Clément Karsenty

The medical history of adults with complex congenital heart disease affects their social development and professional activity

BACKGROUND In recent decades, advances in surgery and therapeutic catheterization have steadily increased the life expectancy and prevalence of adults with congenital heart disease (CHD). AIMS We assessed medical and psychosocial variables of adults with CHD, according to the disease complexity. METHODS We included, from a single-centre observational cohort study, 135 consecutive adults with CHD (median age of 40 years, interquartile range: 28.0-51.0) followed in our cardiology unit, who answered a questionnaire assessing daily activity and psychosocial functioning. Disease complexity was classified according to the Bethesda conference. RESULTS Cardiac malformation complexity was simple in 61 (45.2%), moderate in 50 (37.0%) and complex in 24 (17.8%) patients. Cardiac surgery had been performed in 86.5% of moderate and complex patients. Complications (such as heart failure, arrhythmia and pulmonary hypertension) were mainly observed in the complex group (P=0.003). Physical activity was lower in the complex group (no activity in 58.8%, but sport previously contraindicated in 50% of these; P=0.03). Education level tended to be lower in the complex and moderate groups than in the simple group (respectively, 31.2% and 33.3% vs. 45.7% had passed the Baccalaureate; P=0.47). The pass rate was lower in patients with complications (P=0.037) or more than one cardiac surgery (P=0.03). In the complex group, 56.3% of patients were unemployed (P=0.048). CONCLUSIONS Complexity of heart disease and medical history affect education level and employment of adults with CHD. Academic education of children with a complex defect and career counselling are important to prevent unemployment among adults with CHD.

Characteristics and outcomes of heart failure-related hospitalization in adults with congenital heart disease

BACKGROUND Heart failure (HF) is the main cause of death in adult congenital heart disease (ACHD). AIMS We aimed to characterize HF-related hospitalization of patients with ACHD, and to determine HF risk factors and prognosis in this population. METHODS We prospectively included 471 patients with ACHD admitted to our unit over 24 months. Clinical and biological data and HF management were recorded. Major cardiovascular events were recorded for ACHD with HF. RESULTS HF was the main reason for hospitalization in 13% of cases (76/583 hospitalizations). Patients with HF were significantly older (median age 44±14 years vs. 37±15 years; P<0.01), with more complex congenital heart disease (P=0.04). In the multivariable analysis, pulmonary arterial hypertension (odds ratio [OR] 6.2, 95% confidence interval [CI] 3.5-10.7), history of HF (OR 9.8, 95% CI 5.7-16.8) and history of atrial arrhythmia (OR 3.6, 95% CI 2.2-5.9) were significant risk factors for HF-related admissions (P<0.001). The mean hospital stay of patients with HF was longer (12.2 vs. 6.9 days; P<0.01), and 25% of patients required intensive care. Overall, 11/55 (20%) patients with HF died, 10/55 (18%) were readmitted for HF, and 6/55 (11%) had heart transplantation during the median follow-up of 18 months (95% CI 14-20 months). The risk of cardiovascular events was 19-fold higher after HF-related hospitalization. CONCLUSIONS HF is emerging as a leading cause of morbidity and mortality in the ACHD population. Earlier diagnosis and more active management are required to improve outcomes of HF in ACHD.

Multimodal imaging and three-dimensional cardiac computational modelling in the management of congenital heart disease: The secret to getting ahead is to get started

On 28 December 1895, a crowd milled outside the Grand Café in Paris for a mysterious exhibition. For 1 Franc, promoters promised, the audience would witness the first ‘‘living photographs’’ in history. The grainy footage of women emerging from the shadows of a factory is laughably primitive today, but in the Grand Café basement in central Paris that night, the audience gasped, applauded, and laughed. Some simply sat, dumbfounded. At that moment, brothers Auguste and Louis Lumière screened the first moving picture, or movie, in history. Paris embraced the world’s most famous museums and art galleries, alongside a thriving community of artists. Movies —– considered as the ‘‘Seventh Art’’ —– grew up alongside painting and sculpture. Movies were initially scorned, being considered a minor form of art, but opinion has since e t a f

Feasibility of New Transthoracic Three-Dimensional Echocardiographic Automated Software for Left Heart Chamber Quantification in Children

Background: New three‐dimensional echocardiographic automated software (HeartModel) is now available to quantify the left heart chambers. The aims of this study were to assess the feasibility, reproducibility, and analysis time of this technique and its correlation with manual three‐dimensional echocardiography (3DE) and cardiac magnetic resonance (CMR) in children. Methods: Ninety‐two children (5–17 years of age) were prospectively included in two separate protocols. In protocol 1, 73 healthy children underwent two‐dimensional and three‐dimensional transthoracic echocardiography. Left ventricular (LV) end‐diastolic volume (LVEDV), LV end‐systolic volume (LVESV), LV ejection fraction (LVEF), and left atrial volume at ventricular end‐systole (LAV) by automated 3DE were compared with the same measurements obtained using manual 3DE. In protocol 2, automated three‐dimensional echocardiographic measurements from 19 children with cardiomyopathy were compared with CMR values. Results: Automated 3DE was feasible in 77% of data sets and significantly reduced the analysis time compared with manual 3DE. In protocol 1, there were excellent correlations for LVEDV, LVESV, and LAV between automated 3DE and manual 3DE (r = 0.89 to 0.99, P < .0001 for all) and a weak correlation for LVEF, despite contour adjustment (r = 0.57, P < .0001). Automated 3DE overestimated LVEDV, LVEF, and LAV with small biases and underestimated LVESV with wider bias. With contour adjustment, the biases and limits of agreement were reduced (bias: LVEDV, 0.9 mL; LVESV, −1.2 mL; LVEF, 2.2%). In protocol 2, correlations between automated 3DE with contour edit and CMR were good for LV volumes and LAV (r = 0.76 to 0.94, P < .0003 for all) but remained weak for LVEF (r = 0.46, P = .05). Automated 3DE slightly underestimated LV volumes (relative bias, −7.2% to −7.8%) and significantly underestimated LAV (relative bias, −31.6%). The limits of agreement were clinically acceptable only for LVEDV. Finally, test‐retest, intraobserver, and interobserver variability values were low (<12%). Conclusions: HeartModel is feasible, reproducible, faster than manual 3DE, and comparable with manual 3DE for measurements of LV and left atrial volumes in children >5 years of age. However, compared with CMR, only LVEDV measured by automated 3DE with contour edit seems applicable for clinical practice. HIGHLIGHTSAutomated 3DE is feasible, reproducible and fast in children.Correlation for LV and LA volumes between automated 3DE and manual 3DE is excellent.Correlation for LV and LA volumes between automated 3DE and CMR is good.Correlation for LVEF by automated 3DE against manual 3DE and CMR is weak.Good precision is obtained only for LVEDV by automated 3DE with contour edit.

Systemic right ventricular takotsubo cardiomyopathy

Clémence Vernay, Clément Karsenty*, Alban Redheuil, Gilles Soulat, and Laurence Iserin Adult Congenital Heart Disease Unit, Hôpital Européen Georges Pompidou, 20 Rue Leblanc, 75015 Paris, France; and Service de Radiologie, Hôpital Pitié-Salpêtrière, 47-83 Boulevard de l’Hôpital, 75013 Paris, France * Corresponding author. Tel: 133 156093043, Fax: 133 156096654, Email: clement.karsenty@hotmail.fr † The first two authors contributed equally to the study.

Mechanical tachycardia of the slow pathway induced by a percutaneous tricuspid valve device

A 47-year-old woman with Ebstein’s anomaly was referred to our department for management of narrow QRS tachycardia with short RP’ intervals (Figure 1A, left). Her medical history included tricuspid valve plasty followed 10 years later by transcatheter tricuspid valve replacement. One year before presentation, she underwent percutaneous closure of a septal periprosthetic leak by an Amplatzer Duct Occluder. She experienced palpitations a few weeks following implantation and the symptoms progressive until incessant tachycardia despite antiarrhythmic drug therapy. During electrophysiological study, no evidence of dual atrioventricular node (AVN) physiology was found and tachycardia initiation did not depend on AH-interval prolongation. A 1:1 atrioventricular relationship was found with concomitant atrial and ventricular activations (Figure 1A, right). Right atrial activation mapping showed a centrifugal activation pattern with an earliest inferoseptal activation site (Figure 1B, left, the yellow ball indicates the location of the His bundle), concordant with a scar area corresponding to the device (Figure 1B, middle, the blue tag indicates the position of the device). Fluoroscopic views showed the position of the Amplatzer in the septal right atrium (Figure 1B, right, black arrow). A SmartTouch ablation catheter was positioned at the site of earliest atrial activation, which coincided with the position of the atrial tip of the device. Radiofrequency ablation at this site successfully terminated the tachycardia, with further non-inducibility (Supplementary material online, Video S1). This is an original report of a focal junctional arrhythmia caused by a mechanical irritation of the AVN slow pathway by a trans-tricuspid device. Conceptually, this junctional automaticity could be compared with the classical accelerated junctional rhythm provoked by radiofrequency applications at the slow pathway during ablation of AVN re-entrant tachycardia. In this setting, the mechanism of generating

Left atrial flutter after implantation of atrial septal occluder

A 30-year-old man was referred for catheter ablation of a symptomatic atrial flutter 1 year after a secundum atrial septal defect (ASD) closure with an Amplatzer septal Occluder (ASO 26 mm, St Jude Medical). After trans-septal puncture through the native atrial septum, electroanatomical biatrial mapping showed an early activation coming from the right interatrial septum below the ASO. Local electrogram on the left atrium showed a fractionated low voltage continuous signal, which was consistent with a re-entry between the ASO and the right pulmonary veins. Focal radiofrequency ablation terminated the tachycardia. We described, here, a left atrial re-entry related to percutaneous ASD device closure.

Interventional catheterization and echocardiography: An indefectible link illustrated by atrial septal defect closure

asson SAS.All rights reserved. Document downloaded on 26/01/2020 It is forbidden and illegal t maging was available to define simple cardiac structures 1], then technology improved to provide two-dimensional 2D) real-time imaging of the heart and Doppler, which subequently revolutionized non-invasive assessment. By the ate 1980s, echocardiography had developed to the point here it began to replace cardiac catheterization as the priary diagnostic tool for congenital heart disease, reducing he number of cardiac catheterizations. Interventional cardiac catheterization was launched by r Gruntzig, with the first coronary angioplasty, and its evelopment continued with Dr Porstmann’s use of an VALON ® (First Aid Bandage Co., New London, CT, USA) plug o close persistent patent ductus arteriosus, and Dr King’s evices for the closure of secundum atrial septal defects ASDs) [2] to rescue cardiac catheterization. Nevertheless, echnical difficulties (i.e. limited retrievability and large elivery systems) and residual shunts plagued the develpment of these devices, and their application remained imited.

Risk of thromboembolic complications in adult congenital heart disease: A literature review

Adult congenital heart disease (ACHD) is a constantly expanding population with challenging issues. Initial medical and surgical treatments are seldom curative, and the majority of patients still experience late sequelae and complications, especially thromboembolic events. These common and potentially life-threating adverse events are probably dramatically underdiagnosed. Better identification and understanding of thromboembolic risk factors are essential to prevent long-term related morbidities. In addition to specific situations associated with a high risk of thromboembolic events (Fontan circulation, cyanotic congenital heart disease), atrial arrhythmia has been recognized as an important risk factor for thromboembolic events in ACHD. Unlike in patients without ACHD, thromboembolic risk stratification scores, such as the CHA2DS2-VASc score, may not be applicable in ACHD. Overall, after a review of the scientific data published so far, it is clear that the complexity of the underlying congenital heart disease represents a major risk factor for thromboembolic events. As a consequence, prophylactic anticoagulation is indicated in patients with complex congenital heart disease and atrial arrhythmia, regardless of the other risk factors, as opposed to simple heart defects. The landscape of ACHD is an ongoing evolving process, and specific thromboembolic risk scores are needed, especially in the setting of simple heart defects; these should be coupled with specific trials or long-term follow-up of multicentre cohorts.

Two-dimensional right ventricular strain by speckle tracking for assessment of longitudinal right ventricular function after paediatric congenital heart disease surgery.

BACKGROUND Right ventricular (RV) function is a prognostic marker of cardiac disease in children. Speckle tracking has been developed to assess RV longitudinal shortening, the dominant deformation during systole; little is known about its feasibility in children with congenital heart disease (CHD). AIMS To evaluate the feasibility and reproducibility of RV two-dimensional (2D) strain assessed by speckle tracking in infants undergoing CHD surgery compared with conventional markers. METHODS In this prospective single-centre study, RV peak systolic strain (RV-PSS) was measured using 2D speckle tracking in 37 consecutive children undergoing CHD surgery. Examinations were performed the day before surgery, a few hours after surgery and before discharge. Relationships with the z score of tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular systolic velocity (TA Sa) were assessed. RESULTS Median (interquartile range) age was 19 months (5-63); median weight was 9.2 kg (5.3-18.0). RV-PSS analysis was feasible in 92.9% (95% confidence interval [CI]: 86.0-97.1) of examinations. The coefficient of variation was 9.7% (95% CI: 7.4-11.9) for intraobserver variability and 15.1% (95% CI: 12.7-17.6) for interobserver variability. Correlations between RV-PSS and z score of TAPSE and TA Sa were strong (r=0.71, P<0.0001 and r=0.70, P<0.0001, respectively). RV-PSS was significantly reduced after surgery compared with baseline (-10.5±2.9% vs. -19.5±4.8%; P<0.0001) and at discharge (-13.5±4.0% vs. -19.5±4.8%; P<0.0001). Similar evolutions were observed with TAPSE and TA Sa (both P<0.0001). CONCLUSION RV longitudinal strain by speckle tracking is a feasible and reproducible method of assessing perioperative evolution of RV function in children with CHD.