The time of presentation varied from 16 to 38 weeks of gestation. [karger.com]

There are several reasons behind this, the main one being not considering that long QT may be present. [radcliffecardiology.com]

TEST CHARACTERISTICS 2.1 Analytical sensitivity (proportion of positive tests if the genotype is present) Close to 100% if complete Sanger sequencing and MLPA of the affected gene is performed. [nature.com]

Long QT syndrome (LQTS) should be suspected in individuals on the basis of ECG characteristics, clinical presentation, and family history. [centogene.com]

This is likely an underestimate as sudden death with a normal autopsy may be the first and only presentation. There are 12 genes responsible for 3 main types of c-LQTS. The presentation is one of aborted sudden death or brief arrhythmia. [epmonthly.com]

[…] the systemic circulation. [1] Clinically, the syndrome is characterized by rapid development of hyperuricemia,hyperkalemia, hyperphosphatemia, hypocalcemia, and acute kidney injury. [2, 3] (See Pathophysiology, Etiology, Prognosis, Presentation, and Workup [emedicine.medscape.com]

• Prolonged QT Interval

  It's especially important to avoid medications that can affect the heart rhythm and cause a prolonged QT interval. There is no known prevention for congenital long QT syndrome. [mayoclinic.org]

  In summary, inhaled anticholinergic agents do not result in prolongation of QT interval and inhaled beta agonists do variably prolong the interval. [aaaai.org]

  Syndrome (LQTS) is a rare hereditary disease that is characterized by a prolonged QT-interval on the electrocardiogram (ECG) due to delayed repolarization of the heart. [asperbio.com]

  Overview People with LQTS have prolonged QT intervals on their ECGs. The QT interval represents repolarization, or "recharge," of a cardiac cell. [verywell.com]

  Discussion These cases highlight the inability of attending physicians to at least suspect long QT interval on ECG. Failure to recognize prolonged QT interval has been described before. [radcliffecardiology.com]

• Ventricular Repolarization Abnormalities

  RESULTS: Compared with the analysis of the healthy group (n = 8), age- and sex-matched pair analysis revealed that the mutation carriers (n = 8) had ventricular repolarization abnormality results in the prolongation of corrected QT and QTpeak intervals [ncbi.nlm.nih.gov]

Recent studies suggest feasibility of genotype specific treatment of LQTS and, in near future, mutation specific treatment will probably become a novel approach to this potentially fatal syndrome. [jtd.amegroups.com]

Standard Therapies Treatment is aimed at preventing symptoms such as syncope or cardiac arrest. [rarediseases.org]

With proper treatment, you can manage and prevent the dangerous heartbeats that can lead to long QT syndrome complications. [mayoclinic.org]

The ICD is the remaining treatment option. A device is implanted that includes a computer, battery, and leads that are attached to the heart. [athletesheart.org]

With this innovative discovery of a new treatment in long QT syndrome 2, it marks a big step towards personalised medicine. "Currently, we have been successful in providing a medicine to treat long QT syndrome 2. [news-medical.net]

[…] and metabolic byproducts into the systemic circulation. [1] Clinically, the syndrome is characterized by rapid development of hyperuricemia,hyperkalemia, hyperphosphatemia, hypocalcemia, and acute kidney injury. [2, 3] (See Pathophysiology, Etiology, Prognosis [emedicine.medscape.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]

Prognosis LQTS is a life-long condition. Individuals who are not diagnosed and treated are at an increased risk of syncope and sudden death. Adequate treatment can decrease this risk. There is no cure. [medical-dictionary.thefreedictionary.com]

In addition to an improved prognosis, we can better prepare for changes in a patient’s condition and triage them to more appropriate receiving facilities. How Do I Detect LQTS? As the name implies, simply measure the QT/QTc interval. [emsworld.com]

Female patients with a mutation at the LQT2 locus had a more severe prognosis irrespective of the duration of the QTc, whereas in patients with a mutation at the LQT3 locus, the prognosis was mainly influenced by sex: male patients had a higher probability [nejm.org]

Etiology Tumor lysis syndrome occurs most often in patients with acute leukemia with high white blood cell (WBC) counts and in those with high-grade lymphomas in response to aggressive treatment. [emedicine.medscape.com]

Physicians must remain vigilant and consider LQTS as a possible etiology in patients with a history of syncope. [radcliffecardiology.com]

Management of Polymorphic VT (PMVT) Non-Torsades Etiologies of PMVT (normal QT) Most commonly due to ischemia. These will almost always be overt, severe episodes of ischemia, with chest pain and/or unequivocal ischemic ECG abnormalities. [hqmeded-ecg.blogspot.com]

[…] taught in most EMS curricula and may be overlooked. 1 This condition is identified by a long QT interval on an electrocardiogram (ECG) and may be congenital or medication-induced. 2 An increasing number of sudden cardiac arrests with previously unknown etiology [emsworld.com]

Reed Kl: Fetal arrhythmias, etiology, diagnosis, pathophysiology and treatment. Semin Perinatol 1989;13:294–304. [karger.com]

Epidemiological studies of SIDS have shown some link to sedative medications, but not yet to ingestion of QT prolonging medications. [adc.bmj.com]

and 3) patients with implantable cardioverter-defibrillators (ICD)s. 2 On the other hand, for European athletes with LQTS, sports participation is still regarded as taboo irrespective of their genotype/phenotype relationship or clinical history. 3 Epidemiology [acc.org]

Epidemiology International occurrence The incidence of tumor lysis syndrome is unknown. The prevalence varies among different malignancies; bulky, aggressive, treatment-sensitive tumors are associated with higher frequencies of tumor lysis syndrome. [emedicine.medscape.com]

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]

Pathophysiology Rapid tumor cell turnover results in release of intracellular contents into the circulation. This release can inundate renal elimination and cellular buffering mechanisms, leading to numerous metabolic derangements. [emedicine.medscape.com]

The pathophysiology of CPVT is very different from Long QT syndrome. CPVT, unlike Torsade, is worsened with tachycardia. See this post for management of Torsade. [hqmeded-ecg.blogspot.com]

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]

In this review, we will describe exclusively on LQTS linked arrhythmias, its pathophysiology and presently available clinical management. [frontiersin.org]

Reed Kl: Fetal arrhythmias, etiology, diagnosis, pathophysiology and treatment. Semin Perinatol 1989;13:294–304. [karger.com]

Prevention Regular health checkups and good communication with your health care provider may help prevent health conditions that lead to some types of acquired long QT syndrome. [mayoclinic.org]

For polymorphic VT due to acquired long QT (Torsade de Pointe), isoproterenol is indicated to increase the sinus rate and prevent further torsade. However, this makes CPVT worse. [hqmeded-ecg.blogspot.com]

Long QT Syndrome, Risk Assessment, Secondary Prevention, Torsades de Pointes < Back to Listings [acc.org]

To prevent this stimulus, pretreatment with fentanyl was done during induction of anaesthesia. [ispub.com]

Appropriate therapy will prevent unnecessary deaths in infants, children, and young adults. [omicsonline.org]