Disease: Long Qt Syndrome 2 ×
Source: ALL
Disease Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE LQT1 and LQT2 patients had a higher prevalence of late NS-MTWA during exercise than matched controls. 29183619 2019
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 GeneticVariation disease BEFREE Beta-blockers significantly reduced the risk of cardiac events in LQT1 (HR: 0.49, p = .002) and LQT2 patients (HR: 0.48, p = .001). 29504689 2018
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE The prognostic significance for predicting cardiac events in LQT1 and LQT2 patients was similar for JTpc and QTc. 28942950 2018
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 GeneticVariation disease BEFREE Loss-of-function long QT (LQT) mutations inducing LQT1 and LQT2 syndromes have been successfully translated to human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) used as disease-specific models. 30428582 2018
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE The study included 59 LQT1 and 72 LQT2 patients from the Rochester-based LQTS Registry with corrected QT (QT<sub>c</sub>) prolongation and a history of AD therapy. 29174490 2018
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE A follow-up study covering a mean of 18.6 ± 6.1 years was conducted in 867 genetically confirmed LQT1 and LQT2 patients and 654 non-carrier relatives aged 18-40 years. 29622001 2018
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE No significant differences were noted between LQT1 and LQT2 patients. 28233664 2017
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 GeneticVariation disease BEFREE The calculated QT intervals at an RR interval of 1200 ms were longer in the KCNE1(G38S) carriers and LQT1 and LQT2 patients than in the control subjects. 27255646 2017
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE Follow-up data covering a mean of 12 years were collected for 316 genotyped LQT1 and LQT2 patients aged 0 to 18 years. 26063740 2015
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 GeneticVariation disease BEFREE 24-hour digital Holter ECG were recorded before and after beta-blocking therapy initiation in LQT1 (n = 30) and LQT2 patients (n = 16). 23879280 2013
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE Asymptomatic patients, almost absent among LQT1 and LQT2 patients, represented 45% of LQT3 patients. 20837891 2010
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE Our results suggest that a reduction of both IKr and IKs underlies the combined LQT1 and LQT2 phenotype observed in this patient. 21164565 2010
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE The present findings suggest that beta-blocker therapy should be routinely administered to all high-risk LQT1 and LQT2 patients without contraindications as a first line measure, whereas primary defibrillator therapy should be recommended for those who experience syncope during medical therapy. 20233272 2010
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE The degree of QTc prolongation during exercise was greater in LQTS patients (LQT1 80 ± 47 ms, LQT2 64 ± 41 ms, Control 46 ± 20 ms, P = 0.02), with significant differences between LQT1 and LQT2 patients observed at heart rates ≥ 60% of the predicted maximum (P < 0.05). 20455992 2010
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 GeneticVariation disease BEFREE Conversely, the LQT2 patients began recovery at its lowest mean QTc of 420 +/- 10 ms, which increased by 40 +/- 16 ms. At the end of recovery, a QTc cut-off value of 445 ms distinguished 92% of LQTS patients from unaffected controls, while a start-of-recovery QTc cut-off of 460 ms correctly identified genotype in 80% of LQT1 and 92% of LQT2 patients. 20226272 2010
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE Beta-adrenergic stimulation may unmask occult LQT1, but no maneuver has consistently unmasked the LQT2 phenotype. 18273958 2008
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE More recently, mutation site-specific differences in the clinical phenotype have been reported in LQT1 and LQT2 patients, indicating the possibility of mutation site-specific management or treatment. 18981593 2008
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE Although the QTc interval also was longer in symptomatic patients, only the maximal amplitude ratio between late and early T-wave peaks was independently associated with symptoms in both LQT1 and LQT2 patients. 16386673 2006
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE Overall, phenylephrine exerted no significant effect on either QTd or Tp-e except with subgroup analysis of symptomatic LQTS where LQT1 and LQT2 patients had a divergent response with TDR. 14510655 2003
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE CONCLUSIONS; Age and gender have different, genotype-specific modulating effects on the probability of cardiac events and electrocardiographic presentation in LQT1 and LQT2 patients. 12849668 2003
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE However, there were no significant differences in these indices between the LQT1 and LQT2 patients. 12808265 2003
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE Combining measures for QT duration, rate dependence, and QT end - QT apex interval, derived from Holter recordings, complements the clinical differentiation between LQT1 versus LQT2 patients and between affected and unaffected persons for genotype screening purposes. 11897209 2002
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 GeneticVariation disease BEFREE The QTc was much less prolonged at peak of epinephrine in LQT3 (478+/-44 to 532+/-41 ms; P<0.05, +11%) and controls (394+/-21 to 456+/-18 ms; P<0.0005, +16%) than in LQT1 and LQT2 patients, and shortened to the baseline levels (LQT3; 466+/-49 ms, -3%, controls; 397+/-16 ms, +1%; P=ns vs baseline) at steady state. 12069453 2002
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 Biomarker disease BEFREE Until definitive data become available, antiadrenergic therapy remains the mainstay in the management of LQTS patients, however it may be soon worth considering the addition of a Na+ channel blocker such as mexiletine for LQT3 patients and of interventions such as K+ channel openers or increases in the extracellular concentration of potassium for LQT1 and LQT2 patients. 9272507 1997
CUI: C3150943
Disease: Long Qt Syndrome 2
Long Qt Syndrome 2 		0.200 CausalMutation disease CLINVAR