Introduction Congenitally corrected transposition of the great arteries (ccTGA) is a rare, complex and challenging structural heart disease. Therapeutic management remains controversial, particularly in patients with left ventricle outflow tract obstruction (LVOTO). As the natural history of ccTGA with LVOTO is overall very satisfactory and interventions that increase sub-pulmonary left ventricle (LV) pressure load are associated with a reduction of tricuspid regurgitation (TR) and systemic right ventricle (RV) dysfunction, we began to use restrictive left ventricle-to-pulmonary artery (LV-PA) conduit without ventricular septal defect (VSD) closure as part of the physiological repair of ccTGA/VSD/LVOTO. Objective To report on the outcome of ccTGA/VSD/LVOTO patients treated with restrictive LV-PA conduit without VSD closure. Methods Between 1979 and 2024, 9 consecutive patients with ccTGA/non-restrictive VSD/LVOTO underwent restrictive LV-PA conduit interposition without VSD closure at Necker Sick Children's Hospitals (Paris, France). Results Six (66.7%) patients had pulmonary stenosis and 3 (33.3%) patients had pulmonary atresia. Median age and weight at surgery were 5 (IQR: 16.6) years and 18 (IQR: 36.5) kgs. Five (55.6%) patients had previously undergone surgical palliation. No patient died after surgery with a median follow-up of 9.8 (IQR: 13.4) years. No patient required permanent pacemaker implantation for complete AV block. Freedom from reintervention was 62.5% (95% CI: 22.9–86.1) at 10years. Late reintervention consisted in LV-PA conduit replacement (n=3), PA stenting (n=1) and one and half ventricle repair (hemi-Senning, Glenn, Rastelli, n=1). At last follow-up, only one patient was significantly limited in physical activity (NYHA class III). No patient developed atrial or ventricular arrythmia. No patients developed moderate or severe TR and only 2 (22.2%) patients had decreased RV function and dilatation. Conclusion We believe that restrictive LV-PA conduit placement without VSD closure represents a worthwhile biventricular alternative strategy to anatomic repair when it is deemed too risky and/or impractical.

Several surgical techniques can be used to repair anomalies of the ventriculoarterial connection with sub-pulmonary stenosis. Although there is no equivocal evidence that one technique is superior to another, the arterial switch operation (ASO) and Rastelli procedure are the preferred options to correct transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO). Despite innovative features and satisfactory short- and long-term results, the “réparation à l’étage ventriculaire” (REV) procedure has not gained large popularity and still struggles to establish itself as a legitimate alternative to the Rastelli operation. By describing our long-term results, we wish to address the misconceptions about the technical aspects and the long-term results of this technique that have been suggested to explain the reluctance towards it. Between 1980 and 2022, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, VSD and LVOTO (n = 113, 72.0%). Sixty-seven patients (42.7%) underwent a Rashkind procedure, and 67 patients (42.7%) had a prior surgical palliation (including 62 systemic-to-pulmonary artery shunts). Resection of the conal septum and/or ventricular septal defect enlargement was performed in 65 patients (41.4%). Thirteen patients (8.3%) died, including 4 during the first postoperative month and 2 after heart transplant. Overall survival at 40 years was 89%. Thirty-seven patients (23.6%) required 67 reinterventions on the right ventricular outflow tract (RVOT), including 49 reoperations, with a median delay of 9 years after the REV (8 months – 27 years). Twenty patients (12.7%) underwent RVOT valvulation (16 surgical and 4 interventional). Freedom from RVOT reintervention and reoperation at 40 years were 60.3% and 62.6% respectively. Four patients (2.5%) required reoperation for left ventricular outflow tract obstruction, with a median delay of 4.8 years (Fig. 1). The REV procedure is a good alternative to the Rastelli intervention when an arterial switch operation is not feasible. A quarter of the patients required redo surgery on the right ventricular outflow tract. Reoperations for left ventricular outflow tract obstruction are scarce.

Aortic valve (AV) pathology in adult congenital heart disease (ACHD) patients has its own etiologies: bicuspid aortic valve (BAV), conotruncal anomalies, Laubry-Pezzi syndrome, subvalvular aortic membrane, genetic disorders or postoperative lesions (Ross procedure or previous AV repair). Following the example of mitral plasty in the 1980s, surgical techniques for AV repair have been standardized since the 2000s and show very good results in selected patients, allowing a substantial reduction in the risk of prosthesis-related complications. To present an accurate and reproducible transthoracic and transesophageal echo protocol analysing aorta phenotypes and AV and predict the immediate success of AV repair in ACHD patients. The study involved a retrospective analysis of all the ACHD patients operated between January 2018 and May 2022 for AV surgery at the Georges Pompidou European Hospital in Paris. Clinical characteristics, type of ACHD, severity and type of valve involvement (insufficiency, stenosis, or dilatation of the aortic root) were collected. Preoperative and postoperative transthoracic and transesophageal echography were performed using a unique protocol with a 3D approach. Preoperatively, the AV dysfunction, its lesion at the origin and its etiology were defined. Measurements of aortic annulus, effective heights, geometric heights, commissural angle if BAV were made, and the presence of fenestrations, calcifications was noted. In case of AV repair, all measurements were repeated postoperatively. The surgical techniques of aortic plasty were noted. Sixty-three patients were analyzed, 46% of whom were referred for aortic plasty. 50% of them had BAV, 30% of them were redux of AV repair (20%) or Ross procedure (10%), 20% had conotruncal anomalies. Aortic plasty was performed in 33% of patients, including external ring annuloplasty in case of AV regurgitation with an annulus diameter ≥ 25 mm. ACHD patients are challenging patients for AV repair. The external aortic ring annuloplasty technique makes AV repair accessible in case of dilated aortic annulus, in expert surgeons' hands. A standardized echo protocol for AV repair helps to predict the reparability of an AV and is applicable in ACHD patients.

Central illustration. Evolution of acute procedural success rates. Partial acute procedural success was defined as termination of clinical arrhythmia with subsequent verification of bidirectional conduction block with additional arrhythmias induced not targeted or not successfully targeted or absence of final programmed stimulation performance. Complete acute procedural success was defined as termination of clinical arrhythmia(s) with subsequent verification of bidirectional conduction block and non-inducibility of other arrhythmias or successful ablation of all secondary arrhythmias induced (except inducible atrial fibrillation [AF]). Final programmed stimulation performance was not required to consider complete acute procedural success when the targeted arrhythmia was AF, premature ventricular contraction or atrioventricular node. The red line represents the rate of final programmed stimulation. FAT: focal atrial tachycardia; IART: intra-atrial reentrant tachycardia.

Abstract Background The arterial switch operation (ASO) has replaced atrial switch procedures for D-transposition of the great arteries (D-TGA), with 90% of patients now reaching adulthood. However, patients may have residual lesions and/or sequelae, some of which may remain unrecognized, necessitating lifelong specialist surveillance. We examined the cardiac outcomes of a large number of contemporary ASO patients under tertiary adult congenital heart disease (ACHD) care. Methods We examined late major adverse cardiovascular events (MACE) in adult TGA patients (>16 years) who underwent an ASO between 1981 and 2003 and continued their follow-up in 2 tertiary ACHD centers. MACE were defined as death, re-intervention, myocardial ischemia, arrhythmia, stroke/TIA, infective endocarditis and heart failure. Results Overall, 199 patients (66% male, mean age 27±5 years) were followed in adult life for a median of 10 years [IQR 7–15] and were included in this study. Overall survival during this period was 99.5% (95% confidence interval [CI]: 94.4%-99.8%). Sixty-two (31.2%) patients experience MACE, including 52 reinterventions. MACE and reintervention-free survival at 20, 30 and 35 years were 87.6%, 58.6%, 50.6% and 89.5%, 69.1, 61%, respectively. Atrial arrhythmia was the most frequent cardiac event with an incidence of 5.5 cases per 1000 patient-years, whereas incidence of ventricular tachycardia and sudden cardiac death was 1.8‰ and 0.9‰ patient-years, respectively. Coronary artery disease was diagnosed in 6 (3%) patients, of whom 4 had symptoms, 1 had ST depression on ECG at rest and 3 had abnormal wall motion on echocardiography. The most frequent indication for reoperation was right ventricular outflow tract obstruction (n=35/52, 63.7%), whereas left ventricular outflow tract (LVOT) re-interventions rate increased significantly during adulthood compared to childhood from 1% to 5%, p=0.03 (Figure 1). On multivariate analysis, history of cardiac complications during infancy (HR 2.3, 95% CI:1.3–4.0, p<0.01) and uncommon coronary patterns (HR for type A versus B/C/D/E 0.47, 95% CI:0.26–0.83, p<0.01) were independent predictors of MACE in adulthood. At the latest follow-up, 90.9% of patients were functional class I, left ventricular ejection fraction was 59.6±6.5% and peak oxygen uptake 71.1±24.9% predicted. At least moderate neoaortic regurgitation and aortic dilatation (≥40mm) were present in 8.0% and 35.2%, respectively, with more than mild pulmonary stenosis in 19.6%. Conclusion Adult patients with ASO for TGA have a low late mortality. However, MACE are common requiring reintervention, particularly for RVOT obstruction and neo-aortic valve dysfunction, the latter with rising rates during adulthood. Patients with cardiovascular complications during childhood are at the highest risk for MACE. All patients merit life-long tertiary care. Figure 1 Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Assistance Publique des Hôpitaux de Paris

Prenatal diagnosis of transposition of the great arteries (TGA) reduces neonatal mortality and morbidity. The mortality of prenatally diagnosed TGA remains scarcely described. To review the experience of prenatal diagnosis and in utero transfer of fetuses with TGA to assess the limits of this program and to describe outcomes of prenatally diagnosed TGA. From 1999 to 2017, we reviewed all cases of prenatally diagnosed TGA born at our institution. Files were analyzed for discordances between prenatal and postnatal diagnosis, status at birth, neonatal management, and outcomes. In total, 748 fetuses with TGA (470 simple, 278 complex) were delivered at our institution. Discordance between pre- and postnatal diagnosis was noted in 14.8% with two third having consequence on surgical treatment. Median term was 39 WG. The proportion of premature delivery (< 37WG) was 5.3%, the majority being late-preterm (> 34 WG). Fourteen percent were intubated in the delivery room and 1.7% required resuscitation maneuvers. Sixty-four percent had a Rashkind procedure that was done in 15% of cases in the delivery room. Fifty-five percent received PGE1 infusion and 38% had a Rashkind and PGE1 infusion. There were 9 deaths before surgery (1.2%) with two not related to the TGA. A fourth experienced neonatal complications including mainly respiratory distress requiring ventilation, infection, necrotizing enterocolitis, pulmonary hypertension and iatrogenic events. Surgical mortality was 1.8% (4 simple TGA and 10 complex). Seven additional deaths occurred during the first year follow-up, 6 being related to late cardiac complications. Overall survival at one year was 96%. Prenatal diagnosis and in utero transfer of fetuses with TGA does not eliminate the risk of preoperative mortality. The proportion of immediate distress is high and the need for intensive care in the delivery room is frequent. Finally, preoperative mortality accounts for a third of the one-year mortality in this population.

La naissance anormale de la coronaire gauche a partir du sinus droit avec un trajet interaortopulmonaire est une cause classique de mort subite et d'ischemie myocardique d'effort du sujet jeune. Le mecanisme est une compression de la coronaire lors de la dilatation physiologique a l'effort des gros vaisseaux. Nous rapportons l'observation d'un enfant de 12 ans qui a eu une syncope d'effort precedee d'un angor, lies a cette anomalie. Le coroscanner avec reconstructions tridimensionnelles a permis de confirmer le diagnostic et de preciser le trajet interarteriel de la coronaire gauche ainsi que ses rapports anatomiques avec les vaisseaux de la base. Le scanner cardiaque est l'examen de choix pour le diagnostic et le bilan prechirurgical des anomalies coronaires congenitales. Dans notre pratique, il est realise systematiquement en cas de suspicion d'anomalie de naissance des arteres coronaires, en complement de l'echocardiographie et en substitution de la coronarographie conventionnelle.

Anomalous left coronary artery arising from the right sinus with an inter-aorto-pulmonary trajectory is a classical cause of sudden death and myocardial ischaemia in young adults. The mechanism is compression of the coronary during physiological dilatation of the great arteries on exercise. The authors report the case of a 12 year old child who had syncope on effort preceded by angina due to this malformation. The CT scan with 3-D reconstruction confirmed the diagnosis and the interarterial trajectory of the left coronary artery and the anatomical relationships with the vessels at the base of the heart. Cardiac CT scan is the investigation of choice for diagnosis and preoperative work-up of congenital coronary anomalies. In the authors' experience, the investigation is performed systematically when an anomalous coronary artery is suspected, in addition to echocardiography and instead of conventional coronary angiography.

HomeCirculationVol. 113, No. 2Cardiac Teratoma in a Newborn With Right Ventricular Outflow Tract Obstruction Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessReview ArticlePDF/EPUBCardiac Teratoma in a Newborn With Right Ventricular Outflow Tract Obstruction Phalla Ou, Valérie Dorrière, Daniel Sidi, Damien Bonnet and Pascal Vouhé Phalla OuPhalla Ou From Service de Cardiologie Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP (P.O., V.D., D.S., D.B.), and Service de Chirurgie Cardiaque Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP (P.V.), Paris, France. , Valérie DorrièreValérie Dorrière From Service de Cardiologie Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP (P.O., V.D., D.S., D.B.), and Service de Chirurgie Cardiaque Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP (P.V.), Paris, France. , Daniel SidiDaniel Sidi From Service de Cardiologie Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP (P.O., V.D., D.S., D.B.), and Service de Chirurgie Cardiaque Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP (P.V.), Paris, France. , Damien BonnetDamien Bonnet From Service de Cardiologie Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP (P.O., V.D., D.S., D.B.), and Service de Chirurgie Cardiaque Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP (P.V.), Paris, France. and Pascal VouhéPascal Vouhé From Service de Cardiologie Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP (P.O., V.D., D.S., D.B.), and Service de Chirurgie Cardiaque Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP (P.V.), Paris, France. Originally published17 Jan 2006https://doi.org/10.1161/CIRCULATIONAHA.105.534768Circulation. 2006;113:e17–e18A newborn was referred to our department for severe cyanosis with cardiac systolic murmur. Echocardiography revealed in the right ventricle a 12-mm tumor with the typical aspects of a teratoma. The base of the tumor was implanted in the right ventricle apex and was composed of cysts of various sizes that were separated by solid areas. The sessile portion of the tumor invaginated during systole into the pulmonary artery trunk, causing severe obstruction (Figure 1 and Movies I through III of the Data Supplement). Complete surgical resection was performed without complication. Histological examination confirmed the presumptive imaging diagnosis of cardiac teratoma, revealing extensive foci of necrosis, stratified squamous epithelium, salivary glandular tissue, and mature respiratory epithelium (Figure 2). The child remains free of symptoms and tumor 7 months after surgery. Download figureDownload PowerPointFigure 1. Echocardiography showed in the 4-chamber incidence the typical aspects of a teratoma in the right ventricle, characterized by cysts separated by solid areas (left panel and Movie I). Note the pulmonary obstruction by the sessile part of the tumor (right panel and Movie II and Movie III).Download figureDownload PowerPointFigure 2. Histological examination confirmed the diagnosis of teratoma. a, The sessile part of the tumor that caused pulmonary obstruction; b, histological section revealing cysts separated by foci of necrosis; c, stratified squamous epithelium; d, salivary glandular tissue; and e, mature respiratory epithelium.The online-only Data Supplement can be found with this article at http://circ.ahajournals.org/cgi/content/full/113/e17/DC1.FootnotesCorrespondence to Phalla Ou, MD, Service de Cardiologie Pédiatrique, Hôpital Necker-Enfants Malades, 149 Rue de Sèvres, 75743 Paris Cedex 15, France. E-mail [email protected] Previous Back to top Next FiguresReferencesRelatedDetailsCited By Moreau de Bellaing A, Houyel L and Bonnet D (2019) Perinatal intracardiac teratoma: unusual presentation and review of the literature, Cardiology in the Young, 10.1017/S1047951118002354, 29:3, (439-441), Online publication date: 1-Mar-2019. (2016) Cardiac Lipoma Specialty Imaging: Thoracic Neoplasms, 10.1016/B978-0-323-37706-5.50110-4, (390-393), . Bédard E, Becker A and Gatzoulis M (2010) Cardiac Tumours Paediatric Cardiology, 10.1016/B978-0-7020-3064-2.00054-0, (1055-1065), . Phelps C, Mengshol S and Dunbar Ivy D (2008) A curious isolated cystic lesion of the membranous atrioventricular septum, Cardiology in the Young, 10.1017/S104795110800276X, 18:6, (631-634), Online publication date: 1-Dec-2008. January 17, 2006Vol 113, Issue 2 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.105.534768PMID: 16418441 Originally publishedJanuary 17, 2006 PDF download Advertisement SubjectsCongenital Heart DiseaseEchocardiography

Objective: The main cause of long-term morbidity and mortality after a successful arterial switch operation for transposition of the great arteries is complications at the ostial segments, proximal segments, or both of the retransferred coronary arteries. The purpose of this study was to investigate the clinical usefulness of multislice computed tomographic angiography in detecting ostial and proximal coronary lesions in children having undergone the arterial switch operation for transposition of the great arteries.Methods: Forty-nine children (aged 8.5 +/- 3.9 years) operated on for transposition of the great arteries with the arterial switch operation (follow-up, 8.3 +/- 3.6 years) underwent systematic selective conventional and multislice computed tomographic angiography. The ability of multislice computed tomography in detecting stenosis and other modifications of the coronary arteries' course was analyzed by 2 independent investigators.Results: Multislice computed tomography, as compared with selective conventional coronary angiography, permitted assessment of ostial and proximal coronary segments in every patient. It correctly detected the 4 (8.1%) patients with significant coronary lesions (1 with ostial and 3 with proximal coronary stenosis) that had been identified by means of conventional angiography. Multislice computed tomography clearly showed an abnormal course of the coronary artery between the great arteries with compression of the ostial (1 patient) and proximal (3 patients) segments of the retransferred coronary arteries.Conclusion: These results indicate that multislice computed tomographic angiography is fully accurate in detecting ostial coronary artery stenoses, proximal coronary artery stenoses, or both in pediatric patients having undergone the arterial switch operation for transposition of the great arteries. Our results suggest that multislice computed tomography could be used as a screening technique for detecting coronary complications in the follow-up of the arterial switch operation before having recourse to conventional angiography.

Décrire les protocoles d’acquisition du coroscanner en cardiologie pédiatrique, et discuter ses applications dans la pratique quotidienne. Dans notre pratique quotidienne, nous distinguons deux groupes de patients : le premier concerne les nouveaux-nés et les enfants de moins de six ans pour lesquels nous réalisons une acquisition sans synchronisation à l’ECG. Les enfants reçoivent une légère prémédication et sont maintenus par une contention efficace. Le second concerne les enfants coopérants de plus de six ans pour lesquels nous réalisons une acquisition obligatoirement synchronisée à l’ECG. Ces enfants reçoivent des b-bloquants. Dans tous les cas, nous utilisons les protocoles standard avec un scanner 64 coupes (VCT, GE) : 80 à 100 kV, modulation automatique des mA, injection par une veine périphérique (concentration d’iode, débits, volumes adaptés). L’analyse du coroscanner, sur console de traitement, utilise des reconstructions multiplanaires (MIP, VR, angio3D). En routine, le scanner permet de faire le diagnostic de naissance anormale des artères coronaires dans le cadre d’une malformation cardiaque complexe, ainsi que le suivi des enfants opérés de malformation coronaire en période néonatale. Le coroscanner utilisant les machines de dernière génération (64 coupes) tend à se substituer à la coronarographie invasive. C’est un outil diagnostique décisionnel qui s’intègre dans une activité transversale.

The management of congenital cardiovascular malformations is a diagnostic challenge. It requires accurate assessment of the intra- and extra-cardiac malformations. Three-dimensional imaging by the multislice CT scanner is now a routine investigation for congenital heart disease, complementary to echocardiography, and, increasingly, an alternative to conventional angiography. Three dimensional imaging is particularly useful in the diagnosis of complex congenital heart disease, in the preparation of complex investigations of interventional cardiac catheterisation and the postoperative evaluation of surgical repair. This report describes the different acquisition protocols adapted for children and illustrates the present role of volumic CT scanner in congenital heart disease by clinical examples of everyday clinical practice.

Evaluer la capacité du scanner multicoupe à détecter des lésions coronaires chez les enfants opérés d'une transposition des gros vaisseaux (TGV) par switch artériel. Cinquante patients (âge 5 ± 3,8 ans) opérés d'une TGV (suivi depuis la chirurgie 5,4 ± 2,6 ans) ont eu systématiquement un coroscanner et une coronarographie invasive. Le coroscanner était réalisé avec un scanner 64 coupes (GE, LightSpeed VCT, Milwaukee, USA) : Synchronisation ECG ; 100 kV, modulation des mA ; injection de produit de contraste (300mgI/ml, 3-3,5 ml/sec, 1,5 ml/kg) ; Bêta-bloquant (Propranolol 1 mg/kg). Une visualisation satisfaisante des ostia et des segments proximaux était obtenue avec les 2 examens chez tous les patients. La coronarographie invasive a permis de diagnostiquer des lésions coronaires chez 5 patients (10 %) : occlusion coronaire chez 4 patients, sténose coronaire proximale serrée chez 1 patient. Les 45 autres patients avaient des coronaires normales. Le coroscanner a retrouvé les 4 occlusions et la sténose coronaire proximale chez les 5 mêmes patients qu'à la coronarographie invasive. Il n'y avait aucun cas de faux positif au coroscanner. Le coroscanner permet une excellente visualisation des ostia et des segments proximaux des artères coronaires chez l'enfant. Cette nouvelle technique pourrait être une alternative non invasive et ambulatoire à la coronarographie invasive pour le suivi des enfants opérés d'une TGV par switch artériel.