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.

Comprehensive Two-Dimensional Pediatric Echocardiographic Nomograms for Coronary Artery Sizes in Caucasian Children and Comparison among Major Nomograms.

BACKGROUND: Although coronary artery nomograms in children have been published, data on Caucasian children are lacking. The aim of this study is to provide: (i) a full dataset of coronary artery diameters in healthy children and (ii) a comparison among major previous nomograms. MATERIALS AND METHODS: We prospectively evaluated 606 healthy subjects (age range, 1 days-<18 years; median age 8.7 years; 62.5% male). Coronary artery measurements in a short-axis view were performed. Age, heart rate, and body surface area (BSA) were used as independent variables in different analyses to predict the mean values of each measurement. To assess the accuracy of the predictive models of different studies, a Z-score calculator was created using Lopez's nomograms for comparison. RESULTS: The association with BSA was found to be stronger, and was used for normalization of our data. The best-fit models, satisfying the assumption of homoscedasticity and normality of residuals and showing the highest R2 scores, were logarithmic (ln[y] = a + b*ln[x]). Predicted values and Z-score boundaries by BSA are provided. Our ranges of normality are slightly lower than those, diverging from -0.22 to -0.59 Z-scores for the left main coronary artery and from -0.23 to -0.3 Z-scores for the right coronary artery. CONCLUSIONS: We report a complete dataset of normal echocardiography coronary artery diameter (including new measures of the proximal origin) values in a large population of healthy children. Our data were statistically like those of north American nomograms.

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.

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.

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.

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.

Mid-Term Electrical Remodeling after Percutaneous Atrial Septal Defect Closure with GCO Device in a Pediatric Population.

BACKGROUND AND AIM: The GORE® CARDIOFORM (GCO) septal occluder is an atrial septal defect/patent foramen ovale closure device with theoretical advantages over other commercialized devices thanks to its softness and anatomical compliance. Our aim was to evaluate the short- and medium-term electrocardiographic changes after percutaneous ASD closure with GCO in a pediatric population. METHODS: We enrolled 39 patients with isolated ASD submitted to trans-catheter closure from January 2020 to June 2021. ECG was performed before, at 24 h and 6 months after the procedure. P wave dispersion, QTc and QTc dispersion were calculated. ECG Holter was recorded at 6 months after implantation. RESULTS: Patients' age and body surface area (BSA) were 8.2 ± 4.2 years and 1.0 ± 0.3 m2 respectively. At the baseline, mean P wave dispersion was 40 ± 15 msec and decreased at 24 h (p < 0.002), without any further change at 6 months. At 24 h, PR conduction and QTc dispersion significantly improved (p = 0.018 and p < 0.02 respectively), while the absolute QTc value considerably improved after 6 months. During mid-term follow-up, QTc dispersion remained stable without a significant change in PR conduction. The baseline cardiac frequency was 88.6 ± 12.6 bpm, followed by a slight reduction at 24 h, with a further amelioration at 6 months after the procedure (87.3 ± 14.2, p = 0.9 and 81.0 ± 12.7, p = 0.009, respectively). After device deployment, two patients developed transient, self-limited junctional rhythm. One of them needed a short course of Flecainide for atrial ectopic tachycardia. No tachy/brady-arrhythmias were recorded at the 6-month follow-up. ASD closure resulted in a marked decrease in right heart volumes and diameters at 6 months after percutaneous closure. CONCLUSIONS: Percutaneous ASD closure with the GCO device results in significant, sudden improvement of intra-atrial, atrio-ventricular and intraventricular electrical homogeneity. This benefit persists unaltered over a medium-term follow-up. These electrical changes are associated with a documented positive right heart volumetric remodeling at mid-term follow-up.

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.

3D Printed Model-Guided Neonatal Transcatheter Closure of Left Main Coronary Artery-to-Right Ventricle Fistula.

We report on a 2-week-old infant with huge left main coronary artery-to-right ventricular outflow tract fistula causing myocardial ischemia due to global coronary steal who was successfully submitted to percutaneous closure guided by a 3-dimensional-printed model using a duct-occluder vascular plug. (Level of Difficulty: Advanced.).

Transcatheter Treatment of Native Idiopathic Multiloculated Aortic Aneurysm Guided by 3D Printing Technology.

Pediatric idiopathic aortic aneurysm is rare. Single saccular malformation can complicate native or recurrent aortic coarctation; however, multiloculated dilatations of the descending thoracic aorta, associated with aortic coarctation, have so far never been described in literature. In our case, printed 3D model technology was crucial in planning transcatheter treatment. (Level of Difficulty: Intermediate.).

Uncertainty Quantification in the In Vivo Image-Based Estimation of Local Elastic Properties of Vascular Walls.

INTRODUCTION: Patient-specific computational models are a powerful tool for planning cardiovascular interventions. However, the in vivo patient-specific mechanical properties of vessels represent a major source of uncertainty. In this study, we investigated the effect of uncertainty in the elastic module (E) on a Fluid-Structure Interaction (FSI) model of a patient-specific aorta. METHODS: The image-based χ-method was used to compute the initial E value of the vascular wall. The uncertainty quantification was carried out using the generalized Polynomial Chaos (gPC) expansion technique. The stochastic analysis was based on four deterministic simulations considering four quadrature points. A deviation of about ±20% on the estimation of the E value was assumed. RESULTS: The influence of the uncertain E parameter was evaluated along the cardiac cycle on area and flow variations extracted from five cross-sections of the aortic FSI model. Results of stochastic analysis showed the impact of E in the ascending aorta while an insignificant effect was observed in the descending tract. CONCLUSIONS: This study demonstrated the importance of the image-based methodology for inferring E, highlighting the feasibility of retrieving useful additional data and enhancing the reliability of in silico models in clinical practice.

Rescue atrial septal defect closure with the new GORE® cardioform atrial septal defect occluder.

Atrioventricular block (AVB) is an infrequent but life-threatening complication of transcatheter closure of atrial septal defect (ASD), accounting for 0.1%-6.2% of cases in large series. It has been related to unfavorable defect anatomy as well as size and intrinsic stiffness of the occluding device. In this setting, the new GORE® cardioform ASD occluder (GCA) device could be an appealing technical advance in ASD treatment. We report a case of complete AVB after ASD closure with an Amplatzer septal occluding (Abbott, Plymouth MN, USA) device successfully treated by its percutaneous retrieval and "rescue" deployment of GCA device few months later.

Screening of Coronary Artery Origin by Echocardiography: Definition of Normal (and Abnormal) Take-Off by Standard Echocardiographic Views in a Healthy Pediatric Population.

Background: Echocardiographic screening of anomalous coronary artery origin is of increasing interest for children participating in sport activities. However, criteria to define normal coronary artery origins in children are poorly defined. Thus, the aim of the present investigation is to define the normal origin and angle of emergence of coronary arteries by echocardiography in healthy children. Materials and methods: The distances of the left main and right coronary artery (LMCA, RCA) origins from the aortic annulus were measured in the parasternal long-axis view (LAX). The angle of coronary artery emergence was measured in the parasternal short-axis view (SAX). Results: A total of 700 healthy subjects (mean age: 9.53 ± 5.95 years; range: 1 day−17.98 years) were prospectively enrolled. The distance of the RCA and LMCA from the aortic annulus correlated with body surface area, and nomograms (Z-scores) were generated. The RCA origin was below the sinotubular junction (STJ) in 605 patients (86.43%), at the STJ in 66 patients (9.43%), and above the STJ in 29 patients (4.14%). The LMCA origin was below the STJ in 671 patients (95.86%), at the STJ in 12 patients (1.71%), and above the STJ in 17 patients (2.43%). With respect to the RCA, an emergence angle < 18.5° in the SAX predicted a high take-off. with a sensitivity of 98.3% and a specificity of 93.1% (AUC 0.998). With respect to the LMCA, an emergence angle > 119.5° in the SAX predicted a high take-off, with a sensitivity of 70.6% and a specificity of 82.4% (AUC 0.799). Conclusion: This study establishes nomograms for LMCA and RCA origin in standard echocardiographic projections in healthy children.

Lung Ultrasound Findings in Healthy Children and in Those Who Had Recent, Not Severe COVID-19 Infection.

Background: Lung ultrasound (LUS) is gaining consensus as a non-invasive diagnostic imaging method for the evaluation of pulmonary disease in children. Aim: To clarify what type of artifacts (e.g., B-lines, pleural irregularity) can be defined normal LUS findings in children and to evaluate the differences in children who did not experience COVID-19 and in those with recent, not severe, previous COVID-19. Methods: LUS was performed according to standardized protocols. Different patterns of normality were defined: pattern 1: no plural irregularity and no B-lines; pattern 2: only mild basal posterior plural irregularity and no B-lines; pattern 3: mild posterior basal/para-spine/apical pleural irregularity and no B-lines; pattern 4: like pattern 3 plus rare B-lines; pattern 5: mild, diffuse short subpleural vertical artifacts and rare B-lines; pattern 6: mild, diffuse short subpleural vertical artifacts and limited B-lines; pattern 7: like pattern 6 plus minimal subpleural atelectasis. Coalescent B-lines, consolidations, or effusion were considered pathological. Results: Overall, 459 healthy children were prospectively recruited (mean age 10.564 ± 3.839 years). Children were divided into two groups: group 1 (n = 336), those who had not had COVID-19 infection, and group 2 (n = 123), those who experienced COVID-19 infection. Children with previous COVID-19 had higher values of LUS score than those who had not (p = 0.0002). Children with asymptomatic COVID-19 had similar LUS score as those who did not have infections (p > 0.05), while those who had symptoms showed higher LUS score than those who had not shown symptoms (p = 0.0228). Conclusions: We report the pattern of normality for LUS examination in children. We also showed that otherwise healthy children who recovered from COVID-19 and even those who were mildly symptomatic had more "physiological" artifacts at LUS examinations.

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.

Atrial Function Impairments after Pediatric Cardiac Surgery Evaluated by STE Analysis.

Background: Applications of atrial speckle tracking echocardiography (STE) strain (ε) analysis in pediatric cardiac surgery have been limited. This study aims to evaluate the feasibility of atrial STE ε analysis and the progression of atrial ε values as a function of post-operative time in children after pediatric cardiac surgery. Methods: 131 children (mean 1.69 ± 2.98; range 0.01−15.16 years) undergoing cardiac surgery were prospectively enrolled. Echocardiographic examinations were performed pre-operatively and at 3 different post-operative intervals: Time 1 (24−36 h), Time 2 (3−5 days), Time 3 (>5 days, before discharging). The right and left atrium longitudinal systolic contractile (Ct), Conduit (Cd), and Reservoir (R) ε were evaluated with a novel atrial specific software with both P- and R-Gating methods. One hundred and thirty-one age-matched normal subjects (mean 1.7 ± 3.2 years) were included as controls. Results: In all, 309 examinations were performed over the post-operative times. For each post-operative interval, all STE atrial ε parameters assessed were significantly lower compared to controls (all p < 0.0001). The lowest atrial ε values were found at Time 1, with only partial recovery thereafter (p from 0.02 to 0.04). All atrial ε values at discharge were decreased compared to the controls (all p < 0.0001). Significant correlations of the atrial ε values with cardio-pulmonary-bypass time, left and right ventricular ε values (p < 0.05), and ejection fraction (p < 0.05) were demonstrated. Conclusions: Atrial ε is highly reduced after surgery with only partial post-operative recovery in the near term. Our study additionally demonstrates that post-surgical atrial and ventricular ε responses correlated with each other.

Transcatheter Treatment of Ascending Aorta Pseudoaneurysm Guided by 3D-Model Technology.

Ascending aorta pseudoaneurysm is a rare but potentially life-threatening complication of atherosclerosis, infections, chest trauma, transcatheter or surgical interventions. Due to high surgical risk, percutaneous closure is considered a valuable cost-effective therapeutic alternative. In this setting, 3D printing technology is emerging as a powerful tool to plan transcatheter repair. (Level of Difficulty: Advanced.).

Transcatheter closure of fenestrated atrial septal aneurysm in children: Feasibility and long-term results.

BACKGROUND: To date, extensive experience in transcatheter closure of fenestrated atrial septal aneurysm (ASA) in the pediatric population is limited. METHODS: To report on procedural feasibility, efficacy, and long-term outcome, we enrolled all children submitted to an attempt of transcatheter closure of fenestrated ASA at two, large volume, pediatric cardiology units (Naples and Massa, Italy) between April 2000 to May 2020. RESULTS: This retrospective study included 139 patients (median age 9 years [range 2-18] and weight 36 kg [range 10-102]); 19 (13.7%) children were ≤20 kg (range 10-20) and 14 (10.1%) were ≤5 years old. Single perforation was observed in 28 patients (20.1%), while 111 patients (79.9%) had multifenestrated ASA. The median size of the main defect was 15 mm (range 6-34) and 25 patients (18%) had a defect ≥20 mm. The procedural success rate was 99% (95% confidence interval [CI]: 94.9-99.8) using a single device in 75 (69%), two devices in 31 (28%), and three devices in 3 (3%) cases. Early minor adverse events (AEs) occurred in four patients (2.8%). Late minor AEs were recorded in one patient (0.7%) over a median follow-up of 5 years ([range 0-18 years; total 890.2 person-years, and with 30 patients (22%) followed ≥10 years). Neither mortality nor major AEs were recorded. Freedom from AEs was 99.1% at 10-15 years (95% CI: 93.5-99.8%), without any difference according to atrial septum anatomy or patient age and weight. CONCLUSION: Transcatheter closure of fenestrated ASA is technically feasible and effective in children with excellent long-term outcomes.

Role of cardiac MRI in the prediction of pre-Fontan end-diastolic ventricular pressure.

BACKGROUND: Growing evidence has emphasised the importance of ventricular performance in functionally single-ventricle patients, particularly concerning diastolic function. Cardiac MRI has been proposed as non-invasive alternative to pre-Fontan cardiac catheterisation in selected patients. AIM OF THE STUDY: To identify clinical and cardiac magnetic resonance predictors of high pre-Fontan end-diastolic ventricular pressure. METHOD: In a retrospective single-centre study, 38 patients with functionally univentricular heart candidate for Fontan intervention, who underwent pre-Fontan cardiac catheterisation, beside a comprehensive cardiac MRI, echocardiographic, and clinical assessment were included. Medical and surgical history, cardiac magnetic resonance, cardiac catheterisation, echocardiographic, and clinical data were recorded. We investigated the association between non-invasive parameters and cardiac catheterisation pre-Fontan risk factors, in particular with end-diastolic ventricular pressure. Moreover, the impact of conventional invasive pre-Fontan risk factor on post-operative outcome as also assessed. RESULTS: Post-operative complications were associated with higher end-diastolic ventricular pressure and Mayo Clinic indexes (p < 0.01 and p = 0.05, respectively). At receiver operating characteristic curve analysis end-diastolic ventricular pressure ≥ 10.5 mmHg predicted post-operative complications with a sensitivity of 75% and specificity of 88% (AUC: 0.795, 95% CI 0.576;1.000, p < 0.05). At multivariate analysis, both systemic right ventricle (OR: 23.312, 95% CI: 2.704-200.979, p < 0.01) and superior caval vein indexed flow (OR: 0.996, 95% CI: 0.993-0.999, p < 0.05) influenced end-diastolic ventricular pressure ≥ 10.5 mmHg. CONCLUSIONS: A reduced superior caval vein flow, evaluated at cardiac magnetic resonance, is associated with higher end-diastolic ventricular pressure a predictor of early adverse outcome in post-Fontan patients.

GORE® Cardioform ASD Occluder experience in transcatheter closure of "complex" atrial septal defects.

OBJECTIVE: To evaluate the GORE® Cardioform ASD Occluder (GCO) (WL Gore & Associates, Flagstaff, AZ) device for "complex" atrial septal defects (ASD) closure. BACKGROUND: Transcatheter ASD closure is still challenging in "complex" clinical/anatomic settings. This study evaluated the results of GCO in closure of "complex" ASD in a tertiary referral center. METHODS: Between January 2020 and March 2021, 72 patients with significant ASD were submitted to transcatheter closure with GCO at our Institution. Based on clinical/anatomic characteristics, they were classified as "complex" (n = 36, Group I) or "simple" (n = 36, Group II). We considered as "complex", defects with rim deficiency (< 5 mm) other than antero-superior, relatively large (diameter/patient weight > 1.2 or diameter/patient BSA > 20 mm/m2 ) or within a multifenestrated septum. Procedure results and early outcome were compared between the groups. RESULTS: Absolute and relative ASD size (20 ± 4 vs. 15 ± 3 mm, p < 0.0001; 0.9 ± 0.3 vs. 0.4 ± 0.2 mm/kg, p < 0.0001; 23 ± 7 vs. 12 ± 5 mm/m2 , p < 0.0001), QP/QS (2.0 ± 0.8 vs. 1.4 ± 0.3, p < 0.001), procedure and fluoroscopy times (73 ± 36 vs. 43 ± 21 min, p < 0.0001; 16 ± 9 vs. 9 ± 4 min, p < 0.0001, procedure feasibility (94.4 vs. 100%, p < 0.0001) and overall complication rate (13.9 vs. 0%, p < 0.0001) were significantly different between the groups. Successful closure of "surgical" ASDs was achieved in 92% of cases. Complete closure at last follow-up evaluation did not significantly differ between the groups (97.1 vs. 100%, p = NS), as was wireframe fractures rate (49.1% in the overall population), without clinical, EKG and echocardiographic consequences. CONCLUSIONS: Percutaneous treatment with GCO device is effective and safe in high percentage of "complex" ASDs.