Journal: Nature Cardiovascular Research

Article Title: Unexpected impairment of I Na underpins reentrant arrhythmias in a knock-in swine model of Timothy syndrome

doi: 10.1038/s44161-023-00393-w

Figure Lengend Snippet: a , Representative ECG traces of WT (top, in blue) and TS1 (bottom, in red) pigs showing marked QTc interval prolongation in TS1. b , Box plot shows the distribution of QTc interval in n = 27 WT animals (minimum 419 ms, median 482 ms, IQR: 469–500 ms, maximum 514 ms) and n = 28 TS1 animals (minimum 521 ms, median 586 ms, IQR: 552–615 ms, maximum 657 ms). Statistical analysis was conducted using the two-tailed Mann–Whitney U -test ( P < 0.0001). c , Example of lethal ventricular arrhythmias in TS1 pigs, triggered by PVCs (red asterisks). d , Documentation of a sequence of three PVCs on the 24-h Holter ECG (above) and a PVC-triggered episode of VF recorded by implantable cardioverter defibrillator (below) in a young patient with TS1.

Article Snippet: We recorded the surface ECG at 1 kHz for at least 5 min (Mortara Instruments) (paper speed 25 mm s −1 and voltage settings 10 mm mV −1 ) in both TS1 and WT animals.

Techniques: Two Tailed Test, MANN-WHITNEY, Sequencing

Journal: Nature Cardiovascular Research

Article Title: Unexpected impairment of I Na underpins reentrant arrhythmias in a knock-in swine model of Timothy syndrome

doi: 10.1038/s44161-023-00393-w

Figure Lengend Snippet: a , Graphs show the quantification of LAT, LRT and LRT range of the S2, S3 and S4 as close as possible to ventricular refractoriness during spontaneous sinus rhythm between WT (blue, n = 8) and TS1 (red, n = 10) animals. Each value is represented as mean ± s.d. Statistical analysis was conducted using two-way ANOVA with Šidák post tests. b , Surface ECG (left), unipolar electrograms (central) and electroanatomical maps (right) documenting ventricular fibrillation induction in a TS1 pig after three extrastimuli on sinus rhythm, a situation that never occurred in WT pigs. The first activation during S4 corresponded to the pacing site in the RV (*). The latest activated area was found in the LV (**), whereas the first spontaneously reactivating region was found in the proximal site in the RV (+). Notably, this early reactivated area (reentrant activity) corresponded to the area with the shortest LRT registered during the closest S4 that did not induce ventricular fibrillation. The black arrow indicates the pacing site in the RV endocardium.

Article Snippet: We recorded the surface ECG at 1 kHz for at least 5 min (Mortara Instruments) (paper speed 25 mm s −1 and voltage settings 10 mm mV −1 ) in both TS1 and WT animals.

Techniques: Activation Assay, Activity Assay

Journal: Nature Cardiovascular Research

Article Title: Unexpected impairment of I Na underpins reentrant arrhythmias in a knock-in swine model of Timothy syndrome

doi: 10.1038/s44161-023-00393-w

Figure Lengend Snippet: Surface ECG (left), unipolar electrograms with local activation time depicted with white circles (central) and electroanatomical maps (right) documenting polymorphic VT induction in a TS1 pig after 3 extrastimuli. In analogy with Fig. (see main text), the first activation during S4 corresponds to the pacing site in the right ventricle (*). Latest activated area was found in the left ventricle (**), while the first spontaneously reactivating region was found in the proximal site in the right ventricle (***), which corresponded to the area with the shortest LRT of the closest S4 that did not induce VF and was followed again by left ventricle activation (****). The black arrow indicates the pacing site in the RV endocardium.

Article Snippet: We recorded the surface ECG at 1 kHz for at least 5 min (Mortara Instruments) (paper speed 25 mm s −1 and voltage settings 10 mm mV −1 ) in both TS1 and WT animals.

Techniques: Activation Assay

Journal: Biomedicines

Article Title: Endurance Training Provokes Arrhythmogenic Right Ventricular Cardiomyopathy Phenotype in Heterozygous Desmoglein-2 Mutants: Alleviation by Preload Reduction

doi: 10.3390/biomedicines12050985

Figure Lengend Snippet: ECG parameters of one-year-old, sedentary Dsg2 0/wt and Dsg2 WT mice.

Article Snippet: Surface electrocardiography (ECG) was recorded non-invasively (emka TECHNOLOGIES—Paris, France) in mice anaesthetized with urethane (2 mg/kg) before heart extraction.

Techniques:

Journal: Biomedicines

Article Title: Endurance Training Provokes Arrhythmogenic Right Ventricular Cardiomyopathy Phenotype in Heterozygous Desmoglein-2 Mutants: Alleviation by Preload Reduction

doi: 10.3390/biomedicines12050985

Figure Lengend Snippet: ECG data of Dsg2 WT and Dsg2 mt/wt mice aged 29 weeks, which served as sedentary control group, and trained Dsg2 mt/wt and Dsg2 WT mice with placebo or preload-reducing therapy.

Article Snippet: Surface electrocardiography (ECG) was recorded non-invasively (emka TECHNOLOGIES—Paris, France) in mice anaesthetized with urethane (2 mg/kg) before heart extraction.

Techniques: Control

Journal: Biomedicines

Article Title: Endurance Training Provokes Arrhythmogenic Right Ventricular Cardiomyopathy Phenotype in Heterozygous Desmoglein-2 Mutants: Alleviation by Preload Reduction

doi: 10.3390/biomedicines12050985

Figure Lengend Snippet: Right ventricular enlargement in Dsg2 mt/wt mice after endurance swim training is alleviated by preload-reducing therapy. ( A – D ) Representative echocardiographic images of the short-axis view during diastole after endurance swim training of Dsg2 WT ( A ) and Dsg2 mt/wt ( B ) mice treated with placebo and Dsg2 WT ( C ) and Dsg2 mt/wt ( D ) mice with preload-reducing treatment therapy. The blue dotted line surrounds the area measured of the right ventricle in the short-axis view. ( A’ – D’ ) MRI showing prospectively triggered cardiac Cine magnetic resonance images acquired at 9.4 T (groups and protocols as in ( A – D )). ( E ) Bar graphs showing increased left ventricular mass/body weight (LVmass/BW) index after endurance swim training without (placebo) and with preload-reducing therapy (* p < 0.05). Augmentation of the index provides evidence that the training was effective. ( F ) Bar graphs representing the measured diastolic right ventricular (RV) area in the short axis view during echocardiography before and after training, without (placebo) and with preload reduction therapy in Dsg2 WT and Dsg2 mt/wt mice (* p < 0.05). After training, Dsg2 mt/wt mice showed an increased RV short axis area. ( G ) Bar graphs showing calculated diastolic RV volume before and after training, without (placebo) and with preload reduction therapy in Dsg2 WT and Dsg2 mt/wt mice (* p < 0.05). After training, RV volume was enlarged in trained and placebo-treated Dsg2 mt/wt mice compared with before training and with trained and untreated Dsg2 WT mice. ( H ) Contractility of the RV shown by the fractional area change (FAC) measured in the short axis view (sav) before and after training, with placebo and with therapy in Dsg2 WT and Dsg2 mt/wt mice (* p < 0.05). After training, FAC RV sav was reduced in trained Dsg2 mt/wt mice.

Article Snippet: Surface electrocardiography (ECG) was recorded non-invasively (emka TECHNOLOGIES—Paris, France) in mice anaesthetized with urethane (2 mg/kg) before heart extraction.

Techniques: