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.

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.

Overview of Lung Ultrasound in Pediatric Cardiology.

Lung ultrasound (LUS) is increasing in its popularity for the diagnosis of pulmonary complications in acute pediatric care settings. Despite the high incidence of pulmonary complications for patients with pediatric cardiovascular and congenital heart disease, especially in children undergoing cardiac surgery, the use of LUS remains quite limited in these patients. The aim of this review is to provide a comprehensive overview and list of current potential applications for LUS in children with congenital heart disease, post-surgery. We herein describe protocols for LUS examinations in children, discuss diagnostic criteria, and introduce methods for the diagnosis and classification of pulmonary disease commonly encountered in pediatric cardiology (e.g., pleural effusion, atelectasis, interstitial edema, pneumothorax, pneumonia, and diaphragmatic motion analysis). Furthermore, applications of chest ultrasounds for the evaluation of the retrosternal area, and in particular, systematic search criteria for retrosternal clots, are illustrated. We also discussed the potential applications of LUS, including the guidance of interventional procedures, namely lung recruitment and drainage insertion. Lastly, we analyzed current gaps in knowledge, including the difficulty of the quantification of pleural effusion and atelectasis, and the need to differentiate different etiologies of B-lines. We concluded with future applications of LUS, including strain analysis and advanced analysis of diaphragmatic mechanics. In summary, US is an easy, accurate, fast, cheap, and radiation-free tool for the diagnosis and follow-up of major pulmonary complications in pediatric cardiac surgery, and we strongly encourage its use in routine practice.

Left Ventricular Systolic Impairment after Pediatric Cardiac Surgery Assessed by STE Analysis.

BACKGROUND: Speckle-tracking echocardiography (STE) has gained increasing value in the evaluation of congenital heart diseases (CHD); however, its use in pediatric cardiac surgery is limited. AIM: To evaluate left ventricular (LV) systolic impairment after biventricular pediatric cardiac surgery by STE strain (ε) analysis. METHODS: We prospectively enrolled 117 children undergoing cardiac surgery for CHD. Echocardiography was performed at four different times: pre-operatively, 12-36 h (Time 1), 3-5 days (Time 2), and 6-8 days (Time 3). Images were obtained in the 4-2-and 3 apical chamber's views to derive LV global and regional (basal/mid/apical) ε values. RESULTS: At different postoperative times, we performed 320 examinations in 117 children (mean age: 2.4 ± 3.9, range: 0-16 years); 117 age-matched healthy children served as controls. All global, basal, and mid LVε values decreased after surgery; the lowest values being at Time 1 (p < 0.0001), which increased thereafter. At discharge, all global, basal, and mid LVε values remained lower than in pre-operative and healthy children (p < 0.05). Instead, apical segments (lowest at baseline) increased after surgery (p < 0.0001) but remained lower compared to controls. LV ejection fraction (LVEF) decreased at Time 1 (p = 0.0004) but promptly recovered to Time 2 and normalized at Time 3. CONCLUSIONS: STE ε analysis revealed a significant LV systolic impairment after surgery with amelioration thereafter but incomplete normalization at discharge. Base-apex differences emerged with apical segments that, contrary to all the other regions, showed relative hypercontractility after surgery. The slower recovery of LVε values compared to LVEF suggests that STE ε analysis may be more accurate for the follow-up of mild LV post-surgical impairment.

Transcatheter Treatment of "Complex" Aortic Coarctation Guided by Printed 3D Model.

The transcatheter approach is nowadays considered a cost-effective alternative to surgery in adults with "complex" aortic coarctation. The printed 3D model was crucial in planning transcatheter treatment of a complex case of postsurgical aortic re-coarctation, due to coexistence of transverse aortic arch stenosis and pseudoaneurysm as well as aneurysm of the descending aorta. (Level of Difficulty: Advanced.).

Pericardial effusion after pediatric cardiac surgery: A single-center study.

BACKGROUND: Postoperative pericardial effusion (pPE) still remains a frequent complication after congenital heart surgery and it usually leads to an increased morbidity and re-hospitalization rate. There are only few published papers about pPE clinical course or large randomized studies that analyze its prevalence or preoperative risk factors. In this regard, we report a single-center 10-years retrospective analysis of prevalence, outcomes and risk factors of postoperative pericardial effusion after congenital heart surgery. METHODS: A retrospective analysis was carried out on 624 patients who underwent congenital heart surgery from January 2010 to December 2019. Study population was divided in two groups basing of the presence of pPE during the first 30 days after the surgery and their perioperative data were compared. Univariate and multivariate analysis were used to find possible risk factors for pPE developing. RESULTS: Ninety-four patients were enrolled in pPE group and 530 in ¬ pPE group. Pericardial effusion was assessed as "mild" in 57 patients (60,6%), as "moderate" in 25 (26,6%), and as "severe" in 12 patients (12,8%). Total correction of Tetralogy of Fallot/Pulmonary atresia seems to be associated with a higher prevalence of pPE in the "Infant" subgroup, while atrial septal defect showed to be a risk factor among "Toddler". In addition, pPE was proved to be much more frequent in Fontan patients in all studied subgroups. Univariate and multivariate analysis revealed that total drain amount, Fontan procedure, postoperative Warfarin therapy, Redo-operations and surgical correction of Tetralogy of Fallot/Pulmonary atresia seem to be risk factors for pPE. Postoperative pericardial effusion was diagnosed between the 4th and the 28th postoperative day but in 88,3% of the cases (83/94) it occurred before the 14th day after the operation. In 58 patients, pPE was clinically silent. CONCLUSIONS: Postoperative pericardial effusion was detected in 88.3% of cases within the first 14 days after the operation. About 69% of these patients were asymptomatic therefore it suggests that routinely echocardiogram after intensive care unit discharge could be a useful tool to screen clinically silent pPE at an early stage, especially in high-risk or unstable patients.

Congenital heart disease in the era of COVID-19 pandemic.

The pandemic of COVID-19 presents an unprecedented challenge to identify effective prevention and treatment. Scientific literature has exploded with many news concerning the different experiences and the first studies in the world. To date May 5 2020, if we carry out a search in pubmed with the keyword "COVID-19", more than 9000 papers appear. While if we carry out a search with the keywords "congenital heart disease and COVID-19", we find only 4 results. This is a viewpoint in which we summarize the most important problems that emerged with the most recent data in the literature.

Prognostic Value of a New Lung Ultrasound Score to Predict Intensive Care Unit Stay in Pediatric Cardiac Surgery.

BACKGROUND: Lung ultrasound (LUS) in pediatric cardiac surgery is gaining consensus. We (1) evaluated the prognostic value of a new LUS-score in pediatric cardiac surgery, and (2) compared LUS-score to conventional risk factors including age, The Society of Thoracic Surgeons/European Association of Cardio-Thoracic Surgery (STAT) score, cardiopulmonary bypass time, and prognostic biomarkers including brain natriuretic peptide and cystatin-C. METHODS: LUS examinations were performed in 237 children (median age, 0.55 years; interquartile range, 0.09-4.15 years) at 12 to 36 hours after surgery. For each hemithorax, 3 areas (anterior/lateral/posterior) were evaluated in the upper and lower halves, constituting 12 total scanning areas. For each site a score was assigned: 0 (rare B lines), 1 (separated B lines), 2 (coalescent B lines), 3 (loss of aeration), and total LUS score was calculated as sum of all sites. The primary endpoints were intensive care unit length of stay and extubation time. RESULTS: The mean total LUS score was 12.88 ± 6.41 (range, 0-26) and was higher in newborns (16.77 ± 5.25) compared with older children (5.36 ± 5.57; P < .001). On univariate analysis, LUS score was associated inversely with age (beta 0.26; P = .004) and body surface area (beta 3.41 P = .006) and positively with brain natriuretic peptide (beta 1.65; P < .001) and cystatin-C (beta 2.41; P < .001). The LUS score, when added as continuous predictor to a conventional risk model (age, STAT score, and cardiopulmonary bypass time) emerged significant both for intensive care unit length of stay (beta 0.145, P = .047) and extubation time (beta 1.644; P = .024). When single quadrants were analyzed, only anterior LUS score was significant (intensive care unit length of stay beta, 0.471; P = .020; extubation time beta 5.530; P = .007). CONCLUSIONS: Our data show the prognostic incremental value of a new LUS score over traditional risk factors in pediatric cardiac surgery.

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.

Right thoracotomy for aortic valve replacement in the adolescents with bicuspid aortic valve.

BACKGROUND: In this study, we compared our experience about early and midterm follow-up outcomes for right anterolateral minithoracotomy (RAMT) vs full sternotomy (FS) in surgical aortic valve replacement (AVR) among adolescents with bicuspid aortic valve (BAV). METHODS: Patients were retrospectively enrolled from January 2008 to December 2017. Inclusion criteria were patients with BAV who had to undergo to AVR. They were divided in two groups: RAMT and FS. The choice of RAMT was based on individual surgeon's preferences or when expressly requested by patient that was informed of nonconventional approach. RESULTS: We enrolled 61 patients, 23 in RAMT group and 38 in FS group. The mean age was 15.6 ± 1.7 years for RAMT group and 16.1 ± 1.5 years for FS group (P = .23). The RAMT group had a higher prevalence of female gender (P = .04). The patients in the RAMT group had longer cardiopulmonary bypass (115.2 ± 18.5 vs 102.2 ± 16.5 min; P = .006) and cross-clamp time (78.6 ± 18.1 vs 74.3 ± 15.2 min; P = .01). No patients required intraoperative conversion to FS. No differences were found in ventilation times, postoperative intensive care unit (ICU), and hospital length of stay for both groups. Follow-up echocardiograms were available for all patients at median of 5.2 years (range 0.5-9.6 years, median 5.4 years for RAMT and 5.1 for FS) and no patient required reoperation for aortic prosthesis malfunction. CONCLUSIONS: Our study shows that RAMT is safe and effective as FS. Although the RAMT operation takes slightly more operation time, it is not associated with major adverse effects.

Reevaluating the Importance of Modified Ultrafiltration in Contemporary Pediatric Cardiac Surgery.

OBJECTIVE(S): Modified ultrafiltration has gained wide acceptance as a powerful tool against cardiopulmonary bypass morbidity in pediatric cardiac surgery. The aim of our study was to assess the importance of modified ultrafiltration within conditions of contemporary cardiopulmonary bypass characteristics. METHODS: Ninety⁻eight patients (overall cohort) weighing less than 12 kg undergoing surgical repair with cardiopulmonary bypass were prospectively enrolled in a randomized protocol to receive modified and conventional ultrafiltration (MUF group) or just conventional ultrafiltration (non-MUF group). A special attention was paid to forty-nine neonates and infants weighing less than 5 kg (lower weight (LW) cohort). RESULTS: Post-filtration hematocrit was significantly higher in the MUF group for both cohorts (overall cohort p = 0.001; LW cohort p = 0.04), but not at other time points. During the postoperative course, patients in the MUF group received fewer packed red blood cells, (overall cohort p = 0.01; LW cohort p = 0.07), but required more fresh frozen plasma (overall cohort p = 0.04; LW cohort p = 0.05). There was no difference between groups in hemodynamic state, chest tube output, duration of mechanical ventilation, respiratory parameters, duration of intensive care unit, and hospitalization stay. CONCLUSIONS: If conventional ultrafiltration provides adequate hemoconcentration modified ultrafiltration does not provide additional positive benefits except for reduction in blood cell transfusion, This, however, comes at the cost of needing more fresh frozen plasma. Of particular importance is that this also applies to infants with weight bellow 5 kg where modified ultrafiltration was supposed to have the greatest positive impact.

The Fate of the Tricuspid Valve After the Transatrial Closure of the Ventricular Septal Defect.

BACKGROUND: The transatrial repair of the ventricular septal defect (VSD) requires an adequate exposure of its rim. We retrospectively evaluated the effect of using the tricuspid valve incision (TVI) technique, with detachment or radial incision, on the postoperative outcome of children undergoing surgical VSD repair. METHODS: From January 2008 to September 2017, we retrospectively enrolled 141 patients, divided into two groups: 97 patients (68.8%) underwent TVI and 44 patients (31.2%) did not undergo TVI. All patients received an echocardiogram upon discharge from the hospital and after 1 month, 3 months, 6 months, and 1 year from the treatment. RESULTS: No perioperative or late deaths occurred. TVI was associated with a slightly longer cardiopulmonary bypass and cross-clamp time, but there were no differences in the surgical outcome between the two groups. Moreover, no differences occurred concerning residual VSD, atrioventricular block, or tricuspid regurgitation at discharge. Echocardiograms at follow-up were available for 134 patients (95%) with a median of 5.3 years (range, 0.5 to 9.3 years), and the degree of tricuspid regurgitation did not differ between groups. No patient required reoperation for tricuspid regurgitation or residual interventricular shunt. Finally, no difference was found when the two TVI subgroups were compared. CONCLUSIONS: TVI should be used whenever intraoperative exposure of VSD is compromised to avoid a residual shunt and atrioventricular block. Here we show that this procedure does not significantly compromise tricuspid function, although a large, multicenter, randomized controlled trial is advised to validate this hypothesis.

Normative Data for Left and Right Ventricular Systolic Strain in Healthy Caucasian Italian Children by Two-Dimensional Speckle-Tracking Echocardiography.

BACKGROUND: There is an increasing interest in echocardiographic strain (ε) measurements for the assessment of ventricular myocardial function in children; however, pediatric nomograms remain limited. Our aim was to establish pediatric nomograms for the left ventricular (LV) and the right ventricular (RV) ε measured by two-dimensional speckle-tracking echocardiography (2D-STE) in a large cohort of healthy children prospectively enrolled. METHODS: Echocardiographic measurements included STE LV longitudinal and circumferential and RV longitudinal global end-systolic ε. Age, weight, height, heart rate (HR), and body surface area (BSA) were used as independent variables in different analyses to predict the mean values of each measurement. Echocardiograms were performed by Philips-iE33 systems (Philips, Bothell, WA) and offline measurements on Philips-Q-Lab-9. RESULTS: In all, 721 subjects (age 31 days to 17 years; 48% female) were studied. Low coefficients of determination (R2) were noted among all of the ε parameters evaluated and adjusted for age, weight, height, BSA, and HR (i.e., R2 all ≤ 0.10; range, 0.01-0.088). This hampered the possibility of performing z-scores with a sufficient reliability. Thus, we are limited to presenting data as mean values (±SD) stratified for age groups and divided by gender. LV longitudinal ε values decreased with age (P < .001), while no significant age-related variations were noted for RV longitudinal ε. A significant base-to-apex (lowest to highest) gradient in circumferential LV ε values was noted at all ages (P < .001). CONCLUSIONS: We report pediatric echocardiographic normative data for 2D-STE for the LV and RV ε by using vendor-specific software. Our results confirm previous observations, showing only little variations of strain parameters with age and gender.

Lung ultrasound reclassification of chest X-ray data after pediatric cardiac surgery.

INTRODUCTION: Lung ultrasound is gaining consensus for the diagnosis of some pulmonary conditions. Pulmonary complications are common in pediatric cardiac surgery. However, its use remains limited in this setting. Our aim was to test the feasibility of lung ultrasound following pediatric cardiac surgery and to compare lung ultrasound and chest X-ray findings, assessing whether lung ultrasound may provide additional information. METHODS: One hundred and thirty-eight lung ultrasound examinations were performed in 79 children (median age 9.3 months) at different time points after surgery. For each hemithorax, 3 areas (anterior/lateral/posterior) have been evaluated in the upper and lower halves of the chest (for a total of 6 scanning sites per side). Pleural effusion, atelectasis, and the number of B-lines were investigated. RESULTS: Lung ultrasound was feasible in all cases in at least 1 of the 3 areas. Feasibility was different for the lateral, posterior, and anterior areas (100%, 90%, and 78%, respectively). The posterior areas were more sensitive than anterior and lateral ones in the diagnosis of effusion/atelectasis. In 81 cases, lung ultrasound allowed reclassification of chest X-ray findings, including 40 new diagnoses (diagnosis of effusion/atelectasis with negative chest X-ray reports) and 41 changes in diagnosis (effusions reclassified as atelectasis/severe congestion or vice versa). Although new diagnosis of small-to-moderate effusion/atelectasis was of limited clinical value, in 29 cases the new diagnosis changed the therapeutic approach. CONCLUSION: Lung ultrasound is feasible and accurate for the diagnosis of common pulmonary conditions after pediatric cardiac surgery, allowing reclassification of chest X-ray findings in a significant number of patients.

Use of linear and convex ultrasound transducers for evaluation of retrosternal area in patients after cardiac surgery.

The use of linear and convex transducers is usually reserved for ultrasound imaging of vessels, abdominal organs, and lungs. However, the use of these transducers may be extended for the evaluation of thoracic structures. We describe our experience with the use of linear and convex transducers in the evaluation of the retrosternal area, which is usually difficult to visualize by conventional echocardiography and so could provide improved diagnostic information in patients after cardiac surgery.