Pre-anesthesia evaluation leading to a diagnosis of long QT syndrome – a case study (original) (raw)
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Pre-anesthesia evaluation leading to a diagnosis of long QT syndrome-a case study ABSTRACT
Long QT Syndrome is a rare disorder characterized by prolongation of QT interval on an ECG. Patients with this disorder have an increased risk of developing life-threatening arrhythmias such as torsades de pointes, and occasionally sudden cardiac arrest. We present this case of undiagnosed Long QT Syndrome in a 7 years old boy, who presented to our service for incision and drainage of an abscess in the left axilla under general anesthesia. This case highlights the importance of proper and meticulous pre-anesthesia evaluation, even in low risk patients, so that potentially harmful medical conditions are diagnosed well in time and managed accordingly.
Long QT Syndrome and anaesthesia
Long QT syndrome (LQTS) is an arrhythmogenic cardiovascular disorder resulting from mutations in cardiac ion channels. LQTS is characterized by prolonged ventricular repolarization and frequently manifests itself as QT interval prolongation on the electrocardiogram (ECG). The age at presentation varies from in utero to adulthood. The majority of symptomatic events are related to physical activity and emotional stress. Although LQTS is characterized by recurrent syncope, cardiac arrest, and seizure-like episodes, only about 60% of patients are symptomatic at the time of diagnosis. The clinical features of LQTS result from a peculiar episodic ventricular tachyarrhythmia called`torsade de pointes'.`Twisting of the points' describes the typical sinusoidal twisting of the QRS axis around the isoelectric line of the ECG. Usually torsade de pointes start with a premature ventricular depolarization, followed by a compensatory pause. The next sinus beat often has a markedly prolonged QT interval and abnormal T wave. This is followed by a ventricular tachycardia that is characterized by variation in the QRS morphology, and a constantly changing R-R interval . The`short-long-short' cycle length sequence heralding torsade de pointes is a hallmark of LQTS. Commonly, the episode of torsade de pointes is self-terminating, producing a syncopal episode or pseudoseizure, secondary to the abrupt decrease in cerebral blood ow. The majority of episodes of sudden death in LQTS result from ventricular ®brillation triggered by torsade de pointes, although the mechanism of this deterioration is unknown.
Anesthesia for Patients with Congenital Long QT Syndrome
Long QT syndrome is a malfunction of cardiac ion channels resulting in impaired ventricular repolarization that can lead to a characteristic polymorphic ventricular tachycardia known as torsades de pointes. Stressors, by increasing sympathetic tone, and drugs can provoke torsade de pointes, leading to syncope, seizures, or sudden cardiac death in these patients. Beta blockade , implantation of cardioverter defibrillators, and left cardiac sympathetic denervation are used in the treatment of these patients. However, these treatment modalities do not guarantee the prevention of sudden cardiac death. Certain drugs, including anesthetic agents, are known to contribute to QT prolonga-tion. After reviewing the literature the authors give recommendations for the anesthetic management of these patients in the perioperative period.
Safe Anaesthesia Management in a Child with Congenital Long QT Syndrome
Turkish Journal of Anesthesia and Reanimation, 2016
Long QT syndrome is a cardiac repolarisation disorder that can occur with clinical symptoms such as dizziness, fainting, life-threatening arrhythmias and sudden cardiac death, and its incidence is increasing in the general population. A careful anaesthetic management is required for patients with this syndrome because of the risk of torsades de pointes and malignant arrhythmias. In this case report, we discuss the anaesthetic management of a seven-year-old patient with congenital long QT syndrome that was diagnosed during the preoperative evaluation.
Anesthesia for children with long QT syndrome: Challenges and solutions from pediatric studies
Trends in Anaesthesia and Critical Care, 2017
Long QT syndrome Long QT syndrome and anesthesia Anesthesia and pediatric arrhythmias Anesthesia and genetic disorders in children a b s t r a c t Background: The overall prevalence of Long QT syndrome (LQTS) is as high as 1 in every 2534 live births which exceeds few common genetic-bound anesthesia related disorders. Given less attention than even the rarer malignant hyperthermia, LQTS incidence is expected to be much higher during anesthesia and surgery for very reasons that they contribute to acquired LQTS. Thus, confined to original researches on pediatric patients, the aim of this review was to assess challenges and solution of LQTS during pediatric anesthesia. Methods: and finding: Seventeen studies conducted on pediatric population from Medline and Google scholar search results were finally analyzed. The review protocol was registered in PROSPERO. Assuming that whatever observed in adult researches may not apply for children; we included individual studies carried out just on children. Shortage of purely pediatric researches about LQTS was observed. Widely used in the literature, prolonged QT interval was employed as predictor of LQTS in this review. Narrative approach was used to analyze results. Preoperative anxiety and seizure are risk factors for LQTS. Whereas propofol and halothane shorten QT interval and can be used to treat LQTS; most of inhalational agents and few adjuvant drugs prolong QT interval and predispose children to the syndrome. Beta blockers and magnesium sulphate were effectively used to treat long QT syndrome perioperatively. Conclusion: LQTS worth emphasis as the prevalence and associated complications exceed some commonly discussed genetic disorders in anesthesia. Preoperative evaluation of pediatric patients directed to identifying perioperative risk factors and preparation for respective management minimizes potentially fatal complications associated with long QT syndrome.
Perioperative management of long QT syndrome in a child with congenital heart disease
Acta Anaesthesiologica Scandinavica, 2002
During cardiac catheterization, a 2 1 ⁄ 2-year-old boy developed sudden cardiac arrest. The presence of a long QT interval in the electrocardiogram (ECG) along with ventricular arrhythmia and syncope at that moment enabled us to diagnose long QT syndrome (LQTS). Immediate defibrillation and beta-blocker (metoprolol) therapy saved the life of the child. Cardiac catheterization was completed and the child was planned for Fontan operation. Beta-blocker coverage, prevention of sympathetic stimulation and avoidance of agents which prolong the QT interval made anesthesia uneventful. There were episodes of ventricular fibrillation (VF) in the postoperative period. The child
Acquired Long QT Syndrome: A Review of the Literature
Asploro Journal of Biomedical and Clinical Case Reports (ISSN: 2582-0370), 2020
The QT interval represents the duration of ventricular depolarization and repolarization. It is measured from the beginning of the QRS complex to the end of the T wave. Prolongation of the QT interval may be congenital or acquired. This increases the risk of polymorphic ventricular tachycardia (i.e torsades de pointes) and cardiac arrest. To increase the awareness of this life-threatening phenomenon I outline an illustrative case in which acquired prolongation of the QT interval due to electrolyte derangement and administration of ciprofloxacin resulted in cardiac arrest due to torsade de pointes. Management of a patient with a long QT syndrome includes Immediate cessation of drugs that prolong the QT interval; cardiac monitoring, serial 12 lead ECGs and transthoracic echocardiography; measurement of serum electrolytes; intravenous potassium replacement; intravenous magnesium replacement; beta-blockade. Causes of acquired prolongation of the QT interval are common in critically ill patients. It is important to recognize this and consider screening with 12 lead ECG to reduce the risk of life-threatening ventricular arrhythmias.
Prolonged QT Syndrome: Perioperative Management
Mayo Clinic Proceedings, 1993
Intraoperative cardiac arrhythmias related to prolonged QT syndrome are uncommon. We describe a 26-year-old woman in whom ventricular fibrillation occurred during the final minutes of a partial glossectomy and right supraomohyoid selective neck dissection and discuss the role that this specific operation may have had in the development of the intraoperative event. In addition, we review the perioperative management of patients with prolonged QT syndrome. Congenital prolonged QT syndrome is an uncommon but potentially fatal entity. Jervell and Lange-Nielsen' first described this entity when they published the report of a family in which three children died at an early age. Common characteristics include the triad of prolonged QT interval, syncope, and congenital deafness. Subsequently, two other articles described a similar condition without the characteristic of deafness." Syncope is the result of malignant ventricular arrhythmias, which usually resemble torsades de pointes." Other published case reports have described the anesthetic management of patients known preoperatively to have prolonged QT syndromes" and those who did not have cardiac problems but in whom cardiac complications developed intraoperatively.PP Herein we describe a patient who had had an unexplained episode of cardiopulmonary arrest and who was successfully resuscitated after an episode of intraoperative ventricular fibrillation. We also review the recent literature for the medical and anesthetic management of patients with this potentially fatal syndrome. REPORT OF CASE A 26-year-old woman with squamous cell carcinoma of the tongue was referred to our institution for assessment and management. Her surgical history included an appendectomy and extraction of wisdom teeth, both of which were
Journal of anesthesia, 2017
More than 70% of intensive care unit (ICU) patients experience heart rhythm disturbances, and these patients have correspondingly higher mortality rates. Consequently, one of the standards of care in ICUs is continuous electrocardiography monitoring. One of the potentially preventable dysrhythmic events is the occurrence of torsade de pointes ventricular tachycardia in the setting of acquired prolonged QT interval. This type of ventricular tachycardia can be malignant because it often progresses to ventricular fibrillation. Many factors predispose to lengthening of the QT interval, the most important of which are electrolyte abnormalities and the administration of specific medications. In this review, we discuss the pathophysiology of acquired long QT interval, stressing the medication-induced acquired long QT-interval and factors present in ICU patients that promote prolongation of the QT interval. We also propose guidelines to avoid the occurrence of torsade de pointes ventricular...