Community detection of long QT syndrome with a clinical registry: An alternative to ECG screening programs?

Earle, Nikki; Crawford, Jackie; Smith, Warren; Hayes, Ian; Shelling, Andrew N.; Hood, Margaret; Stiles, Martin K.; Maxwell, Fraser; Heaven, David; Love, Donald R.; Skinner, Jonathan R.

doi:10.1016/j.hrthm.2012.10.043

Cited by 58 publications

(60 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…49 The prevalence of LQTS is reported as at least 4/10,000 (nearly 5/10,000) apparently healthy live births in Italy, 50 and 2.2/10,000 in New Zealand. 51 The prevalence of QT prolongation, defined as QTc ≥460 ms, was reportedly 0.02% in apparently healthy young Korean men, 52 and among 7,961 Japanese school children (4,044 males), the prevalence of a high probability of LQTS, defined as LQTS score >3.5, was 0.038%. 53 Taking these reports together, the prevalence of LQTS in Asia ranges from 0.02% to 0.04%, which is consistent with data from Europe and the USA.…”

Section: Bradycardiasmentioning

confidence: 99%

Epidemiology of Arrhythmias and Sudden Cardiac Death in Asia

Murakoshi

Aonuma

2013

Circ J

122

View full text Add to dashboard Cite

Section: Bradycardiasmentioning

confidence: 99%

Epidemiology of Arrhythmias and Sudden Cardiac Death in Asia

Murakoshi

Aonuma

2013

Circ J

122

View full text Add to dashboard Cite

“…All were enrolled in the New Zealand Cardiac Inherited Diseases Registry and consented to their data being used for research. 11 Procedures were performed between 2008 and 2014 by 1 of 2 surgeons; median age at the time of LCSD was 17 years (range, 2-64 years); 34 patients were women (72%), and 13 were men. Patients who had the same procedure for other indications, or who had open surgery, were excluded.…”

Section: Study Populationmentioning

confidence: 99%

Physical and Psychological Consequences of Left Cardiac Sympathetic Denervation in Long-QT Syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia

Waddell-Smith

Ertresvaag

et al. 2015

Circ: Arrhythmia and Electrophysiology

Self Cite

View full text Add to dashboard Cite

Background— Left cardiac sympathetic denervation reduces risk in long-QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia. Side effects and patient satisfaction have not been systematically analyzed in patients who underwent left cardiac sympathetic denervation. Aims of this study included documenting physical and psychological consequences and patient satisfaction after left cardiac sympathetic denervation in LQTS or catecholaminergic polymorphic ventricular tachycardia. Methods and Results— Patients with LQTS (N=40) and catecholaminergic polymorphic ventricular tachycardia (N=7) underwent video-assisted thoracoscopic left cardiac sympathetic denervation, with a median follow-up of 29 months (range, 1–67 months). Clinical records were reviewed; 44 patients completed a telephone survey. Of 47 patients (53%), 25 were preoperatively symptomatic (15 syncope, 7 near-drowning, and 3 resuscitated sudden death). Indications for left cardiac sympathetic denervation included β-blocker intolerance (15; 32%) or nonadherence (10; 21%) and disease factors (18; 38%; catecholaminergic polymorphic ventricular tachycardia [6], near-drowning [2], exertional syncope [1], symptoms on therapy [2], LQT3 [1], QTc>520 ms [6]). Other indications were competitive sports participation (2), family history of sudden death (1), and other (1). Median QTc did not change among patients with LQTS (461±60 to 476±54 ms; P =0.49). Side effects were reported by 42 of 44 (95%). Twenty-nine patients (66%) reported dryness on left side, 26 (59%) a Harlequin-type (unilateral) facial flush, 24 (55%) contralateral hyperhidrosis, 17 (39%) differential hand temperatures, 5 (11%) permanent and 4 (9%) transient ptosis, 5 (11%) thermoregulation difficulties, 4 (9%) a sensation of left arm paresthesia, and 3 (7%) sympathetic flight/fright response loss. Majority of the patients were satisfied postoperatively: 38 (86%) were happy with the procedure, 33 (75%) felt safer, 40 (91%) recommended the procedure to others, and 40 (91%) felt happy with their scar appearance. Conclusions— Despite significant morbidity resulting from left cardiac sympathetic denervation, patients with LQTS and CPVT have high levels of postoperative satisfaction.

show abstract

“…50%) of disease in the tested population, enhancing the positive predictive value. This approach, termed cascade screening, is useful in families with known disease causing mutations and phenotypes, 134,135 such as LQTS, as well as in cases of sudden unexplained death, [135][136][137][138][139] employing electrocardiographic, genetic or other techniques as appropriate. In one study employing a national registry, an average of 2.1 new cases were identified per each LQTS proband, 134 and observational studies have noted a high diagnostic yield of affected family members of victims of SCD and unexplained cardiac arrest, 137 although this has not been a uniform finding.…”

Section: Identification Of Young Patients At Risk For Scd Prodromal Smentioning

confidence: 99%

Sudden cardiac death in the young

2017

Progress in Pediatric Cardiology

View full text Add to dashboard Cite

Although the occurrence of sudden cardiac death (SCD) in a young person is a rare event, it is traumatic and often widely publicized. In recent years, SCD in this population has been increasingly seen as a public health and safety issue. This review presents current knowledge relevant to the epidemiology of SCD and to strategies for prevention, resuscitation and identification of those at greatest risk. Areas of active research and controversy include the development of best practices in screening, risk stratification approaches and post-mortem evaluation, and identification of modifiable barriers to providing better outcomes after resuscitation of young SCD victims. Institution of a national registry of SCD in the young will provide data that will help to answer these questions.

show abstract

Community detection of long QT syndrome with a clinical registry: An alternative to ECG screening programs?

Cited by 58 publications

References 26 publications

Epidemiology of Arrhythmias and Sudden Cardiac Death in Asia

Epidemiology of Arrhythmias and Sudden Cardiac Death in Asia

Physical and Psychological Consequences of Left Cardiac Sympathetic Denervation in Long-QT Syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia

Sudden cardiac death in the young

Contact Info

Product

Resources

About