Echocardiographic scores for biventricular repair risk prediction of congenital heart disease with borderline left ventricle: a review.

The aim of this review is to highlight the strengths and limitations of major echocardiographic biventricular repair (BVR) prediction models for borderline left ventricle (LV) in complex congenital heart disease (CHD). A systematic search in the National Library of Medicine for Medical Subject Headings and free text terms including echocardiography, CHD, and scores, was performed. The search was refined by adding keywords for critical aortic stenosis (AS), borderline LV, complex left ventricular outflow tract (LVOT) obstruction, hypoplastic left heart syndrome/complex (HLHS/HLHC), and unbalanced atrio-ventricular septal defects (uAVSD). Fifteen studies were selected for the final analysis. We outlined what echocardiographic scores for different types of complex CHD with diminutive LV are available. Scores for CHD with LVOT obstruction including critical AS, HLHS/HLHC, and aortic arch hypoplasia have been validated and implemented by several studies. Scores for uAVSD with right ventricle (RV) dominance have also been established and implemented, the first being the atrioventricular valve index (AVVI). In addition to AVII, both LV/RV inflow angle and LV inflow index have all been validated for the prediction of BVR. We conclude with a discussion of limitations in the development and validation of each of these scores, including retrospective design during score development, heterogeneity in echocardiographic parameters evaluated, variability in the definition of outcomes, differences in adopted surgical and Interventional strategies, and institutional differences. Furthermore, scores developed in the past two decades may have little clinical relevance now. In summary, we provide a review of echocardiographic scores for BVR in complex CHD with a diminutive LV that may serve as a guide for use in modern clinical practice.

Cardiac Troponin-T Release After Sport and Differences by Age, Sex, Training Type, Volume, and Intensity: A Critical Review.

BACKGROUND: Postexercise release of cardiac troponin (cTn) is a well-known phenomenon, although the influence of various confounders remains unclear. The aim of this critical review was to analyze the postexercise release of cTn according to age, sex, different types of sport, exercise intensity and duration, and training level. DATA SOURCES: A literature search was performed within the National Library of Medicine using the following keywords: cTn, peak, release, and exercise. The search was further refined by adding the keywords athletes, children/adolescents, and sport. MAIN RESULTS: For final analysis, 52 studies were included: 43 adult studies, 4 pediatric studies, and 5 with a mixed population of adults and children. Several studies have investigated the kinetics of cTn response after exercise with different biomarkers. The current evidence suggests that sport intensity and duration have significant effects on postexercise cTn elevation, whereas the influence of the type of sport, age, and sex have been not completely defined yet. Most data were obtained during endurance races, whereas evidence is limited (or almost absent), particularly for mixed sports. Data on young adults and professional athletes are limited. Finally, studies on women are extremely limited, and those for non-White are absent. CONCLUSIONS: Postexercise release of cTn can be observed both in young and master athletes and usually represents a physiological phenomenon; however, more rarely, it may unmask a subclinical cardiac disease. The influence of different confounders (age, sex, sport type/intensity/duration, and training level) should be better clarified to establish individualized ranges of normality for postexercise cTn elevation.

Transcatheter closure of "Surgical" ostium secundum atrial septal defects with GORE® Cardioform ASD Occluder.

OBJECTIVE: To evaluate the GORE® Cardioform ASD Occluder (GCA)(WL Gore & Associates) device for closure of ostium secundum atrial septal defects (ASDs) with predicted indication for surgical correction. BACKGROUND: Closure of large ASD in small children by transcatheter approach is still challenging. This study evaluated the results of GCA in this subset of patients in a tertiary referral center. METHODS: Between January 2020 and March 2022, 97 children underwent transcatheter ASD closure at our Institution. Of them, 38 had a large defect (diameter/weight > 1.2 or diameter/body surface area > 20 mm/m2 ), predicted suitable for surgery and underwent closure with GCA. Procedure results and midterm outcome are reported. RESULTS: Patients' age and weight were 5.5 ± 1.5 years and 19.7 ± 4.7 kg, respectively. Absolute and relative ASD size was 21.5 ± 3.6 mm, 1.1 ± 0.2 mm/kg, and 27.7 ± 4.6 mm/m2 , respectively, resulting in QP/QS of 2.0 ± 0.8. Three patients were sent to surgery after balloon sizing. Four of the remaining 35 patients who underwent device deployment, needed rescue or elective surgery due to device embolization (n = 1), device instability (n = 2) or new-onset tricuspid valve regurgitation (n = 1). Procedure feasibility was 88.6%. Major complications were recorded in two patients (5.7%). Minor complications were recorded in five patients (14.3%). Complete closure at discharge was 90.3% (28/31 pts) rising to 100% at the last follow-up evaluation. Wireframe fracture rate at the 6 months examination was 52%, without clinical and instrumental consequences. CONCLUSIONS: Percutaneous treatment with GCA device is effective and safe in a high percentage of ASD children with predicted indications for surgical correction.

Fungi populate deep-sea coral gardens as well as marine sediments in the Irish Atlantic Ocean.

Fungi populate deep Oceans in extreme habitats characterized by high hydrostatic pressure, low temperature and absence of sunlight. Marine fungi are potential major contributors to biogeochemical events, critical for marine communities and food web equilibrium under climate change conditions and a valuable source of novel extremozymes and small molecules. Despite their ecophysiological and biotechnological relevance, fungal deep-sea biodiversity has not yet been thoroughly characterized. In this study, we describe the culturable mycobiota associated with the deepest margin of the European Western Continental Shelf: sediments sampled at the Porcupine Bank and deep-water corals and sponges sampled in the Whittard Canyon. Eighty-seven strains were isolated, belonging to 43 taxa and mainly Ascomycota. Ten species and four genera were detected for the first time in the marine environment and a possible new species of Arachnomyces was isolated from sediments. The genera Cladosporium and Penicillium were the most frequent and detected on both substrates, followed by Candida and Emericellopsis. Our results showed two different fungal communities: sediment-associated taxa which were predominantly saprotrophic and animal-associated taxa which were predominantly symbiotic. This survey supports selective fungal biodiversity in the deep North Atlantic, encouraging further mycological studies on cold water coral gardens, often overexploited marine habitats.

A statistical comparison of reproducibility in current pediatric two-dimensional echocardiographic nomograms.

BACKGROUND: The aim of this study is to compare new pediatric nomograms for clinical parameters from 2D echocardiography. METHODS: 2D pediatric echocardiographic parameters from four recent nomograms were used for statistical analysis. To assess the accuracy of the predictive models from each study, namely multivariate, linear, and nonlinear regression, mean values and 5th and 95th percentiles (µ ± 1.65σ) were calculated. A Z-score calculator was created. RESULTS: Mean values and 5th and 95th percentiles have been provided for a range of BSA (0.15-2.20 m2) for each nomogram assessed in this study. Moreover, plots of Z-scores over the same range of BSA have been generated to assess trends among different studies. For most measurements from the two most recent nomograms, namely Lopez et al. and Cantinotti et al., differences were within a Z-score of 0.5 (Z-score range: 0.001-1.26). Measurements from Sluysmans and Colan and Pettersen et al. were observed to diverge from Lopez et al. at the upper extremities of BSA. Differences among various nomograms emerged at lower extremes of BSA. CONCLUSIONS: The two most recent echocardiographic nomograms were observed to have the most statistically similar ranges of normality. Significant deviations in ranges of normality were observed at extremes of BSA. IMPACT: Echocardiographic nomograms for pediatric age are discordant. Comparison of current pediatric echocardiographic nomograms. A Z-score calculator was created. Clinical relevance of differences among nomograms is highlighted.

Intracardiac flow visualization using high-frame rate blood speckle tracking echocardiography: Illustrations from infants with congenital heart disease.

We report applications of novel high-frame rate blood speckle tracking (BST) echocardiography in a series of infants with congenital heart disease (CHD). BST echocardiography was highly feasible, reproducible, and fast. High-frame rate BST provided complimentary information to conventional color-Doppler data enhancing the visualization and understanding of anomalous blood trajectories (eg, shunt direction, regurgitant volumes, and stenotic jets) and vortex formation. High-frame rate BST echocardiography is a new, promising imaging tool that may be helpful for deeper understanding of complex CHD physiology.

Pediatric ranges of normality for 2D speckle-tracking echocardiography atrial strain: differences between p- and r-gating and among new (Atrial Designed) and conventional (Ventricular Specific) software's.

BACKGROUND: 2D speckle tracking echocardiography (STE) atrial strain (ε) analysis in children is gaining interest; however, pediatric nomograms remain limited. Comparison among conventional software's (designed for left ventricle and adapted to atria and using R-gating analysis) and new software's (designed for atria and allowing for both R- and P-gating) are lacking. The present study aims to establish pediatric nomograms for atrial ε using an atrial dedicated software and to compare values obtained by (a) R- and P-gating and, (b) R-gating with new and conventional software. METHODS: Echocardiographic measurements included STE left (LA) and right (RA) atrial longitudinal reservoir, conduit and contractile ε. Age/weigh/height/heart rate, and body surface area (BSA) were used as independent variables in the statistical analysis. RESULTS: In all, 580 healthy subjects (age range, 31 days-18 years; mean age 7.5 years; median age 7.1 years; inter-quartile range, 4.3-10.7 months; 45.5% female) were included. Feasibility of atrial ε measurements was high (98.9-96.8%). At lower age, atrial conduit ε was lower (p<0.001) while contractile ε was higher (p<0.001). All atrial ε values calculated with P-gating method were lower-than R-gating values (p<0.001). R-gated LA ε reservoir values generated with the new software were lower, and R-gated RA contractile ε higher than with the conventional software (p<0.011). CONCLUSION: We report pediatric atrial ε values from a dedicated atrial software. Maturational changes in STE atrial ε values were demonstrated. Significant differences were observed among ε values obtained with P- and R-gating and with different software's.

Deep-Sea Coral Garden Invertebrates and Their Associated Fungi Are Genetic Resources for Chronic Disease Drug Discovery.

Chronic diseases characterized by bone and cartilage loss are associated with a reduced ability of progenitor cells to regenerate new tissues in an inflammatory environment. A promising strategy to treat such diseases is based on tissue repair mediated by human mesenchymal stem cells (hMSCs), but therapeutic outcomes are hindered by the absence of small molecules to efficiently modulate cell behaviour. Here, we applied a high-throughput drug screening technology to bioprospect a large library of extracts from Irish deep-sea organisms to induce hMSC differentiation toward musculoskeletal lineages and reduce inflammation of activated macrophages. The library included extracts from deep-sea corals, sponges and filamentous fungi representing a novel source of compounds for the targeted bioactivity. A validated hit rate of 3.4% was recorded from the invertebrate library, with cold water sea pens (octocoral order Pennatulacea), such as Kophobelemnon sp. and Anthoptilum sp., showing the most promising results in influencing stem cell differentiation toward osteogenic and chondrogenic lineages. Extracts obtained from deep-sea fungi showed no effects on stem cell differentiation, but a 6.8% hit rate in reducing the inflammation of activated macrophages. Our results demonstrate the potential of deep-sea organisms to synthetize pro-differentiation and immunomodulatory compounds that may represent potential drug development candidates to treat chronic musculoskeletal diseases.

Pediatric nomograms for left ventricle biplane 2D volumes in healthy Caucasian children.

BACKGROUND: Left ventricular (LV) volumes are basic parameters used to estimate ventricular size and function; however, normal values are not available in children. The aim of our study is to provide normal values for LV volumes (measured with the biplane Simpson method) in healthy children. MATERIALS AND METHODS: We prospectively studied 1320 healthy Caucasian Italian children (age 0 days-17 years, 49.4% female). Echocardiographic measurements on LV volumes were performed. Age, heart rate (HR), and body surface area (BSA) were used as independent variables in different analyses to predict the mean values of each measurement. RESULTS: Models with exponential (ln[y] = a + b*ln[x]) equations resulted in the best fit for LV volumes. The association with BSA was found to be stronger than the association of HR and age. Thus BSA was used for normalization of our data. Predicted values and Z-score boundaries by BSA are provided. CONCLUSIONS: We report normal values for 2D biplane LV volumes in a population of healthy children. These data cover a gap in current pediatric echocardiographic nomograms and may serve as baseline for evaluation of children with cardiac defects characterized by LV dilatation or hypoplasia.

Retrosternal Clots After Fontan Surgery by Systematic Evaluation With Transthoracic Ultrasound.

OBJECTIVES: Bleeding is a common, serious, and often subtle complication after total cavopulmonary connection surgery. The aim of the present study was to assess the incidence of retrosternal clots after surgery, which were searched for systematically with transthoracic ultrasound. DESIGN: Retrospective study. SETTING: Single center. PARTICIPANTS: Total cavopulmonary surgeries were reviewed from January 2016 to May 2019. INTERVENTIONS: Thoracic ultrasound with careful evaluation of the retrosternal area was performed at different postoperative times (12-36 hours, 5-7 days, and before discharge) as completion of routine echocardiography. MEASUREMENTS AND MAIN RESULTS: Among 37 children undergoing total cavopulmonary connection (mean age 5.5 ± 1.8 years [range 2.4-11.7]; mean body surface area 0.7 ± 0.1 m2 [range 0.3-1.6 m2]), retrosternal clots were detected in 18 (48.6%). Of these, 7 (13.5%) had small clots (<1 cm), 2 (5.4%) small to moderate sized clots (>1 cm-<2 cm), 3 (8.1%) moderate sized clots (>2-<3 cm), and 6 (16.2%) large clots (>3 cm). Four of the 6 detected large clots required surgical revision, and in the other 2 patients, the clots were not treated because the patients' conditions were clinically stable. When 3 major groups (group 1-no or small clots, group gropu 2 are small to moderate or moderate, group 3-large clots) were evaluated, no significant differences were noted in age, body surface area, CPB time, conduit type, or the number of previous surgeries. CONCLUSIONS: With thoracic ultrasound diagnosis, existence of retrosternal clots was found to be very common after total cavopulmonary connection. Most clots were small or moderate with no clinical effect; however, large clots that required redo surgery also were detected.

A Novel High-Throughput Screening Platform Identifies Itaconate Derivatives from Marine Penicillium antarcticum as Inhibitors of Mesenchymal Stem Cell Differentiation.

Worldwide diffused diseases such as osteoarthritis, atherosclerosis or chronic kidney disease are associated with a tissue calcification process which may involve unexpected local stem cell differentiation. Current pharmacological treatments for such musculoskeletal conditions are weakly effective, sometimes extremely expensive and often absent. The potential to develop new therapies is represented by the discovery of small molecules modulating resident progenitor cell differentiation to prevent aberrant tissue calcification. The marine environment is a rich reserve of compounds with pharmaceutical potential and many novel molecules are isolated from macro and microorganisms annually. The potential of small molecules synthetized by marine filamentous fungi to influence the osteogenic and chondrogenic differentiation of human mesenchymal stem/stromal cells (hMSCs) was investigated using a novel, high-throughput automated screening platform. Metabolites synthetized by the marine-derived fungus Penicillium antarcticum were evaluated on the platform. Itaconic acid derivatives were identified as inhibitors of calcium elaboration into the matrix of osteogenically differentiated hMSCs and also inhibited hMSC chondrogenic differentiation, highlighting their capacity to impair ectopic calcification. Bioactive small molecule discovery is critical to address ectopic tissue calcification and the use of biologically relevant assays to identify naturally occurring metabolites from marine sources represents a strategy that can contribute to this effort.

Nomograms for Cardiovascular Magnetic Resonance Measurements in the Pediatric Age Group: To Define the Normal and the Expected Abnormal Values in Corrected/Palliated Congenital Heart Disease: A Systematic Review.

Our purpose is to provide an overview and to systematically review the strengths and limitations of studies on pediatric and adolescent normal values for cardiovascular MRI parameters. A literature search was performed within the National Library of Medicine using the following keywords: normal, reference values, cardiovascular magnetic resonance imaging, and children/pediatric. Eleven published studies evaluating cardiovascular MRI measurements in normal children were included in the present analysis. Our results revealed reasonable consistencies in the protocols employed for cardiovascular MRI. Inter- and intraobserver variability analyses were performed in most studies and generally showed acceptable reproducibility. However, several numerical and methodological limitations emerged. Besides small sample sizes (the largest study enrolled 114 subjects), data for some structures (pulmonary arteries, aortic arch) were limited, and neonates/infants were poorly represented (eg, only two studies). There was heterogeneity regarding measurement normalization (eg, for gender, age, or both), and data were mostly expressed as mean values, while z-scores (commonly used in pediatric echocardiography) were rarely employed. Theoretically, a z-score or a standard deviation of ±2 is considered pathological. Furthermore, differences among races and ethnic groups were not evaluated. In conclusion, our analyses revealed an important need for generation of pediatric and adolescent cardiovascular MRI nomograms built over a wide population of healthy children, using consistent methodologies and with consideration of potentially relevant confounders. More data on expected abnormal values in specific CHD populations (eg, univentricular hearts) also need to be defined. Level of Evidence: 2 Technical Efficacy Stage: 3 J. Magn. Reson. Imaging 2019;49:1222-1235.

Four-Dimensional Flow Echocardiography: Blood Speckle Tracking in Congenital Heart Disease: How to Apply, How to Interpret, What Is Feasible, and What Is Missing Still.

Blood speckle tracking echocardiography (BSTE) is a new, promising 4D flow ultrafast non-focal plane imaging technique. The aim of the present investigation is to provide a review and update on potentialities and application of BSTE in children with congenital heart disease (CHD) and acquired heart disease. A literature search was performed within the National Library of Medicine using the keywords "echocardiography", "BST", and "children". The search was refined by adding the keywords "ultrafast imaging", "CHD", and "4D flow". Fifteen studies were finally included. Our analysis outlined how BSTE is highly feasible, fast, and easy for visualization of normal/abnormal flow patterns in healthy children and in those with CHD. BSTE allows for visualization and basic 2D measures of normal/abnormal vortices forming the ventricles and in the main vessel. Left ventricular vortex characteristics and aortic flow patterns have been described both in healthy children and in those with CHD. Complex analysis (e.g., energy loss, vorticity, and vector complexity) are also highly feasible with BSTE, but software is currently available only for research. Furthermore, current technology allows for BSTE only in neonates and low-weight children (e.g., <40 kg). In summary, the feasibility and potentialities of BSTE as a complementary diagnostic tool in children have been proved; however, its systemic use is hampered by the lack of (i) accessible tools for complex quantification and for acquisition at all ages/weight, (ii) data on the diagnostic/prognostic significance of BSTE, and (iii) consensus/recommendation papers indicating when and how BSTE should be employed.

Personalized Approach in Transcatheter Palliation of Congenital Heart Disease with Duct-Dependent Pulmonary Circulation: Right Ventricular Outflow Tract Stenting vs. Arterial Duct Stenting.

Despite significant improvements in techniques, the treatment of neonates and infants with congenital heart disease resulting in duct-dependent pulmonary circulation is still significantly challenging. Despite current trends toward early primary surgical repair, temporary palliation is still necessary in those patients who are at high surgical risk for complete correction due to unfavorable clinical or anatomic characteristics. Recent advances in interventional cardiology have led to the emergence of right ventricular outflow tract and arterial duct stenting as cost-effective alternatives to surgical palliation in high-risk surgical candidates or whenever short-term pulmonary blood flow support is anticipated. This review aims to explore the evolving landscape of these transcatheter approaches, highlighting their role, efficacy and potential complications in the context of duct-dependent pulmonary circulation anatomies.

Impact of Gore Cardioform Atrial Septal Defect Occluder on Atrial and Ventricular Electromechanics in a Pediatric Population.

Transcatheter closure is the first-line treatment for ostium secundum atrial septal defect (ASD). The GORE Cardioform ASD Occluder (GCA) is potentially innovative compared with other self-centering devices. This study aimed to compare the mechanic changes in atrial and ventricular properties before and after GCA implantation. All consecutive patients aged <18 years who underwent isolated ASD closure with a single GCA device were enrolled from 2 centers. Echocardiography and electrocardiogram were performed the day before, 24 hours, and 6 months after ASD closure. Between January 2020 and February 2021, 70 pediatric patients with ASD were enrolled. The mean age was 7.9 ± 3.9 years, and the mean defect diameter was 17.1 ± 4.5 mm. Global longitudinal strain analysis showed no change in left ventricular longitudinal function (T0 -23.2 ± 2.8%, 24 hours -23.0 ± 2.8%, and 6 months -23.5 ± 2.7%). An early and transient reduction in longitudinal strain was detected in the basal septal segments (T0 -19.8 ± 3.3%, 24 hours -18.7 ± 3.6%, and 6 months -19.2 ± 3.4%), left atrium (T0 41.4 ± 15.3%, 29.2 ± 1.4%, and 39.0 ± 12.9%), and right ventricle (-27.6 ± 5.4%, -23.6 ± 5.0%, and -27.3 ± 4.6) 24 hours after closure, secondary to hemodynamic changes because of flow redirection after ASD closure. Six months after the procedure, only the left atrium showed a mild global longitudinal strain reduction because of the presence of the device within the septum. GCA device had no impact on global and regional ventricular function. Atrial mechanics were preserved, except for the segments covered by the device. This is the first device demonstrating no impact on the left and right ventricular mechanics, irrespective of the device size.

Normal Values for Echocardiographic Myocardial Work in a Large Pediatric Population.

BACKGROUND: Echocardiographic myocardial work is a new load-independent echocardiographic technique to quantify left ventricle (LV) systolic performance. Our aim was to establish normal values for echocardiographic myocardial work in a large population of healthy children. METHODS: For all the subjects 4-, 2-, and 3-chamber-view videos were stored. The following parameters were obtained by offline analysis: the global myocardial work (GMW), the global myocardial constructive work (GCW), the global myocardial wasted work (GWW), and the global myocardial work efficiency (GWE). Age, weight, height, heart rate, and body surface area (BSA) were used as independent variables in the statistical analysis. RESULTS: In all, 516 healthy subjects (age range, 1 day-18 years; median age, 8.2 ± 5.3 years; 55.8% male; body surface area (BSA) range, 0.16 to 2.12 m2) were included. GWI, GCW, and GWW increased with weight, height, and BSA (ρ ranging from 0.635 to 0.226, p all < 0.01); GWI and GCW positively correlated with age (ρ 0.653 and 0.507). After adjusting for BSA differences, females showed higher mean GWI (p = 0.002) and GCW values (p < 0.001), thus Z-score equations for gender have been presented. CONCLUSIONS: We provided MW values in a large population of healthy pediatric subjects including lower ages. MW values increased with age and body size and, interestingly, were higher in females than in men. These data cover a gap in current nomograms and may serve as a baseline for the evaluation of MW analysis in children with congenital and acquired heart diseases.

Neonates and Infants with Left Heart Obstruction and Borderline Left Ventricle Undergoing Biventricular Repair: What Do We Know about Long-Term Outcomes? A Critical Review.

BACKGROUND: The decision to perform biventricular repair (BVR) in neonates and infants presenting with either single or multiple left ventricle outflow obstructions (LVOTOs) and a borderline left ventricle (BLV) is subject to extensive discussion, and limited information is known regarding the long-term outcomes. As a result, the objective of this study is to critically assess and summarize the available data regarding the prognosis of neonates and infants with LVOTO and BLV who underwent BVR. METHODS: In February 2023, we conducted a review study with three different medical search engines (the National Library of Medicine, Science Direct, and Cochrane Library) for Medical Subject Headings and free text terms including "congenital heart disease", "outcome", and "borderline left ventricle". The search was refined by adding keywords for "Shone's complex", "complex LVOT obstruction", "hypoplastic left heart syndrome/complex", and "critical aortic stenosis". RESULTS: Out of a total of 51 studies, 15 studies were included in the final analysis. The authors utilized heterogeneous definitions to characterize BLV, resulting in considerable variation in inclusion criteria among studies. Three distinct categories of studies were identified, encompassing those specifically designed to evaluate BLV, those focused on Shone's complex, and finally those on aortic stenosis. Despite the challenges associated with comparing data originating from slightly different cardiac defects and from different eras, our results indicate a favorable survival rate and clinical outcome following BVR. However, the incidence of reintervention remains high, and concerns persist regarding residual pulmonary hypertension, which has been inadequately investigated. CONCLUSIONS: The available data concerning neonates and infants with LVOTO and BLV who undergo BVR are inadequate and fragmented. Consequently, large-scale studies are necessary to fully ascertain the long-term outcome of these complex defects.

Percutaneous closure of 'complex' multi-fenestrated atrial septal aneurysm in visceral situs inversus using a multi-device approach: a case report.

Background: Percutaneous closure is nowadays still deemed challenging in patent forame ovale (PFO) associated to multi-fenestrated atrial septal aneurysm (ASA). This anatomic arrangement is still considered a significant risk factor for recurrence of paradoxical embolism. Theoretically, transcatheter approach could be theoretically even more complex in the case of dextrocardia and visceral situs inversus. Case summary: A 59-year-old man with history of migraine with aura and multiple cryptogenic strokes was referred for percutaneous closure of a PFO with associated ASA. He had been previously submitted to repeat attempt of percutaneous closure with not self-centering and self-centering devices that failed due to unfavorable anatomic characteristics (dextrocardia with situs viscerum inversus, huge ASA, multiple fenestrations, large PFO). Based on this "complex" anatomy, a sequential 2-step interventional approach aiming to reduce size and mobility of the atrial septal aneurysm with a suture-based approach (Noblestich™ EL, HeartStitch, Fountain Valley, CA, USA) and to close any eventual accessory fenestrations with a not self-centering occluding device was planned. At the end of the procedure, the ASA completely disappeared and no residual shunt was imaged at TEE bubble test. Discussion: We describe a very rare case of symptomatic ASA-PFO in dextrocardia with situs viscerum inversus as well as an innovative approach to treat such complex anatomic setting by using the suture-based closure of a PFO to reduce size and mobility of an ASA in order to deploy dedicated not-self-centering occluding devices.

Native Aortic Root Thrombosis in Hypoplastic Left Heart Syndrome: An Unusual Presentation (Soon after Atrial Septal Stenting) of a Relatively Unusual Complication-Experience and Literature Review with an Outlook to Diagnosis and Management.

We started with the experience of thrombus formation in the native aorta of a 3-year-old male child with hypoplastic left heart syndrome (HLHS) and severely hypoplastic but patent mitral and aortic valves after Glenn palliation, which occurred soon after left heart decompression by percutaneous stenting of the atrial septum. The diagnosis was incidental, with the child completely asymptomatic, and progressively subsided in a few days with heparin infusion and chronic warfarin therapy. We reviewed the incidence, diagnosis, and management of native aortic thrombosis in HLHS after different stages of Fontan palliation through a systematic literature search. In all 32 cases, native aortic thrombosis in HLHS was found. The HLHS anatomic subtypes included mitral stenosis/aortic stenosis (fourteen cases or 45.2%), mitral stenosis/aortic atresia (eleven cases or 35.5%), and mitral atresia/aortic atresia (four cases or 12.9%). The age at diagnosis ranged from 13 days to 18 years. Clinical presentation varied from incidental findings, chest pain and/or electrocardiographic abnormalities, cardiac arrest, and transient ischemic attack. Diagnosis was feasible in most of the cases with only transthoracic echocardiography. Mostly (59.4%), patients were treated with anticoagulation, while others underwent surgical (18.7%), direct (12.5%), or systemic (9.3%) thrombolysis. Transplant-free survival was 56.2%, and fatal events occurred in 25%. Major events occurred in 26.3% of those treated with anticoagulation, in 33.3% of patients treated with surgical/systemic thrombolysis, and in 100% of patients treated with direct thrombolysis. In summary, native aortic thrombosis in HLHS may occur at different ages, with a wide spectrum of presentation from incidental finding to a sudden major event. Diagnosis is feasible with transthoracic echocardiography, and management with anticoagulation is effective despite the incidence of major events remaining high.

Echocardiographic Parameters for Risk Prediction in Borderline Right Ventricle: Review with Special Emphasis on Pulmonary Atresia with Intact Ventricular Septum and Critical Pulmonary Stenosis.

The aim of the present review is to highlight the strengths and limitations of echocardiographic parameters and scores employed to predict favorable outcome in complex congenital heart diseases (CHDs) with borderline right ventricle (RV), with a focus on pulmonary atresia with intact ventricular septum and critical pulmonary stenosis (PAIVS/CPS). A systematic search in the National Library of Medicine using Medical Subject Headings and free-text terms including echocardiography, CHD, and scores, was performed. The search was refined by adding keywords "PAIVS/CPS", Ebstein's anomaly, and unbalanced atrioventricular septal defect with left dominance. A total of 22 studies were selected for final analysis; 12 of them were focused on parameters to predict biventricular repair (BVR)/pulmonary blood flow augmentation in PAIVS/CPS. All of these studies presented numerical (the limited sample size) and methodological limitations (retrospective design, poor definition of inclusion/exclusion criteria, variability in the definition of outcomes, differences in adopted surgical and interventional strategies). There was heterogeneity in the echocardiographic parameters employed and cut-off values proposed, with difficultly in establishing which one should be recommended. Easy scores such as TV/MV (tricuspid/mitral valve) and RV/LV (right/left ventricle) ratios were proven to have a good prognostic accuracy; however, the data were very limited (only two studies with <40 subjects). In larger studies, RV end-diastolic area and a higher degree of tricuspid regurgitation were also proven as accurate predictors of successful BVR. These measures, however, may be either operator and/or load/pressure dependent. TV Z-scores have been proposed by several authors, but old and heterogenous nomograms sources have been employed, thus producing discordant results. In summary, we provide a review of the currently available echocardiographic parameters for risk prediction in CHDs with a diminutive RV that may serve as a guide for use in clinical practice.