scholarly journals QT-prolongation and noncompaction

2019 ◽  
pp. 01-03
Author(s):  
Josef Finsterer ◽  
Claudia Stöllberger
Keyword(s):  
Ventricular Fibrillation ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Genetic Defect ◽  
Neuromuscular Disorder ◽  
Left Ventricular ◽  
Qt Prolongation ◽  
Long Qt ◽  
Qt Syndrome

In a recent article, Szulik et al. reported about a 22 years old female with ventricular fibrillation, QT-prolongation, and left ventricular hypertrabeculation/noncompaction (LVHT) who died from hypoxic cerebral damage 5 days after admission [1]. We have the following comments and concerns. Patients with LVHT have a disposition for any type of cardiac arrhythmia [2,3]. This is why ventricular fibrillation not only could be due to hereditary long-QT syndrome but also due to LVHT. Ventricular fibrillation was either due to LVHT or a consequence of QT-prolongation. QT-prolongation is not unusual in LVHT and has been reported in several cases (table 1) [3-7]. LVHT has been also reported in association with long-QT-syndrome due to mutations in the KCNQ1 gene [8], in the KCNH2 gene [9], or due to an unidentified genetic defect (table 1) [10]. In a study of 105 patients with LVHT, the QT-interval increased during a mean follow up of 3.6y in 15 patients and normalized in 21 patients [11]. The increase was associated with the extension of LVHT and the presence of a neuromuscular disorder (NMD) [11].

scholarly journals Oral adrenergic agents produced Ventricular fibrillation and QT prolongation in an elderly patient carrying an RYR2 variant.

2021 ◽  
Author(s):  
Kanae Hasegawa ◽  
Kentaro Ishida ◽  
Shinsuke Miyazaki ◽  
Seiko Ohno ◽  
Minoru Horie ◽  
...  
Keyword(s):  
Ventricular Fibrillation ◽  
Long Qt Syndrome ◽  
Qt Prolongation ◽  
Polymorphic Ventricular Tachycardia ◽  
Long Qt ◽  
Causative Gene ◽  
Drug Induced ◽  
Adrenergic Agents ◽  
First Case ◽  
Qt Syndrome

Mutant cardiac ryanodine receptor channels (RyR2) are “leaky,” and spontaneous Ca2+ release through these channels causes delayed afterdepolarizations that can deteriorate into ventricular fibrillation (VF). RYR2 is a causative gene of type 1 catecholaminergic polymorphic ventricular tachycardia (CPVT). Some patients carrying RYR2 mutations in CPVT exhibit QT prolongation and are initially diagnosed with long QT syndrome. However, none have been reported to cause drug-induced VF in patients with RYR2 variants. We describe the first case of an elderly woman with drug-induced QT prolongation and VF who carried a novel RYR2variant but no other mutations related to long QT syndrome.

scholarly journals Outcome by Sex in Patients With Long QT Syndrome With an Implantable Cardioverter Defibrillator

2020 ◽  
Vol 9 (19) ◽  
Author(s):  
Arwa Younis ◽  
Mehmet K. Aktas ◽  
Spencer Rosero ◽  
Valentina Kutyifa ◽  
Bronislava Polonsky ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Fibrillation ◽  
Implantable Cardioverter Defibrillator ◽  
Long Qt Syndrome ◽  
Syndrome Type ◽  
Long Qt ◽  
Cardioverter Defibrillator ◽  
Life Threatening ◽  
Qt Syndrome

Background Sex differences in outcome have been reported in patients with congenital long QT syndrome. We aimed to report on the incidence of time‐dependent life‐threatening events in male and female patients with long QT syndrome with an implantable cardioverter defibrillator (ICD). Methods and Results A total of 60 patients with long QT syndrome received an ICD for primary or secondary prevention indications. Life‐threatening events were evaluated from the date of ICD implant and included ICD shocks for ventricular tachycardia, ventricular fibrillation, or death. ICDs were implanted in 219 women (mean age 38±13 years), 46 girls (12±5 years), 55 men (43±17 years), and 40 boys (11±4 years). Mean follow‐up post‐ICD implantation was 14±6 years for females and 12±6 years for males. At 15 years of follow‐up, the cumulative probability of life‐threatening events was 27% in females and 34% in males (log‐rank P =0.26 for the overall difference). In the multivariable Cox model, sex was not associated with significant differences in risk first appropriate ICD shock (hazard ratio, 0.83 female versus male; 95% CI, 0.52–1.34; P =0.47). Results were similar when stratified by age and by genotype: long QT syndrome type 1 (LQT1), long QT syndrome type 2 (LQT2), and long QT syndrome type 3 (LQT3). Incidence of inappropriate ICD shocks was higher in males versus females (4.2 versus 2.7 episodes per 100 patient‐years; P =0.018), predominantly attributed to atrial fibrillation. The first shock did not terminate ventricular tachycardia/ventricular fibrillation in 48% of females and 62% of males ( P =0.25). Conclusions In patients with long QT syndrome with an ICD, the risk and rate of life‐threatening events did not significantly differ between males and females regardless of ICD indications or genotype. In a substantial proportion of patients with long QT syndrome, first shock did not terminate ventricular tachycardia/ventricular fibrillation.

Video-Assisted Modified Left Sympathectomy for Long-QT Syndrome

2000 ◽  
Vol 8 (1) ◽  
pp. 52-53 ◽  
Author(s):  
Guo Xing Weng ◽  
Hang Ding ◽  
Juan Qi ◽  
Chun Xuan Xu
Keyword(s):  
Ventricular Tachycardia ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Torsade De Pointes ◽  
Horner’S Syndrome ◽  
Horner's Syndrome ◽  
Long Qt ◽  
Video Assisted ◽  
Qt Syndrome

A 22-year-old female suffering from idiopathic long-QT syndrome complicated by frequent syncope, torsade-de-pointes-type ventricular tachycardia, and asthma, was successfully treated by video-assisted extensive left second and third thoracic sympathetic ganglionectomy, instead of left stellate and first thoracic ganglio-nectomy, to avoid postoperative Horner's syndrome. The QT interval was significantly shortened from 0.6 to 0.43 seconds four days after the surgery. It remained at 0.43 seconds during a 3-month follow-up with no recurrence of tachycardia or syncope.

Atrioventricular block in a new born with acquired long QT syndrome

2001 ◽  
Vol 11 (6) ◽  
pp. 680-682 ◽  
Author(s):  
John R. Phillips ◽  
Christopher L. Case ◽  
Paul C. Gillette
Keyword(s):  
Intensive Care Unit ◽  
Atrioventricular Block ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Neonatal Intensive Care ◽  
Qt Prolongation ◽  
Long Qt ◽  
Acquired Long Qt Syndrome ◽  
Qt Syndrome ◽  
Congenital Long Qt Syndrome

We report a case of 2:1 atrioventricular block associated with acquired long QT syndrome. A newborn presented to our neonatal intensive care unit with intermittent bradycardia due to 2:1 atrioventricular block. Initial evaluation showed QT prolongation and significant electrolytic abnormalities. After correction of the electrolytic imbalance, the QT interval normalized and atrioventricular block resolved. Compared to congenital long QT syndrome with 2:1 atrioventricular block, acquired long QT syndrome with comparable atrioventricular block has a benign prognosis, provided treatment is initiated quickly.

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