Phenotypes of Overdiagnosed Long QT Syndrome

Bains, Sahej; Neves, Raquel; Bos, J. Martijn; Giudicessi, John R.; MacIntyre, Ciorsti; Ackerman, Michael J.

doi:10.1016/j.jacc.2022.11.036

Cited by 13 publications

(5 citation statements)

References 20 publications

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“…LQTS is likely to be overdiagnosed due to clinical symptom such as vasovagal syncope, and transient QT interval prolongation during stress condition. 22) In our data, LQTS revealed higher incidence of syncope as well as lower incidence of aborted SCD, which implies possibility of overdiagnosis in LQTS.…”

Section: Discussionsupporting

confidence: 48%

Clinical and Genetic Features of Korean Inherited Arrhythmia Probands

Jeong

Kim

et al. 2023

Korean Circ J

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Author's summary Inherited arrhythmia is a more common cause of sudden cardiac arrest in Asian population, yet less is known of genetic background compared to western population. Certain clinical differences were found in the three most common disease: idiopathic ventricular fibrillation (IVF), Brugada syndrome (BrS), and long QT syndrome (LQTS). Patients detected with certain genotype resulted lower left ventricular ejection fraction compared to those without detected genotype (54.7±11.3 vs. 59.3±9.2%, p=0.005). Genetic yield was relatively low in BrS (19.5%) and LQTS (23.5%), whereas IVF revealed higher genetic yield (54.0%), suggesting its susceptibility to concealed cardiomyopathy.

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Section: Discussionsupporting

confidence: 48%

Clinical and Genetic Features of Korean Inherited Arrhythmia Probands

Jeong

Kim

et al. 2023

Korean Circ J

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“…Since overdiagnosis of long QT syndrome has high detrimental emotional and nancial effects on the patients, after screening with Schwartz score in the follow-up session, appropriate referral should be made to an expert electrophysiologist for assessment of this disease since this condition can have lethal outcomes [13].…”

Section: Discussionmentioning

confidence: 99%

Long QT syndrome diagnosis may be affected by positive tilt table test; a cross-sectional study

Nikoo

Hassani

Kamrava

et al. 2023

Preprint

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Background Tilt Table Test (TTT) is usually used for assessment of syncope with undefined etiology. Positive test occurs in those with parasympathetic overstimulation. This overactivation of parasympathetic system may affect QTc measurement; not only through rate modulation, but also from through potassium ionic channels as muscarinic activated inward rectifying K + current.Methods and Materials: in this cross-sectional study, 478 patients with syncopal attacks of unknown origin underwent evaluation with TTT as well as 24-hour Holter electrocardiogram monitoring. Schwartz score (for diagnosis of Long QT syndrome) was calculated for all of them. Those with secondary causes of long QT excluded from survey. Then the association of positive TTT and Schwartz score was assessed.Results 478 patients entered the study 208 (43.4%) of whom were male and 270 (56.4%) were female. The mean age of the patients was 42.43 ± 17.979 years old. TTT was positive in 289 (60.46%) patients and was negative in 189 (39.54%) patients. the mean Schwartz score was significantly higher in patients with positive TTT (2.77 ± 0.08 vs. 1.99 ± 0.12) (P < 0.001).Conclusion Diagnosis of long QT syndrome as a cause of syncope should not be made hesitantly by the use of Schwartz score as this scoring system might need to be modified based on the results of tilt table test.

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“…A recent review of the Mayo Clinic experience comprising 1841 patients referred for evaluation of LQTS found misdiagnosis in 16%, highlighting the need for exclusion of confounding conditions before making this diagnosis. 8 Awareness of exercise-induced LQT among military healthcare professionals is thus essential, as there are a significant amount of service members that could be affected by an inappropriate diagnosis of LQTS.…”

Section: Discussionmentioning

confidence: 99%