I45.81 (prolonged QT interval) ICD-10-CM Codes Adjacent To I45.81 I45.0 Right fascicular block I45.1 Other and unspecified[icd10data.com]
  • Complications Most of the time, prolonged QT intervals in people with long QT syndrome never cause problems. However, physical or emotional stress might "trip up" a heart that is sensitive to prolonged QT intervals.[mayoclinic.org]

Treatment

  • CLOSE Medical Disclaimer The medical information on this site is provided as an information resource only, and is not to be used or relied on for any diagnostic or treatment purposes.[diseaseinfosearch.org]
  • You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.[rarediseases.info.nih.gov]
  • After a trial of conventional modalities of treatment including left stellate ganglionectomy, primidone (Mysoline) therapy was initiated.[annals.org]
  • Standard Therapies Treatment is aimed at preventing symptoms such as syncope or cardiac arrest.[rarediseases.org]

Prognosis

  • LQT2 Gene T wave tends to be bifid and notched Additional Triger factors are sleep, auditory stimulation Respond to B-Blockers LQT3 Gene Poorest prognosis amongst all T wave is asymmetrical or Late biphasic Trigger factor : Bradycardia ( sleep ) B-Blockers[slideshare.net]
  • […] patients received a comprehensive 2- to 3-day clinical and genetic evaluation, including a 1- to 2-hour consultation with an LQTS specialist (M.J.A.) and additional consultations as needed. 3 - 5 Extensive counseling was provided to discuss individual prognosis[jamanetwork.com]
  • Prognosis The scary thing about long QT syndrome is that it can cause sudden death in otherwise healthy people.[drugs.com]
  • […] may experience symptoms from as early as the first months of life to as late as middle age. 2 LQTS can be detected in children through ECG, although widespread routine screening in asymptomatic patients is not currently recommended in the U.S. 7 The prognosis[uspharmacist.com]
  • Prognosis of LQTS in Children Previous reports showed that males with LQTS before the age of 15 years have a significant increased risk of syncope, aborted cardiac arrest, and sudden cardiac death. 12 However, the risk of these cardiac events is inverted[jstage.jst.go.jp]

Etiology

  • Either mutation etiology can lead to fatal arrhythmias, most notably secondary to TdP.[innovationsincrm.com]
  • Physicians must remain vigilant and consider LQTS as a possible etiology in patients with a history of syncope.[radcliffecardiology.com]
  • LQTS Subtypes: Jervell and Lange-Nielson and Romano-Ward Syndromes When LQTS was initially described, the genetic etiologies and electrophysiologic mechanisms underlying the disorder were not known.[lqts.research.chop.edu]
  • […] abnormal repolarization, usually lengthening the QT interval and thus predisposing to ventricular dysrhythmias.2 It is estimated that as many as 85% of these syndromes are inherited, and up to 15% are acquired or sporadic.3 Depending on the underlying etiology[the-hospitalist.org]
  • It presents when the QT interval is prolonged, regardless of the etiology. It is a polymorphic ventricular tachycardia due to reentry, characterized electrocardiographically by continuous twisting of the QRS axis around an imaginary line.[revespcardiol.org]

Epidemiology

  • Epidemiological studies of SIDS have shown some link to sedative medications, but not yet to ingestion of QT prolonging medications.[adc.bmj.com]
  • Epidemiology: In the United States, the incidence of congenital LQTS is estimated to be one in 7,000-10,000 [5] [6]. There is a female preponderance, ranging from 1.6-2.0:1 [7] [8].[geneticheartdisease.org]
  • […] who are untreated, the risk of death within 15 years is around 50%. [6] With careful treatment this decreases to less than 1% over 20 years. [3] Those who exhibit symptoms before the age of 18 are more likely to experience a cardiac arrest. [18] [36] Epidemiology[en.wikipedia.org]
  • American Journal of Epidemiology, 170, 739-746. doi:10.1093/aje/kwp191 Van Noord, C., Sturkenboom, M.C., Straus, S.M., Hofman, A., Kors, J.A., Witteman, J.C. and Stricker, B.H. (2010).[file.scirp.org]
  • Sudden infant death syndrome and long QT syndrome: an epidemiological and genetic study. Int J Legal Med. 2006; 120 :129–37. [ PubMed : 16012827 ] Westenskow P, Splawski I, Timothy KW, Keating MT, Sanguinetti MC.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology: The ventricular myocyte activation cycle, or action potential, is dependent on ion channels (Figure 2).[geneticheartdisease.org]
  • In this review, we will describe exclusively on LQTS linked arrhythmias, its pathophysiology and presently available clinical management.[frontiersin.org]
  • Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Long QT syndrome (LQTS) is a genetic cardiac disorder characterized by QT prolongation and T-wave abnormalities on electrocardiogram[mayomedicallaboratories.com]
  • "Molecular Pathophysiology of Congenital Long QT Syndrome". Physiological Reviews. 97 (1): 89–134. doi : 10.1152/physrev.00008.2016. PMC 5539372. PMID 27807201. a b Giudicessi JR, Ackerman MJ (October 2013).[en.wikipedia.org]
  • The pathophysiological basis of long-QT syndrome (LQTS) is prolongation of cardiac ventricular repolarization by acquired factors such as drug exposure or genetic variation in the proteins controlling transmembrane ion-concentration gradients ( 8 , 9[pnas.org]

Prevention

  • Appropriate therapy will prevent unnecessary deaths in infants, children, and young adults.[omicsonline.org]
  • Prophylactic β-blocker therapy was started in these two patients to prevent sudden death. In conclusion, this syndrome should be sought in children with syncope or a family history of it, especially those with congenital deafness.[link.springer.com]
  • (November 2015). "2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac[en.wikipedia.org]
  • Beta blocker therapy also may be used acutely to suppress catecholamines, thereby preventing further dysrhythmia.[ahcmedia.com]
  • NHLBI is part of the National Institutes of Health and supports research, training, and education for the prevention and treatment of heart, lung, and blood diseases. In-Depth Information Medscape Reference provides information on this topic.[rarediseases.info.nih.gov]

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