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.

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.

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.

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.

Selective perfusion of coronary vasculature in preterm sheep: a methodological innovation undermined by unfavourable operation of the foramen ovale.

Antenatal cardiac intervention affords new prospects for hypoplastic left heart syndrome. Its success, however, may come not only from absence of impediments to blood flow but also from a sufficiently developed cardiac wall. Here, we examined the feasibility to perfuse selectively the fetal coronary circulation for treatment with growth promoting agents. Pregnant sheep (94-114 days gestation, term 145 days) were used. An aortic stop-flow procedure was developed for intracoronary access in the nonexposed fetus and human mesenchymal stem cells and their exosomes served as test agents. We found that aortic stop-flow ensures preferential distribution of fluorescent microspheres to the heart. However, intracoronary administration of stem cells or exosomes was detrimental, with fetal demise occurring around surgery or at variable intervals afterwards. Coincidentally, stop-flow caused by itself a marked rise of intraluminal pressure within the occluded aorta along with histological signs of coronary obstruction. We conclude that it is feasible to perfuse selectively the coronary circulation of the preterm fetus, but treatments are not compatible with survival of the animals. The cause for failure is found in the absence of hemodynamic compensation to stop-flow via a left-to-right shunt. This unexpected event is attributed to a largely membranous foramen ovale, characteristic of sheep, that collapses under pressure.

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.

Echocardiographic examination of mitral valve abnormalities in the paediatric population: current practices.

We reviewed the recent literature for echocardiographic assessment of mitral valve abnormalities in children. A literature search was performed within the National Library of Medicine using the keywords "mitral regurgitation and/or stenosis, children." The search was refined by adding the keywords "echocardiographic definition, classification, and evaluation." Thirty-one studies were finally included. Significant advances in echocardiographic imaging of mitral valve defects, mainly due to the implementation of three-dimensional technology, contribute to a better understanding of the underlying anatomy. However, heterogeneity between classification systems of mitral valve disease severity is a serious problem. For regurgitant lesions, there is only very limited evidence from small studies that support the adoption of quantitative/semi-quantitative indexes commonly employed in adults. Despite the lack of evidence base, qualitative evaluation of regurgitation severity is often employed. For stenotic lesions, no clear categorisation based on trans-valvular echocardiography-derived "gradients" has been consistently applied to define mild, moderate, or severe obstruction across different paediatric age ranges. Quantitative parameters such as valve area have also been poorly validated in children. Adult recommendations are frequently applied without validation for the paediatric age. In conclusion, significant advances in the anatomical evaluation of mitral valve diseases have been made, thanks to three-dimensional echocardiography; however, limitations remain in the quantitative/semi-quantitative estimation of disease severity, both with respect to valvular regurgitation and stenosis. Because adult echocardiographic recommendations should not be simply translated to the paediatric age, more specific paediatric guidelines and standards for the assessment of mitral valve diseases are needed.

Cardiac Children's Hospital Early Warning Score: Italian Validation.

BACKGROUND/PURPOSE: The Cardiac Children's Hospital Early Warning Score (C-CHEWS) is an early warning scale used to identify paediatric patients experiencing clinical deterioration which may warrant a transfer to an ICU. However, no studies have tested the C-CHEWS in an Italian paediatric cardiac population. The aims of this study were to translate/back-translate and validate the Italian version of the C-CHEWS and its algorithm. DESIGN AND METHOD: Retrospective study. Sensitivity, specificity, accuracy, positive predictive value, negative predictive value were used to evaluate the performance of C-CHEWS. In additions the Cohen's kappa statistic was calculated to evaluate the agreement between patient's status described by C-CHEWS score (≥5) and actual ICU transfer. RESULTS: High discrimination was observed for sensitivity (81.5%), specificity (99.6%), accuracy (99.7%), positive predictive value (86.7%), and negative predictive value (99.8%). The Cohen's kappa score was observed to be equal to 0.837 (p-value <0.001) indicating there was excellent significant agreement between a C-CHEWS score ≥ 5 and effective evaluation for patients transfer to an ICU. CONCLUSION: The Italian version of the C-CHEWS proved to be a sensitive, specific and reliable tool in the early detection of a physical deterioration of hospitalized paediatric cardiac surgical patients. PRACTICE IMPLICATIONS: This tool may help doctors, nurses and all healthcare professionals to promptly recognize and treat clinical deterioration and facilitate urgent transfers to the PICU.

Double Orifice Mitral Valve in Tricuspid Atresia: A Rare Association.

We here report a neonate with prenatal echocardiographic diagnosis of tricuspid atresia, with normally related great vessels, and large ventricular septal defect. This diagnosis could be confirmed with echocardiography at birth. An additional double mitral orifice was also seen. This is a very rare association.

Strengths, Limitations, and Geographical Discrepancies in the Eligibility Criteria for Sport Participation in Young Patients With Congenital Heart Disease.

OBJECTIVE: Benefits of physical activity has been shown in children with congenital heart disease (CHD). In several forms of CHD, the risk of sudden death remains a major concern both for parents and clinicians, who in turn will have to consider the risk-benefit ratio of sport participation versus restriction. DATA SOURCE: A literature search was performed within the National Library of Medicine using the keywords: Sport, CHD, and Eligibility. The search was further refined by adding the keywords: Children, Adult, and Criteria. MAIN RESULTS: Fifteen published studies evaluating sport eligibility criteria in CHD were included. Seven documents from various scientific societies have been published in the past decade but which of them should be adopted remains unclear. Our research highlighted accuracy and consistency of the latest documents; however, differences have emerged between the US and European recommendations. Eligibility criteria were consistent between countries for simple congenital heart defects, whereas there are discrepancies for borderline conditions including moderate valvular lesions and mild or moderate residual defects after CHD repair. Furthermore, some of the more severe defects were not evaluated. Multiple recommendations have been made for the same CHD, and cut-off values used to define disease severity have varied. Published eligibility criteria have mainly focused on competitive sports. Little attention was paid to recreational activities, and the psychosocial consequences of activity restriction were seldom evaluated. CONCLUSIONS: Comprehensive consensus recommendations for sport eligibility evaluating all CHD types and stages of repair are needed. These should include competitive and recreational activities, use standardized classifications to grade disease severity, and address the consequences of restriction.

Echocardiographic assessment of pediatric semilunar valve disease.

We reviewed echocardiography literature for the assessment and management of semilunar valve disease in children. A search was performed within the National Library of Medicine using the keywords aortic stenosis (AS), aortic regurgitation, pulmonary stenosis (PS), and pulmonary regurgitation in children. The search was further refined adding the keywords-pediatric, neonates, echocardiographic definition, classification, evaluation. Thirty-eight studies were included. For stenotic lesions, there were sufficient consistencies between Doppler and invasive gradients (especially for PS), while other quantitative parameters used in adults showed significant limitations when applied to children. Heterogeneities remain in the range of Doppler measurements utilized to define mild vs moderate vs severe AS/PS, and to guide management. There is sufficient consensus regarding indications for interventions. In regurgitant lesions, there is weak evidence supporting the use of quantitative or semiquantitative parameters after correction for body surface area; clear indications for intervention are lacking. Because adult echocardiographic recommendations cannot be simply translated to the pediatric age, more specific pediatric guidelines and standards for the assessment of semilunar valve disease are needed.

Custodiol Solution and Cold Blood Cardioplegia in Arterial Switch Operation: Retrospective Analysis in a Single Center.

BACKGROUND: The cardioplegia is one of the most significant tools used to increase myocardial protection. The aim of our study is to compare the use of Custodiol solution versus intermitted blood cardioplegia in a retrospective analysis of data for patients who underwent arterial switch operation in our institution. MATERIAL AND METHODS: From January 2008 to March 2011, myocardial protection was performed in 44 neonates (blood group) with intermittent blood cardioplegia. From March 2011 to November 2014, myocardial protection was performed in 50 neonates (Custodiol group) with one-shot anterograde Custodiol cardioplegia. RESULTS: Cardiopulmonary bypass and aortic cross-clamp were more favorable in Custodiol group (p-value 0.005 and ≤ 0.00001). The rate of delayed sternal closure was 63.6% in the blood group and 52% in the Custodiol group (p = 0.25). In the postoperative outcomes we did not find differences between the two groups. The 30-day mortality was one patient in the blood group (p = 0.46). We observed a transient ischemic electrocardiogram in 10 patients of the blood group and in 14 of the Custodiol group (p = 0.72), all cases with full resolution during hospitalization without coronary reoperation. A trend of higher peak of troponin-I and brain natriuretic peptide in Custodiol group has been reported. CONCLUSION: No prefect cardioplegia exists, the Custodiol solution does not cause extra/additional myocardial damage in arterial switch operation. In our experience this strategy seems warranted to simplify the procedure and to be more comfortable for the surgeon.

Strengths and Limitations of Current Adult Nomograms for the Aorta Obtained by Noninvasive Cardiovascular Imaging.

Normalized measurements for the evaluation of aortic disease severity are preferred to the adoption of generic cutoff values. The purpose of this review is to evaluate the strengths and limitations of currently available aortic nomograms by echocardiography, computed tomography (CT), and magnetic resonance imaging (MRI). A literature search was conducted accessing the National Library of Medicine using the keywords normal values, aorta, echocardiography, CT, and MRI. Addition of these keywords further refined the results: reference values, nomograms, aortic arch, and adults. Thirty studies were included in the final analysis. Despite the strengths noted in the recent investigations, multiple methodological and numerical limitations emerged. The numerical limitations included sample size limitation in most of the studies (only few investigations consisted of >800 subjects and many had 70-300), lack of aortic arch measurements, and paucity of data for non-Caucasian subjects. Methodological limitations consisted of lack of standardization in measurements (systole vs. diastole, internal vs. external border, axial vs. orthogonal planes), heterogeneity and data normalization issues (various age intervals used, body size often not evaluated, data expressed as observed values rather than estimated values by z-score), and study design issues. The designs were mostly retrospective with poorly defined inclusion and exclusion criteria. The nomograms presented range of normality with significant differences, but also with some reproducible pattern. Despite recent advances, multiple methodological or numerical limitations exist in adult nomograms for the aorta. Comprehensive nomograms of aortic dimensions at multiple levels including the aortic arch for different imaging techniques, involving a wide sample size, and using standardized methodology for measurements and data normalization are warranted. The availability of robust nomograms may encourage the use of personalized rather than generic cutoff values.

Review and status report of pediatric left ventricular systolic strain and strain rate nomograms.

Interest in strain (ε) and strain rate (SR) for the assessment of pediatric left ventricular (LV) myocardial function has increased. However, the strengths and limitations of published pediatric nomograms have not been critically evaluated. A literature search was conducted accessing the National Library of Medicine using the keywords myocardial velocity, strain, strain rate, pediatric, reference values, and nomograms. Adding the following keywords, the results were further refined: neonates, infants, adolescents, range/intervals, and speckle tracking. Ten published studies evaluating myocardial velocities, ε, or SR nomograms were analyzed. Sample sizes were limited in most of these studies, particularly in terms of neonates. Heterogeneous methods-tissue Doppler imaging, two- and three-dimensional speckle tracking-were used to perform and normalize measurements. Although most studies adjusted measurements for age, classification by specific age subgroups varied. Few studies addressed the relationships of ε and SR measurements to body size and heart rate. Data have been generally expressed by mean values and standard deviations; Z scores and percentiles that are commonly employed for pediatric echocardiographic quantification have been never used. Reference values for ε and SR were found to be reproducible in older children; however, they varied significantly in neonates and infants. Pediatric nomograms for LV ε and SR are limited by (a) small sample sizes, (b) inconsistent methodology used for derivation and normalization, and (c) scarcity of neonatal data. Some of the studies demonstrate reproducible patterns for systolic deformation in older children. There is need for comprehensive nomograms of myocardial ε and SR involving a large population of normal children obtained using standardized methodology.

Supra-annular mitral valve implantation in very small children.

OBJECTIVE: Mitral valve replacement (MVR) is a surgical option when mitral valvuloplasty is not feasible/successful. This study reviews our experience with MVR in very young children. METHODS: From July 2004 to January 2014, seven children (mean age 13.3 ± 11.2 months; range 4 months to 35 months; mean body weight 6.0 ± 2.2 kg) underwent MVR with a mechanical prosthesis in the supra-annular position. To provide better exposure in the left atrium, we performed in all but one case a biatrial transeptal incision according to Guiraudon. Six patients had congenital defects of the mitral valve and one had rheumatic. Six patients had undergone previous cardiosurgical procedures. RESULTS: All patients were implanted with a CarboMedics (CarboMedics, Austin, TX, USA) mechanical prosthesis. Mean prosthesis size was 19.0 ± 3.1 mm (range 16 to 25). There were no cases of operative or late mortality. At follow-up (mean 67.1 ± 34.8 months; range 25 to 108 months) two patients (28.6%) required reoperation both for thrombotic pannus formation over the disc at two and three months from first operation, respectively; only in one case was replacement necessary. CONCLUSION: Supra-annular MVR may be considered a feasible secondary surgical option in children with a small annulus when mitral valvuloplasty is unsuccessful or unsuitable. Early and mid-term outcomes are acceptable but complications are not uncommon, especially related to thrombotic events.

V-shape reduction plasty to reduce the neoaortic root-ascending aorta discrepancy in children undergoing arterial switch operation.

Preoperative aortopulmonary mismatch is considered a risk factor for the development of aortic insufficiency after surgery for transposition of the great arteries. We have approached these children using a neoaortic root reduction plasty. Five children with severe PA-AO discrepancy (median age 57 days) underwent arterial switch operation (ASO) associated with a V-shape neoaortic reduction plasty. At follow-up aortic regurgitation remains stable in all patients and no supra-aortic obstruction and/or neoaortic root dilatation were observed.