Mid-term outcomes after catheter ablation in patients with congenital heart disease.

INTRODUCTION: Cardiac arrhythmias are a major concern in patients with CHD. The purpose of this study was to evaluate the long-term outcomes in patients with CHD submitted to catheter ablation. MATERIALS AND METHODS: Observational retrospective study of patients with CHD referred for catheter ablation from January 2016 to December 2021 in a tertiary referral centre. Acute procedural endpoints and long-term outcomes were assessed. RESULTS: A total of 44 ablation procedures were performed in 36 CHD patients (55% male, mean age 43 ±3 years). Fifty-four arrhythmias were ablated: 23 cavotricuspid isthmus atrial flutters, 10 atrial re-entrant tachycardias, eight focal atrial tachycardias, eight atrial fibrillations, three atrioventricular re-entrant tachycardias, and two ventricular tachycardias. During a median follow-up time of 37 months (interquartile range 12-51), freedom from arrhythmia recurrence was achieved in 93%, with 1.2 procedures per patient (18% with anti-arrhythmic drugs). There were no adverse events related to catheter ablation. No predictors of recurrence were identified. CONCLUSION: In patients with CHD, catheter ablation presents a high mid-term efficacy while maintaining a safe profile.

Off-label use of a vena caval filter in the SVC in an adolescent with upper deep vein thrombosis.

Vena caval filters remain as a useful tool in patients with deep vein thrombosis and contraindications to anticoagulation. Although they are rarely used in paediatric patients, they have been shown to be safe and effective when used in the inferior vena cava.In this case report, we describe the off-label use of a retrievable vena caval filter in the superior vena cava in an adolescent with acute lymphoblastic leukaemia with extensive thrombosis of the right upper neck veins as a means to reduce the risk of pulmonary embolism.

Characterisation and long-term follow-up of children with Brugada syndrome: experience from a tertiary paediatric referral centre.

AIMS: Brugada syndrome is an inherited condition, which typically presents in young adults. It can also be diagnosed in children, but data in this group remain scarce. This study aims to describe the clinical features, management, and follow-up of children with personal or family history of Brugada syndrome. METHODS: Retrospective study of consecutive patients with Brugada history followed up in a tertiary paediatric referral centre between 2009 and 2021. Patients were assessed according to the phenotype: positive (with variable genotype) or negative (with positive genotype). RESULTS: Thirty patients were included (mean age at diagnosis 7 ± 6 years, 53% male). Within the positive phenotype (n = 16), 81% were male, and 88% had spontaneous type 1 ECG pattern. A genetic test was performed in 88% and was positive in 57%. Fourteen patients had a negative phenotype-positive genotype, 79% female, all diagnosed during family screening; 43% mentioned family history of sudden cardiac death. Although most of the patients were asymptomatic, the prevalence of rhythm/conduction disturbances was not negligible, particularly if a positive phenotype. No clinically significant events were reported in the negative phenotype patients. Three patients were hospitalised due to an arrhythmic cause, all in patients with a positive phenotype. CONCLUSION: In our study, the documentation of rhythm and conduction disturbances was not infrequent, especially in patients with a positive phenotype. Despite the significant family history, phenotype negative patients had no relevant events during follow-up. Nevertheless, the management of these patients is not clear cut, and a personalised therapeutic strategy with close follow-up is essential.

Satellite tracking reveals sex-specific migration distance in green turtles (Chelonia mydas).

Satellite tracking is a key tool for studying sea turtles in the wild. Most tracking has been performed on adult females however, leaving knowledge gaps regarding other population segments, such as adult males. By satellite tracking 12 male green turtles (Chelonia mydas) at a breeding site in West Africa, we describe their movements from the breeding to the foraging grounds and compare migrations with those of 13 females tracked in the same season. During the mating period, some males remained near the focal nesting site, while others performed exploratory movements, apparently to visit other nearby rookeries. Males migrated on average shorter distances to foraging grounds (377 km, range 50-1081, n = 9) compared to females (1038 km, range 957-1850, n = 11]). Importantly, male foraging areas overlapped with previously described areas for females, suggesting sex-specific migration distances are not derived from differences in habitat selection. Strong support for differential migration by sex in sea turtles has hitherto been found in just one other species, but indications are that it may be a general feature in this group. These findings have important implications for our understanding of the interplay between reproductive roles and movement ecology of these emblematic animals.

Cardiac tumours in children: a single-centre experience and literature review.

Cardiac tumours are extremely rare in children. Although more than 90% are benign, children can develop obstructive or embolisation derived symptoms, arrhythmias, constitutional symptoms, cardiac tamponade, or even sudden death. Although the majority are asymptomatic, and some spontaneously regress, appropriate follow-up is needed on a case-by-case basis, as patients may develop late symptoms. Definitive diagnosis is only possible through histological analysis; however, it is possible to infer tumour type with a high grade of certainty based on imaging features, particularly cardiac magnetic resonance. Surgical resection is advocated for those causing symptoms, obstruction, cardiac dysfunction, and high risk of embolisation. The aim of this review is to present the state of the art related to cardiac tumours in the paediatric population, in the context of our own experience.

Cardiovascular manifestations of Hutchinson-Gilford progeria syndrome.

Cardiovascular complications are the most frequent cause of death in patients with the Hutchinson-Gilford progeria syndrome. However, due to its rarity, studying the course of cardiac abnormalities has been a challenge. The cardiovascular phenotype helps to provide greater insight into the natural history of these abnormalities.

Simultaneous transcatheter pulmonary and tricuspid valve-in-ring implantation.

We report on a 50-year-old female patient, with several severe comorbidities and high-surgical risk, in whom we successfully performed a simultaneous transcatheter pulmonary and tricuspid valve-in-ring implantation to treat both bioprosthetic pulmonary valve dysfunction and native torrential tricuspid valve regurgitation, the latter previously managed with a Carpentier annuloplasty ring.

A rare case of pulmonary lymphangiectasia associated with CHD.

CHD may, at times, occur in the framework of other rare pathologies. These, having similar clinical manifestations, present a diagnostic dilemma for the clinician.The authors present the case of an infant with non-syndromic complete atrioventricular septal defect, whose post-operative period was surprisingly complicated by progressive pulmonary hypertension. Despite intensive care, the infant ultimately died. The diagnosis of unilateral primary pulmonary lymphangiectasia was only possible post mortem.

Repositioning an embolised stent during a percutaneous pulmonary valve implantation.

Percutaneous pulmonary valve implantation is a less invasive procedure to treat right outflow tract dysfunction related to surgical procedures such as repair of Tetralogy of Fallot. Despite the lower risks, complications have been reported, namely embolisation of the pre-stent. We report a case of a 16-year-old boy, whose procedure was complicated by embolisation of the pre-stents and the strategy used to reimplant them, prior to the successful implantation of a pulmonary valve.

Production of high-quality SARS-CoV-2 antigens: Impact of bioprocess and storage on glycosylation, biophysical attributes, and ELISA serologic tests performance.

Serological assays are valuable tools to study SARS-CoV-2 spread and, importantly, to identify individuals that were already infected and would be potentially immune to a virus reinfection. SARS-CoV-2 Spike protein and its receptor binding domain (RBD) are the antigens with higher potential to develop SARS-CoV-2 serological assays. Moreover, structural studies of these antigens are key to understand the molecular basis for Spike interaction with angiotensin converting enzyme 2 receptor, hopefully enabling the development of COVID-19 therapeutics. Thus, it is urgent that significant amounts of this protein became available at the highest quality. In this study, we produced Spike and RBD in two human derived cell hosts: HEK293-E6 and Expi293F™. We evaluated the impact of different and scalable bioprocessing approaches on Spike and RBD production yields and, more importantly, on these antigens' quality attributes. Using negative and positive sera collected from human donors, we show an excellent performance of the produced antigens, assessed in serologic enzyme-linked immunosorbent assay (ELISA) tests, as denoted by the high specificity and sensitivity of the test. We show robust Spike productions with final yields of approx. 2 mg/L of culture that were maintained independently of the production scale or cell culture strategy. To the best of our knowledge, the final yield of 90 mg/L of culture obtained for RBD production, was the highest reported to date. An in-depth characterization of SARS-CoV-2 Spike and RBD proteins was performed, namely the antigen's oligomeric state, glycosylation profiles, and thermal stability during storage. The correlation of these quality attributes with ELISA performance show equivalent reactivity to SARS-CoV-2 positive serum, for all Spike and RBD produced, and for all storage conditions tested. Overall, we provide straightforward protocols to produce high-quality SARS-CoV-2 Spike and RBD antigens, that can be easily adapted to both academic and industrial settings; and integrate, for the first time, studies on the impact of bioprocess with an in-depth characterization of these proteins, correlating antigen's glycosylation and biophysical attributes to performance of COVID-19 serologic tests.

Percutaneous management of a ruptured sinus of Valsalva aneurysm in an infant.

Ruptured sinus of Valsalva aneurysm is a rare entity in children. To our knowledge, this is the youngest child reported in the literature with a sinus of Valsalva ruptured aneurysm being successfully treated percutaneously using an AmplatzerTM Duct Occluder II Additional Sizes device.

Myocardial peak systolic velocity-a tool for cardiac screening of HIV-exposed uninfected children.

HIV-uninfected children exposed prenatally to the virus and to prophylactic antiretroviral therapy are at an uncertain risk of long-term myocardial dysfunction. This study aimed to analyse the structure and function of their ventricles and to identify potential screening tools for this at-risk population. One hundred and fifteen children (77 exposed vs 38 controls) aged between 2.7 and 16.2 years were included. An echocardiographic study was performed where both ventricles' dimensions and systolic functions were evaluated. In the left ventricle, parameters related to diastolic function were also analysed. Tissue Doppler values were determined in the basal state and after passive leg raising. Serologic analysis of amino-terminal pro-B-type natriuretic peptide (NT-proBNP) was carried out. The two groups had identical ventricular sizes and left ventricular diastolic functions. However, contractility assessed by myocardial peak systolic velocity was significantly inferior in the exposed group. These systolic echocardiographic differences were present despite similar values of NT-proBNP in both groups.Conclusion: HIV-exposed uninfected children may be vulnerable to ventricular systolic dysfunction at long term. Cardiovascular surveillance and periodic monitoring of biventricular function are therefore recommended. Myocardial peak systolic velocity may be a useful screening tool for this purpose.What is Known:• Previous studies on HIV-exposed uninfected children subjected prenatally to antiretroviral therapy have alerted to potential long-term cardiovascular toxicity effects on the left ventricle.What is New:• The study gives new insights on ventricular function and morphology in HIV-exposed uninfected children.• Myocardial peak systolic velocities are significantly inferior in this paediatric sub-group, therefore long-term cardiac surveillance is recommended.

Experience on statin therapy in paediatric age: retrospective study in a Portuguese referral centre.

BACKGROUND: The use of statins in children, although not frequent, is recommended in specific clinical contexts, namely, familial hypercholesterolaemia, conditions carrying a moderate-high cardiovascular risk and sub-optimal cholesterol levels after implementation of lifestyle modifications. The aim of this study is to characterise children with dyslipidaemia managed with statins, followed at a tertiary referral centre in central Portugal. METHODS AND RESULTS: The authors carried out a retrospective and descriptive study made up of 66 patients (50% males, mean age of therapy onset 11.9 years) followed up at the Cardiovascular Clinic of a tertiary referral centre between January, 2012, and May, 2018. Clinical, analytical, and echocardiographic parameters were analysed. About 60.6% had clinical and/or molecular diagnosis of familial hypercholesterolaemia. On average, each patient had three cardiovascular risk factors, obesity (31%) being most prevalent, followed by arterial hypertension (14%). Statin therapy showed a statistically significant reduction in the lipid profile, particularly in the total cholesterol (23%) and low-density lipoprotein cholesterol (30%) levels, as well as in the carotid intima-media thickness (p = 0.015). Hepatic and muscle integrity markers were within normal range. CONCLUSIONS: Statins are safe and efficient in the management of children with hypercholesterolaemia. Our study showed that apart from its lipid-lowering properties, it also reduced significantly the carotid intima-media thickness and, implicitly, the cardiovascular risk of these patients.

A case of double-outlet left ventricle, malposition of the great arteries, and superior-inferior ventricular arrangement.

Superior-inferior ventricular arrangement with double-outlet left ventricle and malposition of the great arteries is an extremely rare congenital cardiac anomaly. The authors present the case of an infant who presented with cyanosis and respiratory distress. To the best of our knowledge, this is the first case reported.

How to develop a sustainable telemedicine service? A Pediatric Telecardiology Service 20 years on - an exploratory study.

BACKGROUND: Telemedicine services are promoting more access to healthcare. Portugal was an early adopter of telemedicine to overcome both its geological barriers and the shortage of healthcare professionals. The Pediatric Cardiology Service (PCS) at Coimbra University Hospital Centre (CHUC) has been using telemedicine to increase access and coverage since 1998. Their Pediatric Telecardiology Service has been daily connecting CHUC with 13 other Portuguese national hospitals, and regularly connecting with Portuguese-speaking African countries, through a teleconsultation platform. METHODS: This study aims at exploring the Pediatric Telecardiology Service's evolution, through a comprehensive assessment of the PCS's development, evolution and impact in public health, to better understand the critical factors for implementation and sustainability of telemedicine, in the context of healthcare services digitalization. A case study was performed, with cost-benefit, critical factors and organizational culture assessment. Finally, the Kingdon's framework helped to understand the implementation and scale-up process and the role of policy-making. RESULTS: With the total of 32,685 out-patient teleconsultations, growing steadily from 1998 to 2016, the Pediatric Telecardiology Service has reached national and international recognition, being a pioneer and an active promotor of telemedicine. This telemedicine service has saved significant resources, about 1.1 million euros for the health system (e.g. in administrative and logistic costs) and approximately 419 euros per patient (considering an average of 1777 patients per year). PCS presents a dominant "Clan" culture. The Momentum's critical factors for telemedicine service implementation enabled us to understand how barriers were overcome (e.g. political forces). Willingness, perseverance and teamwork, allied with partnership with key stakeholders, were the foundation for professionals' engagement and service networking development. Its positive results, new regulations and the increasing support from the hospital board, set up a window of opportunity to establish a sustainable telemedicine service. CONCLUSION: The Pediatric Telecardiology Service enables real-time communication and the sharing of clinical information, overcoming many barriers (from geographical ones to shortage of healthcare professionals), improving access to specialized care both in Portugal and Africa. Motivation and teamwork, and perseverance, were key for the Pediatric Telecardiology Service to tackle the window of opportunity which created conditions for sustainability.

Management of Asymptomatic Wolff-Parkinson-White Pattern in Young Patients: Has Anything Changed?

The approach to pediatric asymptomatic Wolff-Parkinson-White (WPW) patients is controversial. The objective of this review is to update the last consensus of specialists of the Pediatric and Congenital Electrophysiology Society/Heart Rhythm Society on this subject in order to summarize the most recent evidence on the management of young patients with asymptomatic WPW pattern. A systematic review of the literature published between 2008 and 2018 was performed taking into account the protocol of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) in PubMed (including Cochrane), Embase, and Web of Science. Observational, experimental, and multicentric studies were included. Out of a total of 37 articles selected, 4 were considered eligible. Most studies considered a cutoff age of 8 or greater as recommended in the 2012 consensus. The identification of a shortest pre-excitatory RR interval (SPERRI) ≤ 250 ms seems to be the best predictor for risk stratification. The importance of routine isoprenaline use to improve the sensitivity of the electrophysiological study to identify patients at high risk of sudden death was consensual. Prophylactic ablative therapy has been indicated in asymptomatic children with an accessory pathway (AP) who have a low SPERRI and/or a low effective anterograde period of the AP and/or multiple APs. Despite the evidence found in the most recent studies, more studies are warranted in this setting.

Rare cause of cardiac failure in an infant.

Congenital stenosis of the pulmonary veins is a rare condition whose outcome is guarded despite the available treatment options. We report a case of a 6-month-old infant with significant stenosis of all four pulmonary veins.

Stylus/tablet user input device for MRI heart wall segmentation: efficiency and ease of use.

OBJECTIVES: To determine whether use of a stylus user input device (UID) would be superior to a mouse for CMR segmentation. METHODS: Twenty-five consecutive clinical cardiac magnetic resonance (CMR) examinations were selected. Image analysis was independently performed by four observers. Manual tracing of left (LV) and right (RV) ventricular endocardial contours was performed twice in 10 randomly assigned sessions, each session using only one UID. Segmentation time and the ventricular function variables were recorded. The mean segmentation time and time reduction were calculated for each method. Intraclass correlation coefficients (ICC) and Bland-Altman plots of function variables were used to assess intra- and interobserver variability and agreement between methods. Observers completed a Likert-type questionnaire. RESULTS: The mean segmentation time (in seconds) was significantly less with the stylus compared to the mouse, averaging 206±108 versus 308±125 (p<0.001) and 225±140 versus 353±162 (p<0.001) for LV and RV segmentation, respectively. The intra- and interobserver agreement rates were excellent (ICC≥0.75) regardless of the UID. There was an excellent agreement between measurements derived from manual segmentation using different UIDs (ICC≥0.75), with few exceptions. Observers preferred the stylus. CONCLUSION: The study shows a significant reduction in segmentation time using the stylus, a subjective preference, and excellent agreement between the methods. KEY POINTS: • Using a stylus for MRI ventricular segmentation is faster compared to mouse • A stylus is easier to use and results in less fatigue • There is excellent agreement between stylus and mouse UIDs.

Molecular analysis reveals hidden diversity in Zungaro (Siluriformes: Pimelodidade): a genus of giant South American catfish.

The genus Zungaro contains some of the largest catfish in South America. Two valid species are currently recognized: Zungaro jahu, inhabiting the Paraná and Paraguay basins, and Zungaro zungaro, occurring in the Amazonas and Orinoco basins. Analysing Zungaro specimens from the Amazonas, Orinoco, Paraguay and Paraná basins, based on the sequencing of COI and D-loop, we found at least three MOTUs, indicating the existence of hidden diversity within this fish group. Considering the ecological and economic values of this fish, our results are surely welcomed for its conservation, disclosing new findings on its diversity and pointing out the necessity for a detailed taxonomic revision.

Dyslipidemia and cardiovascular changes in children.

PURPOSE OF REVIEW: In this review, we firstly highlight the role of dyslipidemia as a trigger in the initiation and progression of endothelial dysfunction, considered the earliest atherosclerotic lesion and patent in children with risk factors.In this context, we also revise methods that reflect the impact of endothelial dysfunction not only on arterial stiffness but also on cardiovascular morphology, namely, the common carotid intima-media thickness and the ventricular geometry. RECENT FINDINGS: In view of its atherogenic burden, the most widely studied lipoprotein has been low density lipoprotein cholesterol. However, the smaller, denser, low density lipoprotein cholesterol particles, the nonhigh density lipoprotein cholesterol fraction, appear to be more atherogenic and a more sensitive cardiovascular risk marker. Studies have shown that in children, atherogenic lipids have also been linked to cardiovascular morphological changes, such as the common carotid intima-media thickness and the ventricular geometry, both independent cardiovascular risk markers. SUMMARY: In infancy, atherosclerosis is a preclinical disorder in which dyslipidemia plays a crucial role. Due to its impact on cardiovascular structures, potentially reversible during childhood, dyslipidemia ought to be managed aggressively to prevent further disease progression that will ultimately culminate in cardiac disease, a leading cause of mortality in adults.