Although echocardiography remains the primary imaging modality for assessing Fontan palliation, a standardized systematic approach for evaluating the univentricular heart throughout the different stages of palliation has yet to be established. This document aims to provide a narrative review and practical guide for the echocardiographic assessment of the univentricular heart at various phases of Fontan palliation. Additional objectives include highlighting the potential of advanced three-dimensional (3D) and four-dimensional (4D) imaging modalities, as well as outlining a systematic strategy for detecting thromboembolic complications. We propose a sequential framework for echocardiographic evaluation, encompassing key anatomical and functional components of the univentricular heart. This includes the atrial septum and pulmonary veins, ventricular function, atrioventricular valve, aorta and neo-aorta, sub-aortic region, aortic arch, and pulmonary arteries. Furthermore, we detail the assessment of pulmonary blood supply at different stages of palliation, including the modified Blalock-Taussig-Thomas (mBTT) shunt, Sano conduit, Glenn procedure, and total cavo-pulmonary connection (TCPC). A comprehensive analysis of potential thrombus formation sites is provided, along with diagnostic pitfalls. Additionally, we outline methods for screening for extracardiac clots following Glenn and TCPC procedures. This document serves as a practical guide for the systematic echocardiographic evaluation of the univentricular heart across all stages of Fontan palliation, offering guidance for clinical practice. It also explores the capabilities of emerging 3D and 4D echocardiographic techniques in univentricular heart assessment and provides an in-depth review of thromboembolic complications, emphasizing key diagnostic challenges.

Cross-sectional cardiac imaging including cardiac computed tomography (CCT) and cardiac magnetic resonance imaging (CMR) allows three-dimensional imaging of intracardiac and extracardiac structures. This study was undertaken to better understand current European practice of pediatric cardiology cross-sectional imaging, in addition to how training is undertaken in different centers. A structured detailed 64-question survey focusing on cross-sectional imaging was circulated to all Imaging Working Group members of the 95 AEPC affiliated centers. Consultants from 42 centers (44

Background Lymphatic disorders such as protein‐losing enteropathy, plastic bronchitis, and chylothorax are important complications of the Fontan circulation and ultimately result in an increased risk of early death. Several European centers are now performing lymphatic procedures. The aim of this study is to map the extent of these lymphatic disorders and treatments provided across European pediatric cardiology centers. Methods and Results A survey was circulated to 49 European pediatric cardiology centers consisting of 37 questions including a mix of binary, categorical, and continuous variables. Thirty‐one centers (63%) participated in the study, performing a median of 250 (interquartile range, 178 ‐ 313) cardiopulmonary bypass procedures per year. Chylothorax emerged as the most prevalent lymphatic disorder followed by protein‐losing enteropathy and plastic bronchitis. The most common diagnostic investigation method was noncontrast magnetic resonance lymphangiography (52%). Eleven centers (35%) conducted lymphatic interventions with a median of 3 (interquartile range, 1 ‐ 4) procedures per year and 12 (interquartile range, 5 ‐ 15) interventions in total per center. Conclusions This study confirms the rarity of and variation in treatment approaches for lymphatic disorders across Europe. With at least 11 centers offering lymphatic interventions, the adoption of these procedures is on the rise in Europe. To improve the quality of care and treatment outcomes for these complex patients, it is crucial to consider evidence‐based lymphatic diagnostics, interventional lymphatic procedures, and the centralization of services in Europe.

BACKGROUND:There is limited data on the organisation of paediatric echocardiography laboratories in Europe. METHODS:A structured and approved questionnaire was circulated across all 95 Association for European Paediatric and Congenital Cardiology affiliated centres. The aims were to evaluate: (1) facilities in paediatric echocardiography laboratories across Europe, (2) accredited laboratories, (3) medical/paramedical staff employed, (4) time for echocardiographic studies and reporting, and (5) training, teaching, quality improvement, and research programs. RESULTS:Respondents from forty-three centres (45%) in 22 countries completed the survey. Thirty-six centres (84%) have a dedicated paediatric echocardiography laboratory, only five (12%) of which reported they were European Association of Cardiovascular Imaging accredited. The median number of echocardiography rooms was three (range 1-12), and echocardiography machines was four (range 1-12). Only half of all the centres have dedicated imaging physiologists and/or nursing staff, while the majority (79%) have specialist imaging cardiologist(s). The median (range) duration of time for a new examination was 45 (20-60) minutes, and for repeat examination was 20 (5-30) minutes. More than half of respondents (58%) have dedicated time for reporting. An organised training program was present in most centres (78%), 44% undertake quality assurance, and 79% perform research. Guidelines for performing echocardiography were available in 32 centres (74%). CONCLUSION:Facilities, staffing levels, study times, standards in teaching/training, and quality assurance vary widely across paediatric echocardiography laboratories in Europe. Greater support and investment to facilitate improvements in staffing levels, equipment, and governance would potentially improve European paediatric echocardiography laboratories.

Background This study explored long-term outcome and functional status of patients born with critical aortic stenosis (CAS) following neonatal surgical or catheter interventions. Methods A 40-year retrospective review of all consecutive patients within a large, single-center referral unit who required neonatal (<30 days) intervention for CAS. Additional detailed evaluation of surviving patients >7 years age was performed, with clinical assessment, objective cardiopulmonary exercise testing and state-of-the-art characterization of myocardial function (advanced echocardiography and cardiac MRI). Results Between 1970 and 2010, ninety-six neonates underwent CAS intervention (mean age 9 ± 7.5 days). Early death occurred in 19 (19.8%) and late death in 10 patients. Overall survival at 10 and 30 years was 70.1% and 68.5%, freedom from reintervention was 41.8% and 32.9% respectively.Among the 25 long-term survivors available for detailed assessment (median age 15.7 ± 6.4 years), 55% exhibited impaired peak oxygen uptake. Mean left ventricle (LV) ejection fraction was 65 ± 11.2%, with a mean LV end-diastolic volume z-score of 0.02 ± 1.4. Mean LV outflow tract Vmax was 2.3 ± 1.02 m/s. CAS patients had reduced LV longitudinal and increased radial strain (p = 0.003, p < 0.001 respectively). Five patients had severe LV diastolic dysfunction associated with endocardial fibroelastosis (EFE) (p = 0.0014). Conclusion Despite high early mortality rate, long-term survival of patients with CAS is reasonable at the expense of high reintervention rate. With successful intervention, there remained long-term clinical and subclinical LV myocardial impairment, of which EFE was one marker. Long-term follow-up of all CAS patients is crucial, involving detailed myocardial functional assessment to help elucidate physiology and optimise management.

In 2018, the position paper 'Imaging the adult with congenital heart disease: a multimodality imaging approach' was published. The paper highlights, in the first part, the different imaging modalities applied in adult congenital heart disease patients. In the second part, these modalities are discussed more detailed for moderate to complex anatomical defects. Because of the length of the paper, simple lesions were not touched on. However, imaging modalities to use for simple shunt lesions are still poorly known. One is looking for structured recommendations on which they can rely when dealing with an (undiscovered) shunt lesion. This information is lacking for the initial diagnostic process, during repair and at follow-up. Therefore, this paper will focus on atrial septal defect, ventricular septal defect, and persistent arterial duct. Pre-, intra-, and post-procedural imaging techniques will be systematically discussed. This position paper will offer algorithms that might help at a glance. The document is prepared for general cardiologists, trainees, medical students, imagers/technicians to select the most appropriate imaging modality and to detect the requested information for each specific lesion. It might serve as reference to which researchers could refer when setting up a (imaging) study.

•Evaluation of the mechanism of MR is essential for appropriate repair.•2D and 3D TEE are the gold standard for presurgical MV repair in children.•Enhanced echo imaging improves benefits in complex congenital heart disease.

We describe the management and clinical course of two children with congenital bicuspid aortic valve. Neo-tricuspidization was performed in one case using CardioCel leaflets and two cusps were formed from CardioCel and grafted alongside one native leaflet in the other. Both patients developed bacterial endocarditis associated with varicella zoster virus infection and required a second surgical procedure.

Cardiac tumors are rare in children, with an incidence ranging between 0.01% and 0.32% ([1][1]). Although most cardiac tumors are benign, some of them may lead to serious complications. Early diagnosis is crucial because treatment is dependent on the type of tumor. Because of their rarity, centers

OBJECTIVES:The treatment of aortic valve disease in children and adolescents requires an individualized approach to provide a long-term solution with optimal hemodynamic profile. The role of aortic leaflet reconstruction techniques is evolving. METHODS:We retrospectively reviewed the charts of 58 patients who underwent aortic valve tricuspidalization either by an Ozaki procedure (neo-tricuspidalization) or single leaflet reconstruction between 2015 and 2019. Immediate operative results as well as hospital and short-term outpatient follow-up data were evaluated. RESULTS:Fifty-eight patients underwent leaflet reconstruction with 40 (69%) receiving a neo-tricuspidalization and 18 patients (31%) undergoing single leaflet reconstruction, using either a glutaraldehyde fixed autologous pericardium or tissue engineered bovine pericardium (CardioCel; Admedus, Queensland, Australia). The median age at the time of surgery was 14.8 years (interquartile range, 10.6-16.8 years). Twenty-three patients (40%) had isolated aortic regurgitation. The peak velocity across the aortic valve decreased from 3.4 ± 1.2 meters per second (m/s) preoperatively to 2.0 ± 0.4 m/s (P < .001) after surgery and remained stable (2.2 ± 0.7 m/s) during a median echocardiographic follow-up of 14.1 months (7.2-20.1 months) for the whole cohort. Freedom from reoperation or moderate and greater aortic regurgitation at 1, 2, and 3 years was 94.2% ± 3.3%, 85.0% ± 5.8%, and 79.0% ± 8.0%, respectively, with no difference between the neo-tricuspidalization and single leaflet reconstruction groups (P = .635). There were 6 late reoperations (10%) of which 3 were due to endocarditis. CONCLUSIONS:Aortic leaflet reconstruction provides acceptable short-term hemodynamic outcomes and proves the utility of this technique as an adjunctive strategy for surgical treatment of aortic valve disease in children and young adults.

Once surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy. This study explored practical, easily measureable, cardiovascular magnetic resonance (CMR)-derived attributes that may contribute to the complexity and risk of cone reconstruction. A retrospective assessment was performed of Ebstein anomaly patients older than 12 years age, with pre-operative CMR, undergoing cone surgical reconstruction by one surgeon. In addition to clinical data, the CMR-derived Ebstein valve rotation angle (EVRA), area ratios of chamber size, indexed functional RV (RVEDVi) and left ventricular (LV) volumes, tricuspid valve regurgitant fraction (TR%) and other valve attributes were related to early surgical outcome; including death, significant residual TR% or breakdown of repair. Of 26 operated patients older than 12 years age, since program start, 20 had pre-op CMR and underwent surgery at median (range) age 20 (14–57) years. TR% was improved in all patients. Four of the 20 CMR patients (20%) experienced early surgical dehiscence of the paravalve tissue, with cone-shaped tricuspid valve intact; one of whom died. A larger EVRA correlated with Carpentier category and was significantly related to dehiscence. If EVRA >60o, relative risk of dehiscence was 3.2 (CI 1.3–4.9, p = 0.03). Those with dehiscence had thickened, more tethered anterior leaflet edges (RR 17, CI 3–100, p < 0.01), smaller pre-operative functional RVEDVi; (132 vs 177 mL/m2, p = 0.04), and were older (median 38 vs 19 years, p = 0.01). TR %, chamber area ratios and LV parameters were not different. Comprehensive CMR assessment characterizes patients prior to cone surgical reconstruction of Ebstein anomaly. Pragmatic observation of larger EVRA, smaller RVEDVi and leaflet thickening, suggests risk of repair tension and dehiscence, and may require specific modification of cone surgical technique, such as leaflet augmentation.

HomeCirculation: Cardiovascular ImagingVol. 12, No. 1Value of Stress Transesophageal Echocardiography in an Asymptomatic Patient With Single Coronary Artery From Noncoronary Sinus, Intramural Course, and Ostial Stenosis Free AccessCase ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessCase ReportPDF/EPUBValue of Stress Transesophageal Echocardiography in an Asymptomatic Patient With Single Coronary Artery From Noncoronary Sinus, Intramural Course, and Ostial Stenosis Beatrice Bonello, MD, Ilaria Bo, MD, Kristian Mortensen, MD, PhD, Rebecca L. Banks, BSc, Ian W. Mc Leod, BSc, Juan P Kaski, MD, Tain-Yen Hsia, MD, MSc and Jan Marek, MD, PhD Beatrice BonelloBeatrice Bonello Beatrice Bonello, MD, Great Ormond Street Hospital, Great Ormond St, London WC1N 3JH, United Kingdom. Email E-mail Address: [email protected] Great Ormond Street Hospital for Children, London, United Kingdom (B.B., I.B., K.M., R.L.B., I.W.M.L., J.P.K., J.M.). , Ilaria BoIlaria Bo Great Ormond Street Hospital for Children, London, United Kingdom (B.B., I.B., K.M., R.L.B., I.W.M.L., J.P.K., J.M.). , Kristian MortensenKristian Mortensen Great Ormond Street Hospital for Children, London, United Kingdom (B.B., I.B., K.M., R.L.B., I.W.M.L., J.P.K., J.M.). , Rebecca L. BanksRebecca L. Banks Great Ormond Street Hospital for Children, London, United Kingdom (B.B., I.B., K.M., R.L.B., I.W.M.L., J.P.K., J.M.). Institute of Cardiovascular Sciences, University College London, United Kingdom (R.L.B., J.P.K., J.M.). , Ian W. Mc LeodIan W. Mc Leod Great Ormond Street Hospital for Children, London, United Kingdom (B.B., I.B., K.M., R.L.B., I.W.M.L., J.P.K., J.M.). , Juan P KaskiJuan P Kaski Great Ormond Street Hospital for Children, London, United Kingdom (B.B., I.B., K.M., R.L.B., I.W.M.L., J.P.K., J.M.). Institute of Cardiovascular Sciences, University College London, United Kingdom (R.L.B., J.P.K., J.M.). , Tain-Yen HsiaTain-Yen Hsia Yale New Haven Children's Hospital (T.-Y.H.), New Haven, CT. Yale University (T.-Y.H.), New Haven, CT. and Jan MarekJan Marek Great Ormond Street Hospital for Children, London, United Kingdom (B.B., I.B., K.M., R.L.B., I.W.M.L., J.P.K., J.M.). Institute of Cardiovascular Sciences, University College London, United Kingdom (R.L.B., J.P.K., J.M.). Originally published11 Jan 2019https://doi.org/10.1161/CIRCIMAGING.118.008560Circulation: Cardiovascular Imaging. 2019;12Anomalous origin of the coronary arteries is a rare condition that has been associated with sudden cardiac death. The 2 most common subtypes that predispose to sudden cardiac death in the young are interarterial anomalous right coronary artery (RCA) and interarterial anomalous left coronary artery, but risk stratification is challenging.A 7-year-old boy was referred to our department for cardiac screening in the setting of a family history of catecholaminergic polymorphic ventricular tachycardia. He was completely asymptomatic other than for occasional episodes of atypical chest pain. A 12-lead ECG demonstrated normal sinus rhythm without evidence of any underlying ion channelopathy or cardiomyopathy. However comprehensive transthoracic echocardiogram incidentally revealed a single coronary artery originating from the noncoronary sinus. The ostium of the coronary artery appeared to be close to the commissure, and there was a suspicion of an intramural course of the RCA (Figure 1A and 1C, Movies I and II in the Data Supplement). Exercise testing did not reveal ventricular ectopy or bidirectional ventricular tachycardia, and no evidence of ischemia was detected.Download figureDownload PowerPointFigure 1. Single coronary artery ostium form the posterior cusp. Two-dimensional (2D) echocardiography with color flow mapping (A) and contrast-enhanced computed tomography coronary angiographic image (B) demonstrating the juxtacommissural single coronary ostium predominantly from posterior sinus. Interarterial course of the right coronary artery as seen on 2D and color flow mapping echocardiography (C). Virtual angioscopic image showing the slit-like origin of the single coronary and its proximity to the commissure (D). Ao indicates aorta; Cx, circumflex coronary artery; LAD, left anterior descending coronary artery; PA, main pulmonary artery; and RCA, right coronary artery.A cardiac computed tomography angiography was performed and confirmed the anomalous left main coronary artery originating from right posterior sinus, immediately juxtacommissural and the anomalous RCA origin from left main coronary artery with interarterial course (Figure 1B and 1D).These findings were concerning but the decision of whether or not to intervene was difficult in the absence of symptoms or evidence of ischemia and without the certainty about a true intramural course. Exercise echocardiography was attempted to detect ischemia but was not conclusive because of patient size and inability to complete the protocol.We decided to perform a pharmacological stress transesophageal echocardiography (Figure 2) under general anesthetic. At rest, we could demonstrate the juxtacommissural origin of the single coronary artery with a narrowing at its origin and thin-walled RCA with irregular color flow mapping pattern suggestive of an intramural course (Figure 2A and 2B, Movie III in the Data Supplement). Dobutamine infusion provoked a rapid increase in RCA velocity on color flow mapping and pulsed Doppler echocardiography (Figure 2C and 2D, Movie IV in the Data Supplement). Importantly, no regional wall motion abnormalities were detected.Download figureDownload PowerPointFigure 2. Transesophageal echocardiography at rest and during dobutamine infusion. Stress transesophageal echocardiography demonstrating narrow and slit-like coronary orifice and its Doppler velocity at rest (A and B) with marked increase of flow velocity during dobutamine infusion (C and D). Ao indicates aorta; and PA, main pulmonary artery.Because we were concerned by tightening of the single coronary origin, the evidence of dynamic flow acceleration in the coronary ostium during dobutamine infusion, the juxtacommissural origin, and the intramural course of the RCA, we made the decision to undertake surgery.During surgical inspection, a single orifice of the coronary system arising from the posterior aortic sinus was confirmed and there was no coronary arising from the right- or left-sided aortic sinuses. The coronary orifice led to an oblique rising vessel that was the main coronary artery. With the use of a fine probe, it was evident that the main vessel was travelling posterior to the posterior commissure, and only a few millimeters after it was giving rise to the left main coronary artery and RCA. The RCA was running intramural, within the aortic wall, facing main pulmonary artery. The RCA was coming into surface of the aortic wall, only after it has come anteriorly enough—a few millimeters before the commissure between right and left coronary sinuses.Surgery consisted of the unroofing of the total course of the RCA (Figure 3), from the branching point of the main coronary artery all the way until it came up the surface from the aortic wall. The proximal part of the unroofed RCA served as an unroofing of the left main stem orifice as well. The main pulmonary artery was translocated toward the left pulmonary artery.Download figureDownload PowerPointFigure 3. Extensive unroofing of the coronary artery. Perioperative view from above the aortic root (A) and computed tomography reconstruction (B) confirming extensive unroofing of the proximal course of the coronary artery (asterisk) next to the native ostium (arrow). Postoperative 2-dimensional (2D) echocardiography with color flow mapping after coronary unroofing (C and D) demonstrating widely patent origin of the left main coronary artery (arrow) as well as the origin of the right coronary artery (asterisk). Ao indicates aorta; Cx, circumflex coronary artery; LAD, left anterior descending coronary artery; PA, main pulmonary artery; and RCA, right coronary artery.Postoperative course was uneventful; coronary arteries were confirmed widely patent on echocardiography (Movies V and VI in the Data Supplement) and computed tomography angiography. Repeat echocardiography confirmed preserved left ventricular function with absence of regional wall motion abnormalities.DiscussionTo our knowledge, this is the first case report of single ostium coronary artery arising from the posterior noncoronary aortic sinus and first description of the value of stress transesophageal echocardiography to guide management in an extremely rare coronary anomaly. A previous case in which both coronary arteries were originating from the posterior sinus causing sudden cardiac death has been reported.1Our case is unique in terms of coronary anomaly as it was a single coronary ostium, origination from the posterior sinus and with an intramural course of the RCA. Risk stratification was challenging. The risk of sudden cardiac death associated with anomalous aortic origin and course of coronary arteries from opposite sinus has been well documented in vigorously exercising athletes and young adults, with highest risk conferred to the LCA originating from the right coronary sinus with a slit-like ostium and intramural course.2According to the most recent guidelines,2 the indications for surgery are well established for patients with interarterial anomalous LCA and symptomatic patients with interarterial anomalous RCA. However, there is some uncertainty in regards to asymptomatic patients, particularly those with an anomalous interarterial RCA, for whom a more assertive approach is required.2Stress testing is generally considered as a useful tool in the diagnostic work-up of these patients, but the number of false negative is high.3 Detection of an abnormal coronary flow pattern in the absence of segmental wall motion anomaly can help in determining whether or not to intervene.4 Stress transthoracic echocardiography has a good sensitivity and specificity for the detection of myocardial ischemia.2–4 We could not perform an exercise echocardiography because of the size of the patient, and transthoracic dobutamine echocardiography would require a general anesthesia. In our case, stress transesophageal echocardiography provided superior image quality of the coronary artery ostium, the intramural course of the RCA, as well as the dynamic changes of flow within the coronary arteries during pharmacological stress.Determining whether surgical intervention will improve the outcome is still uncertain.DisclosuresNone.FootnotesThe Data Supplement is available at https://www.ahajournals.org/doi/suppl/10.1161/CIRCIMAGING.118.008560.Beatrice Bonello, MD, Great Ormond Street Hospital, Great Ormond St, London WC1N 3JH, United Kingdom. Email [email protected]frReferences1. Catanzaro JN, Makaryus AN, Catanese C. Sudden cardiac death associated with an extremely rare coronary anomaly of the left and right coronary arteries arising exclusively from the posterior (noncoronary) sinus of valsalva.Clin Cardiol. 2005; 28:542–544.CrossrefMedlineGoogle Scholar2. Brothers JA, Frommelt MA, Jaquiss RDB, Myerburg RJ, Fraser CD, Tweddell JS. Expert consensus guidelines: anomalous aortic origin of a coronary artery.J Thorac Cardiovasc Surg. 2017; 153:1440–1457. doi: 10.1016/j.jtcvs.2016.06.066CrossrefMedlineGoogle Scholar3. Cifra B, Dragulescu A, Border WL, Mertens L. Stress echocardiography in paediatric cardiology.Eur Heart J Cardiovasc Imaging. 2015; 16:1051–1059. doi: 10.1093/ehjci/jev159CrossrefMedlineGoogle Scholar4. Osaki M, McCrindle BW, Van Arsdell G, Dipchand AI. Anomalous origin of a coronary artery from the opposite sinus of valsalva with an interarterial course: clinical profile and approach to management in the pediatric population.Pediatr Cardiol. 2008; 29:24–30. doi: 10.1007/s00246-007-9054-6CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetails January 2019Vol 12, Issue 1 Advertisement Article InformationMetrics © 2019 American Heart Association, Inc.https://doi.org/10.1161/CIRCIMAGING.118.008560PMID: 30632390 Originally publishedJanuary 11, 2019 Keywordsechocardiography, stresspediatricsuncertaintycoronary vessel anomaliesdobutaminePDF download Advertisement SubjectsComputerized Tomography (CT)EchocardiographyExercise TestingImaging

Although the high prevalence of aortic root dilatation in adults with repaired tetralogy of Fallot (rTOF) is well established [(1,2)][1], evidence to guide clinical follow-up and decision making remains sparse. We sought to define the features, determinants, and rate of progression of aortic

Advances in the diagnosis and management of congenital heart disease have led to a marked improvement in the survival of adult with congenital heart disease (ACHD) patients. However, ACHD patients are a heterogeneous population, with a large spectrum of anatomic substrates even within specific lesions. In addition, the nature of previous surgery and other intervention is highly variable rendering each patient unique and residual anatomic and haemodynamic abnormalities are very common. As the ACHD population continues to age, acquired heart disease will also require cardiac imaging assessment. It is increasingly recognized in ACHD community that the diagnostic utility of a multimodality cardiovascular approach is greater than the sum of individual tests. In ACHD patients, diagnostic information can be obtained using a variety of diagnostic tools. The aims of this document are to describe the role of each diagnostic modality in the care of ACHD patients and to provide guidelines for a multimodality approach. The goal should be to provide the most appropriate and cost-effective diagnostic pathway for each individual ACHD patient.

Received 31 May 2016; revision accepted for publication 1 August 2016. A 28-year-old woman with a history of long-standing dysphagia (since the age of 10 years) was admitted to the emergency room with respiratory distress and marked stridor in the context of upper respiratory tract infection. Chest X-ray suggested right aortic arch and trans-thoracic echocardiography revealed normal intracardiac anatomy. Computed tomography (CT) scanning revealed a double aortic arch with a prominent right aortic arch constricting the oesophagus (the right subclavian and right common carotid arteries originated from the right aortic arch; the left common carotid and subclavian arteries originated from the left aortic arch) (figure 1). The patient underwent surgery (division and section of the compressive left aortic segment) and the immediate post-operative course was uneventful. However, despite initial satisfying evolution, dysphagia for solid food persisted and the patient was referred to our centre for further investigations. The CT scan revealed oesophageal strictures at the former site of compression (“footprint” of the vascular ring). Symptomatic double aortic arch in an adult patient

OBJECTIVES:Infants with symptomatic partial and transitional atrioventricular septal defect undergoing early surgical repair are thought to be at greater risk. However, the outcome and risk profile of this cohort of patients are poorly defined. The aim of this study was to investigate the outcome of symptomatic infants undergoing early repair and to identify risk factors which may predict mortality and reoperation. METHODS:This multicentre study recruited 51 patients (24 female) in three tertiary centres between 2000 and 2015. The inclusion criteria were as follows: (1) partial and transitional atrioventricular septal defect, (2) heart failure unresponsive to treatment, (3) biventricular repair during the first year of life. RESULTS:Median age at definitive surgery was 179 (range 0-357) days. Sixteen patients (31%) had unfavourable anatomy of the left atrioventricular valve: dysplastic (n=7), double orifice (n=3), severely deficient valve leaflets (n=1), hypoplastic left atrioventricular orifice and/or mural leaflet (n=3), short/poorly defined chords (n=2). There were three inhospital deaths (5.9%) after primary repair. Eleven patients (22%) were reoperated at a median interval of 40 days (4 days to 5.1 years) for severe left atrioventricular valve regurgitation and/or stenosis. One patient required mechanical replacement of the left atrioventricular valve. After median follow-up of 3.8 years (0.1-11.4 years), all patients were in New York Heart Association (NYHA) class I. In multivariable analysis, unfavourable anatomy of the left atrioventricular valve was the only risk factor associated with left atrioventricular valve reoperation. CONCLUSIONS:Although surgical repair is successful in the majority of the cases, patients with partial and transitional atrioventricular septal defect undergoing surgical repair during infancy experience significant morbidity and mortality. The reoperation rate is high with unfavourable left atrioventricular valve anatomy.

Objective Advances in early management of congenital heart disease (CHD) have led to an exponential growth in adults with CHD (ACHD). Many of these patients require cardiac surgery. This study sought to examine outcome and its predictors for ACHD cardiac surgery. Methods This is an observational cohort study of prospectively collected data on 1090 consecutive adult patients with CHD, undergoing 1130 cardiac operations for CHD at the Royal Brompton Hospital between 2002 and 2011. Early mortality was the primary outcome measure. Midterm to longer-term survival, cumulative incidence of reoperation, other interventions and/or new-onset arrhythmia were secondary outcome measures. Predictors of early/total mortality were identified. Results Age at surgery was 35±15 years, 53% male, 52.3% were in New York Heart Association (NYHA) class I, 37.2% in class II and 10.4% in class III/IV. Early mortality was 1.77% with independent predictors NYHA class ≥ III, tricuspid annular plane systolic excursion (TAPSE) <15 mm and female gender. Over a mean follow-up of 2.8±2.6 years, 46 patients died. Baseline predictors of total mortality were NYHA class ≥ III, TAPSE <15 mm and non-elective surgery. The number of sternotomies was not independently associated with neither early nor total mortality. At 10 years, probability of survival was 94%. NYHA class among survivors was significantly improved, compared with baseline. Conclusions Contemporary cardiac surgery for ACHD performed at a single, tertiary reference centre with a multidisciplinary approach is associated with low mortality and improved functional status. Also, our findings emphasise the point that surgery should not be delayed because of reluctance to reoperate only.

Évaluer les performances de l’angioscanner non synchronisé dans la description des anomalies pulmonaires et coronaires chez les patients porteurs de tétralogie de Fallot (TOF). Étude rétrospective des patients porteurs de TOF pour lesquels un angioscanner préopératoire non synchronisé a été réalisé entre février 2007 et septembre 2012. Les paramètres cliniques étudiés ont été : l’âge moyen lors du scanner, le sexe, la présence d’une maladie génétique et le besoin de sédation pour le scanner. Les données tomodensitométriques analysées ont été : le(s) niveau(x) de la sténose pulmonaire (infundibulaire, valvulaire, artérielle), la taille des artères pulmonaires et la présence d’une anomalie de trajet des artères coronaires. Elles ont été comparées aux constatations morphologiques opératoires. Trente-cinq patients ont été inclus dans l’étude. L’âge moyen en était de 4,30 ± 1,91 mois (garçons/filles = 17/18), avec deux patients dans un contexte syndromique (1 microdélétion 22 q.11 et 1 syndrome CHARGE). Dans 16 cas, l’angioscanner a été réalisé sous sédation (45,71 %). Concernant la morphologie des artères pulmonaires, le scanner a montré que 24 patients (68,57 %) souffraient de sténose infundibulaire, 5 (17,5 %) avaient développé une sténose infundibulaire et/ou valvulaire et 6 patients (21 %) avec des anomalies des artères pulmonaires. L’angioscanner a également identifié 8 patients (22,85 %) qui présentaient des anomalies coronariennes. Par sa nature non invasive, la durée réduite de balayage et la haute résolution spatiale qui lui sont caractéristiques, l’angioscanner non synchronisé peut fournir des informations précises concernant l’anatomie des artères pulmonaires et coronaires dans les patients porteurs de TDF.