Presentation
There are several reasons behind this, the main one being not considering that long QT may be present. [radcliffecardiology.com]
The time of presentation varied from 16 to 38 weeks of gestation. [karger.com]
Presently, such identification relies on the 12-lead electrocardiogram (ECG). The purpose of this study was to evaluate the efficacy of the automated ECG as a screening tool for LQTS. Method. [pediatrics.aappublications.org]
It was decided that cardiologist and pacemaking devices would be present at the time of surgical procedure. [ispub.com]
Entire Body System
- Sudden Infant Death Syndrome
Abstract Prolongation of the QT interval is the cause of about 10% cases of sudden infant death syndrome (SIDS) in newborns but it is also responsible for about 5% of sudden deaths in adults. [omicsonline.org]
Molecular diagnosis in a child with sudden infant death syndrome. Lancet 2001 ; 358 : 1342 –3. ↵ Haddad GG, Epstein MA, Epstein RA, et al. The QT interval in aborted sudden infant death syndrome infants. [adc.bmj.com]
Relation with sudden infant deaths Sudden infant death syndrome (SIDS) remains the leading cause of sudden death during the first year of life in the western world. [ojrd.biomedcentral.com]
[…] including subarachnoid bleed Structural heart disease For syncope or sudden death in the young: Sudden infant death syndrome (SIDS) Drug-induced QT prolongation Vasovagal syncope (orthostatic hypotension) Anomalous coronary artery Familial ventricular [centogene.com]
The sudden infant death syndrome gene: Does it exist? Pediatrics 114(4):e506-512, 2004. 4. Schwartz PJ, Stramba-Badiale M, Segantini A, et al. Prolongation of the QT interval and sudden infant death syndrome. N Engl J Med 338(24):1709-1714, 1998. 5. [emsworld.com]
Gastrointestinal
- Diarrhea
[…] increase the risk of developing long QT syndrome: A history of cardiac arrest Having a parent, sibling or child with long QT syndrome Using medications known to cause prolonged QT intervals Being female and on heart medication Excessive vomiting or diarrhea [mayoclinic.org]
Other Causes of Acquired Long QT Syndrome Excessive diarrhea or vomiting that causes a major loss of potassium or sodium ions from the bloodstream may cause LQTS. The syndrome lasts until the levels of these ions in the blood return to normal. [ourmed.org]
Gastrointestinal illnesses such as diarrhea that may potentially lead to hypokalemia should be treated promptly and adequately. [thecardiologyadvisor.com]
- Failure to Thrive
Related Articles Step2 Twinnies of the Month Lilah and Olivia From “Failure to Thrive” to Thriving [twiniversity.com]
Cardiovascular
- Hypotension
QT-prolonging drugs Hypokalemia Certain neurologic conditions including subarachnoid bleed Structural heart disease For syncope or sudden death in the young: Sudden infant death syndrome (SIDS) Drug-induced QT prolongation Vasovagal syncope (orthostatic hypotension [centogene.com]
Following 24 h of intermittent 2:1 AV block and transient episodes of TdP accompanied by hypotension, a dual-chamber epicardial ICD was inserted, and a bilateral stellate ganglionectomy was performed. [pnas.org]
When dealing with borderline cases, the following conditions should be considered: vasovagal syncope, orthostatic hypotension, arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), catecholaminergic polymorphic ventricular tachycardia (CPVT [ojrd.biomedcentral.com]
Fetus
- Quickening
The breakthrough is expected to quicken the quest for a cure. Prof Wong said, “Using this platform we can potentially halve the time required for drug testing by doing away with multiple drug screenings. [healthxchange.sg]
Workup
[…] 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]
QT, RR, ST Intervals
- Prolonged QT Interval
In summary, inhaled anticholinergic agents do not result in prolongation of QT interval and inhaled beta agonists do variably prolong the interval. [aaaai.org]
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]
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]
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]
Overview People with LQTS have prolonged QT intervals on their ECGs. The QT interval represents repolarization, or "recharge," of a cardiac cell. [verywell.com]
Rhythm
- Sinus Arrhythmia
Sinus pauses Several LQTS patients have sudden pauses in sinus rhythm exceeding 1.2 seconds that are not related to sinus arrhythmia [ 11 ] and may contribute to the initiation of arrhythmias in LQTS patients. [ojrd.biomedcentral.com]
Other ECG Findings
- 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]
- Abnormal ECG
However, the diagnosis of LQTS can be challenging since around 2.5% of the healthy population have prolonged QT-interval while some of LQTS patients do not exhibit abnormal ECG findings. [asperbio.com]
Treatment
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]
Treatment A conventional treatment is the oral administration of beta-blockers, medications that decrease the input from the sympathetic nervous system to the heart. [medical-dictionary.thefreedictionary.com]
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]
Treatment goals for LQTS focus on preventing heart arrhythmia and sudden cardiac arrest. [medstarwashington.org]
These analyses - which are comprehensively reviewed and explored here for the first time - are now beginning to suggest treatment strategies specific to each genotype. [books.google.de]
Prognosis
[…] 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
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]
Epidemiology
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
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]
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]
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]
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
Long QT Syndrome, Risk Assessment, Secondary Prevention, Torsades de Pointes < Back to Listings [acc.org]
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]
To prevent this stimulus, pretreatment with fentanyl was done during induction of anaesthesia. [ispub.com]
Since LCSD is an invasive procedure, it should be used as a secondary prevention modality. [thecardiologyadvisor.com]