Focal scar and diffuse myocardial fibrosis in patients with history of repaired Tetralogy of Fallot

Focal scar and diffuse myocardial fibrosis in patients with history of repaired Tetralogy of Fallot

Archives of Cardiovascular Disease Supplements (2018) 3, 273—276 Available online at ScienceDirect www.sciencedirect.com 13 SEPTEMBER 2018 Oral po...

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Archives of Cardiovascular Disease Supplements (2018) 3, 273—276

Available online at

ScienceDirect www.sciencedirect.com

13 SEPTEMBER 2018

Oral posters session PC 1

Accuracy of new transthoracic 3D echocardiographic automated software for left heart chamber quantification in children Romain Amadieu 1,2,∗ , Khaled Hadeed 2 , Marion Jaffro 3 , Clément Karsenty 2 , Miarisoa Ratsimandresy 2 , Aitor Guitarte Vidaurre 2 , Yves Dulac 2 , Philippe Acar 2 1 Pediatric Intensive Care Unit, Children’s Hospital, CHU Toulouse, France 2 Department of Pediatric Cardiology, Children’s Hospital, CHU Toulouse, France 3 Department of Radiology, Toulouse University Hospital, CHU Toulouse, France ∗ Corresponding author. E-mail address: [email protected] (R. Amadieu) Introduction A new three-dimensional echocardiographic (3DE) automated software (HeartModel) is now available to quantify left heart chamber. The aims of this study were to assess the feasibility of this technique in children; and its correlation with manual 3DE and cardiac magnetic resonance (CMR) for measuring left ventricular (LV) and left atrium (LA) volumes and LV ejection fraction (LVEF). Methods Ninety-two children (5 to 17 years) were prospectively included in two separate protocols. In protocol, 1, 73 healthy children (8.8 ± 3.0 years) underwent 2D and 3D transthoracic echocardiography (EPIQ 7 C, X5-1, Philips Healthcare). LV enddiastolic volume (LVEDV), LV end-systolic volume (LVESV), LVEF and LA volume at ventricular end-systole (LAV) obtained with automated 3DE (Fig. 1) were compared with the manual 3DE measurements. In protocol 2, automated 3DE measurements from 19 children with cardiopathy (12.8 ± 2.9 years) were compared with CMR values. Test-retest, intraobserver and interobserver variability and the mean analysis time per patient were also examined for 3DE measurements. Results Automated 3DE was feasible in 77% of datasets and reduced significantly the mean time per patient required for indices analysis compared with manual 3DE (20 ± 2 versus 125 ± 24 seconds, P < 0.0001), even when contour adjustment was performed (29 ± 10, p < 0.0001). In protocol 1, there was excellent correlation for LVEDV, LVESV and LAV between automated and manual 3DE (r = 0.89 to 0.99, all P < 0.0001) but less for LVEF despite contour adjustment (r = 0.56 to 0.57, all P < 0.0001). Compared with manual 3DE, automated 3DE without contour edit overestimated LVEDV, LVEF and LAV with small biases and underestimated LVESV with wider bias. With contour adjustment, the biases and limits of agreement (LOA) were reduced (biases: 0.9 mL for LVEDV, −1.2 mL for LVESV, 2.2% for

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LVEF; relative biases: 1.3% for LVEDV, −4.5% for LVESV). In protocol 2, there were excellent correlation for LV volumes and moderate correlation for LAV between automated 3DE with contour edit and CMR (r = 0.76 to 0.94, all p < 0.0003) but the correlation for LVEF remained weak (r = 0.46, p = 0.05). Compared with CMR, automated 3DE with contour edit slightly underestimated LVEDV and LVESV (relative biases: −7.2 to −7.8%), underestimated LAV with larger bias (relative bias: −31.6%), and had a negligible bias for LVEF (1.0%). However, LOA were clinically acceptable only for LVEDV and LVEF. Test-retest, intraobserver and interobserver variability for automated 3DE measurements were low (< 12%). Conclusions HeartModel is a promising software for fast assessment of left heart chamber volume and function. Its feasibility in children aged more than 5 years is good, with high reproducibility. The automated 3DE measurements of LV and LA volumes are comparable to manual 3DE, especially when contour adjustment of automated 3DE values is performed. Compared with CMR, LVEDV and LVEF measured by automated 3DE with contour edit seem interesting in clinical practice. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvdsp.2018.06.004 PC 2

Focal scar and diffuse myocardial fibrosis in patients with history of repaired Tetralogy of Fallot Hubert Cochet a,b , Xavier Iriart c,∗ , Antoine Allain-Nicolaï a , Claudia Camaioni a , Soumaya Sridi a , Hubert Nivet a , Emmanuelle Fournier c , Marie-Lou Dinet c , Zakaria Jalal c , Francois Laurent a,b , Michel Montaudon a,b , Jean-Benoît Thambo a,c a Department of Cardiovascular Imaging, hôpital Cardiologique du Haut-Lévêque, CHU de Bordeaux, Pessac, France b IHU LIRYC, université de Bordeaux—Inserm U1045, Pessac, France c Department of Pediatric and Adult Congenital Cardiology, hôpital Cardiologique du Haut-Lévêque, CHU de Bordeaux, Pessac, France ∗ Corresponding author. E-mail address: [email protected] (X. Iriart) Background Left and right ventricular (LV and RV) remodelling in repaired tetralogy of Fallot (TOF) is poorly understood. Objectives To identify correlates of focal scar and diffuse fibrosis in patients with history of TOF repair by using cardiac magnetic resonance (CMR).

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13 September 2018 PC 3

The first population-based evaluation of complex congenital heart diseases and its management in French Guiana: An insight from the M3 C network project Hugues Lucron 1,∗ , Mélanie Brard 1 , Véronique Lambert 2 , Laurence Long 3 , Marion Restrepo 2 , Alexandre Bretonneau 1 , Narcisse Elanga 3 , Jean-Luc Deshayes 4 , Michele Gueneret-Bru 1 , Jean-Albert Will 4 , Sylvie Duversnois 5 , Eugénie Jolivet 1 , Ahoussou Dotou 3 , Bruno Schaub 1 , Rishika Banydeen 1 , Jocelyn Inamo 1 1 CHU de Martinique, Pediatric Cardiology, Antilles-Guyane M3 C Center, 97261 Fort-de-France, Martinique, France 2 Centre Hospitalier de L’Ouest Guyanais, Fetal and Pediatric Department, 97393 Saint-Laurent-du-Maroni, Guyane, France 3 Centre Hospitalier Andrée Rosemon, Fetal Unit, 97306 Cayenne, Guyane, France 4 Imagerie Médicale, Cayenne, Guyane, France 5 Centre hospitalier de Kourou, Fetal Unit, 97387 Kourou, Guyane, France ∗ Corresponding author. E-mail address: [email protected] (H. Lucron)

Fig. 1 Three-dimensional echocardiographic (3DE) automated software (HeartModel) analysis of left heart chambers. Methods Patients with prior TOF repair underwent CMR including cine imaging to assess ventricular volumes and ejection fraction (EF), T1 mapping to assess LV and RV diffuse fibrosis, and high resolution late gadolinium-enhanced (LGE) imaging to quantify scar size. Structural imaging data were related to clinical characteristics and functional imaging markers. In 40 patients, cine and T1 mapping results were compared to age- and sex-matched controls. Results One hundred and three patients were enrolled (age 28 ± 15 years, 36% women), including 36 with prior PV replacement. Compared to controls, TOF patients showed lower LV and RVEF and higher RV volume, RV wall thickness, and native T1 and ECV values on both ventricles. Scar size related to LVEF and RVEF while LV and RV native T1 related to RV dilatation. On multivariable analysis, scar size and LV native T1 were independent correlates of ventricular arrhythmia. Patients with history of PV replacement showed larger scar on RV outflow tract but LV and RV native T1 were shorter. Conclusions Focal scar and biventricular diffuse fibrosis are detected on CMR after TOF repair. Scar size relates to systolic dysfunction, and diffuse fibrosis to RV dilatation. Both may be implicated in ventricular arrhythmias. The finding of shorter T1 after PV replacement suggests that diffuse fibrosis may reverse. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvdsp.2018.06.005

Basics Due to its diverse population composition and the paucity of medical resources, the Antilles-Guyane M3 C pediatric cardiology center based in Martinique, provides regular medical support to French Guiana (other overseas territory) using a collaborative dedicated network. To date, prevalence, efficacy of prenatal screening, as well as outcomes of complex congenital heart disease (cCHD), remain totally unknown in this part of the world. Methods A prospective population-based study of all cCHD cases identified from January 2012 to December 2016 including live births, terminations of pregnancy for fetal anomalies (TOPFA), fetal deaths identified either prenatally or up to 1 year of age in the birth cohorts. Results We identified a total of 71 (47 fetal, 24 post natal) cCHD cases over 33796 births (32,975 live births). The total prevalence of cCHD was 21 for 10,000 births while live birth prevalence was 16.98 per 10000. Non-chromosomal isolated cCHD (39 fetal, 17 post natal, TOPFA in 38.4%) occurred in 12.43 per 10,000 live births. Fetal studies allowed an overall detection rate of 66% of all cCHD and 100% of univentricular hearts. The prevalence (7.98 per 10,000) of functionally univentricular hearts (27 cases including 19 hypoplastic left heart syndrome: TOPFA rate 51.8%) exceeded the expected average observed in other European or American populations. Finally, the overall one- year mortality of live births infants with cCHD was found significant (51.5%), mostly related to compassionate care policy, geographical location and other major considerations (anatomical findings, prematurity, birth weight, non-cardiac problems, social issues). Conclusions Despite very low medical resources, the cCHD detection program in French Guiana appears effective. Complex congenital heart diseases, especially univentricular hearts, are common and deserve strict medical attention. High prevalence might suggest for additional unknown risk factors. Disclosure of interest The authors have not supplied their declaration of competing interest. https://doi.org/10.1016/j.acvdsp.2018.06.006