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Transcatheter correction of sinus venosus defects (SVD) with covered stent has recently emerged, with promising results, as an alternative to surgery. Our aim was to assess the feasibility and diagnostic value of 4D flow MRI in patients who underwent transcatheter repair of SVD. Since may 2023, we scanned 16 patients who underwent transcatheter repair of SVD with cardiac magnetic resonance (CMR) including a 4D Flow MR sequence, using a 1.5T magnet. For all patients, balanced steady-state free precession (bSSFP) cine images were obtained for evaluation of left and right ventricle (RV) volumes and function. Coronal ECG-gated 4D flow MR sequences were performed, with complete thoracic coverage. Global objective was, by a visual and quantitative analysis, to detect abnormal flow patterns and location of persistent shunts (PS). Pulmonary-to-systemic flow ratios (Qp/Qs) and RV end-diastolic volume indexed (RVEDVi) were also measured. CMR and 4D Flow MR were feasible and assessed global success of the procedure in all 16 patients. RVEDVi decreased in all patients. Average Qp/Qs was 1.24 (± 0.33), with RVEDVi of 77 mL/m2 (± 21). Qp/Qs measured by catheterization, 4D Flow MR and RV/LV stroke volume ratio were well correlated. Twelve out of 16 patients (75%) had no significant PS with Qp/Qs < 1.5, including 8 patients without any PS at all. Four patients showed significant shunting with Qp/Qs > 1.5. PS visual analysis provided valuable insights into the understanding and the mechanism analysis of the PS. We demonstrated the feasibility of 4D Flow MRI in all patients who had undergone transcatheter correction of SVD. 4D flow MRI accurately identified PS with comprehensive visualization of their location and mechanism, providing valuable information for their follow-up and to further discuss reintervention. Qp/Qs ratio assessment using 4D Flow data showed good correlation compared to other functional measurements. [ABSTRACT FROM AUTHOR]

Since the 2000s, transcatheter closure has been the primary treatment for ostium secundum atrial septal defect (osASD) in children and adults. This study aims to identify factors associated with short-term adverse outcomes following this procedure in a large cohort. A prospective, single-center cohort study included 2,253 consecutive patients (median age 28 years; children: n = 865, 38.4%) who underwent transcatheter ASD closure with the Amplatzer Septal Occluder (ASO; Amplatzer™ Atrial Septal Occluder Device, Abbott, Chicago, USA) from May 1998 to December 2021. Peri-procedural data associated with major adverse events were investigated retrospectively. The mean ASD diameter, as measured by transthoracic echocardiography, was 18 mm. About 8.9% of patients had an ASD size-to-body surface area (BSA) ratio of ≥ 20 mm/m2. Deficient rims (< 5 mm) were identified in 27.9% of patients, with retroaortic rim deficiency in 22.7% and inferior rim deficiency in 0.9%. The median ASO diameter was 24 mm, with a procedural success rate of 98.2%. ASD/BSA ≥ 20 mm/m2 was associated with procedural failure, while age and weight were not. Major peri-procedural adverse events occurred in 31 patients (1.4%), with 19 device embolizations and 2 cardiac erosions. No peri-procedural deaths were reported. Multivariate analysis showed that deficiency of the inferior vena cava rim and an ASD size/BS ratio ≥ 20 mm/m2 were significantly associated with major adverse events (P = 0.002 and P = 0.035, respectively) (Fig. 1). Transcatheter osASD closure using ASO is safe and effective in a large spectrum population with low rate of peri-procedural adverse events and favorable short-term outcomes. ASD size-to-body surface area ratio (≥ 20 mm/m2) and inferior rim deficiency are key morphological risk markers for major adverse events following this procedure. [ABSTRACT FROM AUTHOR]

Ventricular arrhythmias and sudden death are recognized complications of tetralogy of Fallot (TOF). Electrophysiological study (EPS) is increasingly performed prior to pulmonary valve replacement (PVR) in these patients to evaluate and treat the arrhythmic substrate. We aimed to assess the value of cardiac magnetic resonance (CMR) to refine pre-operative risk stratification in this population. A prospective multicenter study was conducted to systematically assess EPS in patients with TOF referred for PVR from January 2020 to December 2021 in 5 tertiary centers in France. CMR parameters including right ventricular (RV) - left ventricular (LV) volumes, function, and as well as pulmonary regurgitant fraction and pulmonary annulus diameter were assessed. A positive EPS was defined as inducibility of a sustained ventricular arrhythmia (VA). A total of 120 patients were enrolled. Among the 108 (90.0%) patients who had a CMR prior to EPS, 24 (22.2%) had a VA inducible. Clinical characteristics of patients associated with EPS results are presented in table1. Concerning CMR variables, mean RV end-diastolic volume index was 157 ± 39 ml and was not statistically different in patients with positive vs. negative EPS (164 ± 49 vs. 155 ± 35, p = 0.395). However, a RV/LV end-diastolic volume index ratio > 2.4 was significantly associated with a higher likelihood of inducibility (OR 3.10, 95%CI 1.14–8.74, p = 0.040). Mean RV end-systolic volume index tended to be upper in patient with positive EPS (101 ± 35 vs. 86 ± 26, p = 0.072). The RV ejection fraction (RVEF) measured on CMR also tended to be slightly lower in patients with positive EPS (40 ± 7 vs. 43 ± 9, p = 0.069) unlike the LV ejection fraction (LVEF) (p = 0.600). Mean pulmonary annulus diameter were significantly associated with positive EPS (31 ± 8 vs. 26 ± 7, OR 1.10 [1.02–1.18], p = 0.012) and also with a cut-off > 28 mm (60.9% vs 34.4%, OR 2.92 [1.10–8.11] p = 0.047). Others non-CMR factors associated with inducible VA are presented in Table 1. In patients with TOF prior to PVR, RV/LV end-diastolic volume index ratio and pulmonary annulus diameter are associated with ventricular arrhythmia inducibility during EPS. CMR parameters, in combination with other clinical factors, may be useful to improve pre-operative risk stratification. [ABSTRACT FROM AUTHOR]

Hereditary transthyretin (ATTRv) p. Val142Ile (V122I) genetic variant is the most common inherited cause of cardiac amyloidosis. Homozygote phenotype is still little studied. The objective of this study was to compare phenotypic characteristics and outcomes between heterozygous and homozygous patients with ATTRv V122I pathogenic variant. We conducted a monocentric, observational, retrospective study of patients referred to the National Reference Center for Cardiac Amyloidosis (Henri Mondor Hospital, Créteil), carrying ATTRv V122I. We analyzed and compared epidemiological, clinical, electrocardiographic, cardiac imaging and prognostic data. In all, 185 patients carrying the V122I mutation, including 161 heterozygotes and 24 homozygotes, we found 13% of homozygosity prevalence and a significantly earlier onset in homozygous population compared to heterozygous concerning: median age at diagnosis (67 years vs. 76 years), first cardiac symptom (66 years vs. 74 years) and first extracardiac symptom (59 years vs. 69 years) (Fig. 1). In the Kaplan-Meier event free survival curve according to age, median age at death, heart transplantat, circulatory support or first acute heart failure was significantly lower in homozygotes compared with heterozygotes (68 vs. 76 years, P = 0.018) (Fig. 2). This study is the largest homozygous V122I cohort reported and gives new insights regarding this condition. [ABSTRACT FROM AUTHOR]

French guideline recently added the presence of an "abnormal left ventricle" on transthoracic echocardiography (TTE) to define very high-risk diabetic patients who may benefit from silent coronary artery disease (CAD) screening. We aimed to investigate the incremental value of resting Global Longitudinal Strain (GLS) in predicting stress TTE positivity in asymptomatic patients with diabetes mellitus (DM). Consecutive asymptomatic patients with DM referred for screening for silent CAD by stress TTE (effort of dobutamine) between January 2017 and December 2018 were included (n = 273). Stress test positivity was defined as stress-induced new or worsening of preexisting left ventricular wall motion abnormalities. Patients with a positive stress test (n = 28; 10%) were more likely to be smokers (71% vs. 17%), to have known diabetes for more than 10 years, carotid atheroma, diabetic nephropathy, diabetic retinopathy (57.1% vs. 18.0%), moderate or severe aortic or mitral calcifications and a lower GLS (−16.7 ± 2.9 vs. −18.9 ± 2.2%) than those with a negative test (all P ≤ 0.014). On multivariate logistic regression analysis, a DM duration > 10 years, diabetic retinopathy and lower GLS (OR [95% CI] = 0.76 [0.64–0.93] per % decrease) remained independently associated with a positive stress TTE. The Chi2 of the model was significantly improved by the addition of GLS on top of the baseline clinical variables, repolarization abnormalities and DM related complications (Chi2 56.9 to 73.8; P < 0.001) (Table 1, Fig. 1). Screening CAD in asymptomatic patients with DM is challenging and debated. In this context, it is crucial to identify early markers of CAD in this population. Our study shows that GLS is strongly associated with the presence of silent ischemia and could be one of these new parameters to be routinely assessed in this population. [ABSTRACT FROM AUTHOR]

Wolff Parkinson White syndrome is responsible of junctional tachycardia and potentially sudden death in children. Precise location of accessory pathway (AP) is crucial to increase safety and success rate of ablation procedures. Known ECG localization algorithms have limited accuracy in children close to 50%. The aim of study was to evaluate a new ECG algorithm relying on maximal pre-excitation (EMP) in children. ECG with EMP (ventricular activation secondary to AP without His bundle activation), induced by atrial pacing or adenosine test/vagal maneuver, was prospectively recorded during an electrophysiological study in 90 consecutive children under 16, in 3 tertiary centers between 2008 and 2020. 3 blinded investigators (1: expert rythmologist, 2: pediatric cardiologist and 3: pediatric cardiologist in training) tested a new algorithm based on EMP to predict AP location and 2 validated control algorithms, from Arruda and Min Baek , relying on basal pre-excitation. Predicted locations were matched with ablation-verified AP location. Sensibility, specificity, negative/positive predictive values and reproducibility, defined as level of agreement between investigators in determining AP location, were evaluated. AP were Left sided in 38%, septal in 47% and right sided in 16%. With the new algorithm, predictive accuracy for the 9 exact locations was 91%, 89% and 90% for investigator 1, 2 and 3, respectively. Sensibility was 80-100% for right sided, 95–100% for left sided and 62-100% for septal AP. Concordance between investigators was excellent (k ≥ 0,86). The accuracy for the reference algorithms remain poor (45% for Arruda and 51% for Min Baek) with less concordance between investigators. Our new algorithm based on EMP allows accurate and easy to use localization of AP in children. This algorithm can be employed before cardiac catheterization and can allow better evaluation of the benefit/risk ratio of the intervention. [ABSTRACT FROM AUTHOR]