A case of long QT syndrome: challenges on a bumpy road

Magnusson, Peter; Gustafsson, Per-Erik

doi:10.1002/ccr3.985

Cited by 3 publications

(2 citation statements)

References 39 publications

(47 reference statements)

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“…Long QT syndrome (LQTS) is a rare condition that affects electrophysiological cardiac activity leading to life-threatening conditions, and it can be congenital or acquired [ 23 ]. The diagnosis is made when the QT interval exceeds 500 msec after correcting heart rate [ 24 ]. While acquired LQTS is usually caused by cardiac conduction abnormalities, electrolyte disturbances, or QT-prolonging drugs, congenital LQTS is a diagnosis of exclusion of those causes [ 25 , 26 ].…”

Section: Discussionmentioning

confidence: 99%

Salbutamol-Induced QT Interval Prolongation in a Two-Year-Old Patient

Elgassim¹,

Abdelrahman²,

Saied³

et al. 2022

Cureus

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Salbutamol-induced QT interval prolongation is a relatively rare adverse effect of beta2-agonists. We report a case of a two-year-old female patient with no known past medical history, brought by her parents to the ED 30 minutes after ingesting a total dose of 97 mg of salbutamol solution. ECG was done for the patient when she arrived and showed sinus tachycardia with prolonged QTc (509 ms) and normal QRS complex. The patient was admitted to the Pediatric Intensive Care Unit (PICU) with persistent tachycardia and tachypnea in the initial reassessment. ECG was repeated with normal QT interval after IV Mg sulfate. The patient was observed in PICU for 12 hours with serial ECG and venous blood gas (VBG). IV potassium chloride (KCL) infusion started, and serial VBG showed normal potassium and lactate. The patient was doing well in the next six hours, with normal serial ECG, labs, and vital signs. In conclusion, salbutamol-induced QT prolongation has infrequently been reported in the literature. Although inhaled salbutamol is commonly used in clinical practice, physicians have limited experience with the severe features of its toxicity. Salbutamol is known to cause minimal side effects, which may be under-recognized and progress to serious manifestations such as hypokalemia, QT prolongation, and sudden cardiac death.

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Section: Discussionmentioning

confidence: 99%

Salbutamol-Induced QT Interval Prolongation in a Two-Year-Old Patient

Elgassim¹,

Abdelrahman²,

Saied³

et al. 2022

Cureus

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“…The prolongation of QTc interval is best seen in lead II, V5 or V6 but all 12 leads should be examined and avoid for appearance U wave in QTc interval measurement [5,10]. In general, LQTS can be diagnosed by any of the following (1) when the duration of QTc interval ≥ 500 ms in asymptomatic patient on repeated 12-lead ECG examination, but in patients with syncope LQTS can be diagnosed when the duration of QTc interval ≥ 480 ms on repeated 12-lead ECG examination (based on Heart Rhythm Society guidelines); (2) risk score ≥3.5 (based on Schwartz scoring system); or (3) when there is evidence of pathogenic variant genes of LQTS [10,11,12]. In this case, we diagnose this patient with acquired LQTS because of the duration of QTc interval in this patient 638 ms based on Heart Rhythm Society guidelines.…”

Section: Discussionmentioning

confidence: 99%

Acquired Long QT Syndrome (LQTS) Secondary to Electrolyte Imbalance: A Case Report

Pintaningrum¹,

Pramana²

2021

Advances in Health Sciences Research

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The long QT syndrome (LQTS) is a one type of severe cardiac arrhythmia syndrome that leading cause to unexpected cardiac death, characterized with impaired ventricular repolarization caused by hereditary disorder in sodium and potassium channels on cardiac myocyte and acquired caused by drugs or electrolyte imbalance, especially hypokalemia, hypomagnesemia, and hypocalcemia. This condition result in abnormality on the electrocardiogram (ECG) examination as QT interval prolongation and may increase risk of syncope, ventricular arrhythmias, seizure, and cardiac death to the patient. We present a case of a 46-year-old woman came to emergency department at hospital with a chief complaint of dizziness. Three days before these complaints, the patient also complaints of epigastric pain and profuse vomitting more than five times. The patient has no history of taking certain any medications before. On physical examination, there is no abnormality in patient's vital signs and only palpable pain on epigastric region. On clinical laboratory examinations showed electrolyte imbalance such as hyponatremia and hypokalemia. From electrocardiography showed prolongation of QTc interval (638 ms) and venticular extrasystole trigeminy. On echocardiography, the patient had diastolic dysfunction grade I and concentric left ventricular hypertrophy with normal ejection fraction. From that examination, the patient diagnoses with acquired long QT syndrome and the focus of therapy in this patient is to restore the electrolyte balance. The diagnostic for LQTS in this patient is based on QTc interval ≥ 500 ms in electrocardiography examination. This diagnose criteria was based on Heart Rhythm Society guidelines. Electrolyte imbalance, especially hypokalemia, hypomagnesemia, and hypocalcemia is one of the most common indirect mechanisms of QT interval prolongation. The point of management LQTS in this patient includes recognition and discontinuation of any encouraging medication and the forcefull correction of any electrolyte imbalance, such as hypokalemia, hypomagnesemia, and hypocalcemia. Early detection in QTc interval may be helpful to diagnose and treatment LQTS to prevent further complications such as syncope, ventricular arrhythmias, and sudden cardiac death.

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Challenges and opportunities for integrating genetic testing into a diagnostic workflow: heritable long QT syndrome as a model

et al. 2019

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BackgroundAn increasing number of diagnostic evaluations incorporate genetic testing to facilitate accurate and timely diagnoses. The increasing number and complexity of genetic tests continue to pose challenges in deciding when to test, selecting the correct test(s), and using results to inform medical diagnoses, especially for medical professionals lacking genetic expertise. Careful consideration of a diagnostic workflow can be helpful in understanding the appropriate uses of genetic testing within a broader diagnostic workup.ContentThe diagnosis of long QT syndrome (LQTS), a life-threatening cardiac arrhythmia, provides an example for this approach. Electrocardiography is the preferred means for diagnosing LQTS but can be uninformative for some patients due to the variable presentation of the condition. Family history and genetic testing can augment physiological testing to inform a diagnosis and subsequent therapy. Clinical and laboratory professionals informed by peer- reviewed literature and professional recommendations constructed a generalized LQTS diagnostic workflow. This workflow served to explore decisions regarding the use of genetic testing for diagnosing LQTS.Summary and outlookUnderstanding the complexities and approaches to integrating genetic testing into a broader diagnostic evaluation is anticipated to support appropriate test utilization, optimize diagnostic evaluation, and facilitate a multidisciplinary approach essential for achieving accurate and timely diagnoses.

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A case of long QT syndrome: challenges on a bumpy road

Cited by 3 publications

References 39 publications

Salbutamol-Induced QT Interval Prolongation in a Two-Year-Old Patient

Salbutamol-Induced QT Interval Prolongation in a Two-Year-Old Patient

Acquired Long QT Syndrome (LQTS) Secondary to Electrolyte Imbalance: A Case Report

Challenges and opportunities for integrating genetic testing into a diagnostic workflow: heritable long QT syndrome as a model

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