DATA in BRIEF of: Interventional Cardiac Catheterization in Neonatal Age: Results in a Multi-centre Italian Experience.

A comprehensive description of morbidity and mortality as well as risk factors of interventional cardiac catheterization performed in neonatal age was reported in our paper recently published on the International Journal of Cardiology (IJCA28502; PII: S0167-5273(20)30384-3; DOI: 10.1016/j.ijcard.2020.04.013). Eight Italian high-volume centres of Paediatric Cardiology were involved in this observational, retrospective data collection and analysis. In this dataset, clinical and procedural characteristics of 1423 newborns submitted to 1551 interventional cardiac catheterization procedures were analyzed. Primary outcomes were considered procedure and in-hospital mortality as well as major adverse event and procedural failure rates. Secondary outcomes were considered minor adverse events and need for blood transfusion. Targets of this data analysis were: 1) to evaluate the overall major risk factors of interventional cardiac catheterization; 2) to identify the most hazardous interventional procedures; 3) to assess possible trends of individual procedures as well as their outcome over time; 4) to find possible relationships between the volume activity of any centre and the procedure and follow-up outcome. In particular, this Data in Brief companion paper aims to report the specific statistic highlights of the multivariable analysis (binary logistic regression) used to assess the impact of any potential risk factors on the type of procedure over a short-term follow-up.

Interventional cardiac catheterization in neonatal age: results in a multicentre Italian experience.

BACKGROUND: Despite recent technical advances, interventional cardiac catheterization is still challenging in neonatal age and no specific data concerning early outcome are so far published in literature. METHODS: Neonatal trans-catheter cardiac interventions performed in high-volume Italian referral centers were retrospectively analyzed. Primary outcomes were procedural major adverse events, in-hospital mortality and procedural failure. Secondary outcomes were minor adverse events and need for blood transfusion. RESULTS: From January 2000 to December 2017, 1423 newborns (mean weight 3.0 ± 0.6 kg, range 1.0-5.8; median age 2.0 days) underwent interventional cardiac catheterization. Overall, global procedure adverse event rate and in-hospital mortality were 10.2% and 5.2%, respectively. At multi-variable analysis, primary composite outcome was significantly related to low-weight (<2.5 kg) (p < 0.01) and younger age (≤7 days) (p < 0.01) at the procedure, prematurity (p < 0.01), uni-ventricular physiology (p < 0.01), associated genetic syndromes (p < 0.01) and procedure risk category (p < 0.01). No relationship between volume of activity of any single center and procedure outcome was found. Over time, a trend toward an increased number of procedures and their complexity was recorded. Trans-catheter management of cardiac malformations with critical, duct-dependent pulmonary blood flow by arterial duct stenting or right ventricular outflow tract stenting showed the highest increase. CONCLUSIONS: Interventional cardiac catheterization is relatively safe and feasible in neonatal age. Peri-natal age, low weight, uni-ventricular physiology and genetic syndromes still significantly contribute to procedural morbidity and in-hospital mortality of this approach.

Sex differences for major congenital heart defects in Down Syndrome: A population based study.

BACKGROUND: Down syndrome (DS) is the most common autosomal chromosomal anomaly in liveborn infants. About 40% of infants with DS have a major congenital heart defect (CHD). Among them, atrioventricular septal defects (AVSD), atrial septal defects (ASD), ventricular septal defect (VSD) and Tetralogy of Fallot (ToF) are the most common. The aim of this study was to estimate the sex difference in the occurrence of CHD in infants with DS comparing it with non-DS infants. METHOD: Live birth cases of DS diagnosed by the first year of life were extracted from the Registry of Congenital Anomalies of Tuscany (2003-2015 period). CHDs associated with DS were detected both from the registry and the hospital discharge data. Sex differences in total CHDs and ASD, VSD, AVSD, severe CHDs, ToF subgroups were investigated. Relative Risks between males and females (RRMF) with p-values and 95% confidence interval (95% CI) were estimated. RRMF of CHD in infants with DS was compared to RRMF in infants without DS. The ratio between relative risks (RRR) was estimated. RESULTS: A total of 230 live birth cases of DS were analyzed, with a prevalence of 5.70 per 10,000 births. Sex ratioMF was 1.3. One hundred and one DS cases (43.9%) were associated with at least one CHD. Among them, CHDs are more frequent in females (total CHD: RRMF = 0.62; 95% CI: 0.46-0.83, ASD: RRMF = 0.40; 95% CI: 0.21-0.77, severe CHD: RRMF = 0.58; 95% CI: 0.35-0.95, AVSD: RRMF = 0.57; 95% CI: 0.32-1.00, VSD: RRMF = 0.59; 95% CI: 0.35-1.00). Four cases of ToF were observed (all males). Sex difference was more evident in DS than in non-DS infants (RRR = 0.63; 95% CI: 0.52-0.77), in particular for severe CHDs (RRR = 0.38; 95% CI: 0.25-0.57). CONCLUSION: The increased sex difference for CHDs in DS suggests a possible role of sex as effect modifier in the association between DS and CHD. The results enforce the evidence on sex differences for CHDs in DS and can stimulate future genetic research activities.

Optimus covered stent: Advanced covered stent technology for complex congenital heart disease.

AIM: To assess the acute results of the first human use of the Optimus covered stent in complex coarctation of the aorta. METHODS AND RESULTS: We successfully implanted the Optimus covered stent in eight cases in patients whose preprocedural anatomy looked challenging for currently available covered stents. Six of the patients had native coarctation with one recoarctation following surgical repair. There were no significant complications with reduction in the mean invasive gradient from 22 to 1 mm Hg. The length of stents used ranged from 33 to 57 mm with a median shortening after expansion of 13%. Postprocedural follow-up with magnetic resonance imaging or computed tomography has not shown evidence of fracture or migration or renarrowing. The median duration of follow-up is 10 months. CONCLUSIONS: Preliminary results show that the Optimus covered stent is safe and efficacious for use in patients with coarctation of challenging morphology. A systemic trial will be required to evaluate this stent for more widespread practice.

Incidence and natural history of neonatal isolated ventricular septal defects: Do we know everything? A 6-year single-center Italian experience follow-up.

BACKGROUND: Despite ventricular septal defects (VSDs) are the most common congenital heart diseases (CHDs) in the neonatal period, their incidence and natural history are still debated and their follow-up and management strategies remain controversial. Our aim was to evaluate the incidence and natural history of isolated VSDs. METHODS: From January 1996 to December 2015 all neonates with a CHD suspicion were referred to the Cardiological Department of Grosseto Misericordia Hospital. Only newborns with confirmed isolated VSD were enrolled in this study and followed for 6 years. RESULTS: Our 343 newborns with an isolated VSD (incidence of 10.45/1000/births) account for 64% of all detected CHDs. VSDs location were as follows: muscular (73.8%), perimembranous (11.3%), inlet (1%), and outlet (0.8%). Of the located VSDs, 90% were small, 7.5% moderate, and 2.5% large, respectively. Spontaneous closure was observed in 96 (29.2%) of the VSD patients at 6-month, 198 (60.2%) at 1-year, 261 (79.3%) at 2-year, and in 302 (91.8%) at 6-year follow-up. Risk factors for defect persistence were a perimembranous location (P = .001; HR: 0.508, CI: 0.342-0.755), detection of multiple defects (P = .043; HR: 0.728, CI: 0.536-0.990), and male gender (P < .048; HR: 0.783, CI: 0.615-0.998), respectively. CONCLUSIONS: We here provide an incidence and natural history of neonatal isolated VSDs in a neonatal Caucasian population. These data may be useful for the development of expert consensus/standard recommendation guidelines for the follow-up and VSD management, data that are currently lacking.

Echocardiographic nomograms for upper abdominal aorta Doppler systolic wave values and systo-diastolic diameters variations in children.

BACKGROUND: Abdominal aorta pulsatility and blood flow patterns are important diagnostic indicators in congenital heart disease. Reference values for these indexes are lacking. METHODS: We prospectively studied abdominal aorta pulsed-wave Doppler systolic peak velocity, deceleration time, and wave duration, and two-dimensional vessel diameters in systole and diastole in healthy Caucasian children. Heteroscedasticity was accounted for by White or Breusch-Pagan test. 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. Structured Z-scores were then computed. RESULTS: In all, 853 subjects (age 0 days to 17 years; 45% females; BSA 0.12-2.12m2) were studied. The predicted values and Z-score boundaries are presented. Data are also presented as mean±2 SDs for a given BSA. CONCLUSIONS: We report paediatric echocardiographic nomograms for multiple proximal abdominal aorta parameters including pulsed-wave Doppler systolic velocities, deceleration time, wave duration, and two-dimensional vessel diameter variations. Significant variations in these functional indexes with age were found that should be taken into account in clinical practice. At lower ages, steeper and shorter pulsed-wave Doppler peak velocity and limited pulsatility should be expected as physiologic findings.

Nomograms for two-dimensional echocardiography derived valvular and arterial dimensions in Caucasian children.

BACKGROUND: Despite recent advances, current pediatric echocardiographic nomograms for valvular and arterial dimensions remain limited. METHODS: We prospectively studied healthy Caucasian Italian children by two-dimensional (2D) echocardiography. Echocardiographic measurements for 18 valvular and arterial dimensions were performed and models were generated testing for linear, logarithmic, exponential, and square root relationships. Heteroscedasticity was accounted for by White or Breusch-Pagan test. Age, weight, height, heart rate, and body surface area (BSA) were used as independent variables in different analyses to predict the mean values of each measurement. Structured Z-scores were then computed. RESULTS: In all, 1151 subjects (age 0 days to 17 years; 45% females; BSA 0.12-2.12m2) were studied. The Haycock formula was used when presenting data as predicted values (mean±2 SDs) for a given BSA and within equations relating echocardiographic measurements to BSA. The predicted values and Z-score boundaries for all measurements are presented. CONCLUSIONS: We report echocardiographic nomograms for valvular and arterial dimensions derived from a large population of children. Integration of these data with those of previous reports would allow for a comprehensive coverage of pediatric 2D echocardiographic nomograms for measurement of 2D cardiac structures.

Major Aortopulmonary Collaterals in Transposition of the Great Arteries: A Cause for Preoperative and Postoperative Hemodynamic Imbalance.

We describe a case of transposition of the great arteries (TGA) with significant aortopulmonary collateral vessels causing management difficulties before and after an arterial switch operation. Preoperatively, the presence of collaterals exacerbated aortic diastolic runoff and led to myocardial ischemia. Postoperatively, despite uneventful extubation, the infant developed clinical heart failure characterized by pericardial and pleural effusions and feeding difficulties, which promptly resolved with percutaneous embolization of collaterals. Major aortopulmonary collaterals are rarely associated with TGA, but if present they may cause important hemodynamic imbalance in infants with TGA.

Nomograms for mitral inflow Doppler and tissue Doppler velocities in Caucasian children.

BACKGROUND: Pediatric echocardiographic nomograms for systolic/diastolic functional indices are limited by small sample size and inconsistent methodologies. Our aim was to establish pediatric nomograms for mitral valve (MV) pulsed wave Doppler (PWD) and tissue Doppler imaging (TDI) velocities. METHODS: We performed PWD/TDI measurements of MV velocities and generated models testing for linear/logarithmic/exponential/square root relationships. Heteroscedasticity was accounted for by White test or Breusch-Pagan test. 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. RESULTS: In all, 904 Caucasian Italian healthy children (age 0 days-17 years; 45.5% females; BSA 0.12-2.12m(2)) were prospectively studied. No individual variable provided equations with an acceptable coefficient of determination (R(2)) and even the inclusion of multiple variables in the model resulted in only a partial amelioration of the R(2). Higher R(2) were obtained for PWD-E deceleration time (0.53), septal (Se') and lateral (Le') MV-TDI e' velocity (Se': 0.54; Le': 0.55). Variability was higher at lower age and BSA. In older children patterns were more reproducible; however, the exclusion of neonates did not substantially improve the final models. The low R(2) hampered building of z-scores and calculation of estimated percentiles. Thus normative data have been presented as observed percentile according to age for all measurements. CONCLUSIONS: We report normal ranges for PWD and TDI mitral velocities derived from a large population of Caucasian children. Variability of diastolic patterns especially at lower ages needs to be taken into account.

[Prevalence, mortality and lethality of congenital heart defects from the Tuscan Registry (Tuscany Region, Central Italy), 1992-2009]. / Prevalenza, mortalità e letalità dei difetti cardiaci congeniti dal Registro toscano, 1992-2009.

OBJECTIVES: to estimate prevalence, mortality and lethality of congenital heart defects (CHDs), and to evaluate time trend by comparing two birth cohorts (1992- 2000 vs. 2001-2009). DESIGN: descriptive study using a population-based registry: the Tuscan Registry of congenital defects (RTDC), which is a member of the European network of surveillance of congenital anomalies (EUROCAT) since 1979. SETTING AND PARTICIPANTS: CHD cases were collected by the RTDC between 1992 and 2009. MAIN OUTCOME MEASURES: prevalence and mortality rates were calculated for all and non-chromosomal CHDs. The non-chromosomal cases were classified in three severity classes (SI, SII, SIII), according to decreasing perinatal mortality. It was estimated prevalence of: live births, foetal deaths (stillbirths and spontaneous abortions from 20 weeks gestation), terminations of pregnancy for foetal anomaly (TOPFAs) at any gestational age, and of prenatally diagnosed cases. Perinatal, neonatal and infant mortality were calculated. Early-neonatal, neonatal and infant lethality were calculated for the non-chromosomal cases. RESULTS: 3,653 cases were identified out of 486,947 live births and 1,883 stillbirths, 95% of which were non-chromosomal cases. The total average prevalence was 7.47/1,000 births. The overall prevalence decreased significantly in all CHDs (Prevalence ratio - PR: 0.86; 95%CI 0.80-0.91) as well as in non-chromosomal (PR: 0.86; 95%CI 0.80-0.92), while in the more severe defects (SI/SII combined cases) the total prevalence was stable over time. In more severe cases, prevalence of live births decreased significantly (PR: 0.83; 95%CI 0.71-0.97), while the greater increase of TOPFAs and of diagnosed cases was observed in the prenatal period. All three mortality rates reduced significantly over time. In the more severe cases, the greatest rate mortality reduction was observed for neonatal mortality (Mortality ratio - MR: 0.29; 95%CI 0.16-0.49) than perinatal (MR: 0.37; 95%CI 0.19-0.70) and infant mortality (MR: 0,40; 95%CI 0.28-0.56). In this group of CHDs, early neonatal lethality decreased from 10% to 3%, neonatal lethality from 17% to 6%, and infant lethality from 24% to 10%. CONCLUSIONS: the results suggest that the improvements in prenatal diagnosis and in foetal and neonatal treatment and care have reduced the burden of congenital heart defects on the health of the Tuscany population.

Congenitally palliated scimitar syndrome.

We present a rare case of scimitar syndrome in which the scimitar vessel, collecting all the right pulmonary veins, was stenotic at its junction, with the inferior caval vein and two anomalous vessels, connecting to the same venous collector, draining most of the flow to the left atrium. We arbitrarily defined this rare anatomical variant as a congenitally palliated scimitar syndrome.

Recommendations from the Association of European Paediatric Cardiology for training in diagnostic and interventional cardiac catheterisation.

Cardiac catheterisation in congenital heart disease is a developing field. Patients' ages range from foetus to adulthood. This document is a revision and update of the previously published recommendations and summarises the requirements for training in diagnostic and interventional cardiac catheterisation.

Echocardiographic nomograms for chamber diameters and areas in Caucasian children.

BACKGROUND: Although a quantitative evaluation of cardiac chamber dimensions in pediatric echocardiography is often important, nomograms for these structures are limited. The aim of this study was to establish reliable echocardiographic nomograms of cardiac chamber diameters and areas in a wide population of children. METHODS: A total of 1,091 Caucasian Italian healthy children (age range, 0 days to 17 years; 44.8% female) with body surface areas (BSAs) ranging from 0.12 to 1.8 m(2) were prospectively enrolled. Twenty-two two-dimensional and M-mode measurements of atrial and ventricular chamber diameters and areas were performed. Models using linear, logarithmic, exponential, and square-root relationships were tested. Heteroscedasticity was tested by the White test and the Breusch-Pagan test. Age, weight, height, and BSA, calculated by the Haycock formula, were used as the independent variables in different analyses to predict the mean value of each echocardiographic measurement. The influence of various confounders, including gender, type of delivery, prematurity, and interobserver variability, was also evaluated. Structured Z scores were then computed. RESULTS: The Haycock formula provided the best fit and was used when presenting data as predicted values (mean ± 2 SDs) for a given BSA and within equations relating echocardiographic measurements to BSA. Confounders were not included in the final models, because they did not show significant effects for most of the measurements. CONCLUSIONS: Echocardiographic reference values are presented for chamber area and diameters, derived from a large population of healthy children. These data partly cover a gap in actual pediatric echocardiographic nomograms. Further studies are required to reinforce these data, as well as to evaluate other parameters and ethnicities.

Controversies in the prophylaxis and treatment of postsurgical pericardial syndromes: a critical review with a special emphasis on paediatric age.

Postsurgical pericardial syndromes are common complications after cardiac surgery; however, their treatment is not well established yet. We reviewed the accuracy and limits of clinical trials of prophylaxis and treatment of these diseases to identify an evidence-based therapeutic approach. We performed a literature search in the National Library of Medicine using the keywords pericardial effusion, cardiac surgery and paediatric/congenital. The research was then redefined adding separately the keywords postpericardiotomy syndrome, NSAIDs, steroids and colchicine. We found 12 clinical trials (eight for the prophylaxis and four regarding treatment), testing three major agent classes: NSAIDs, corticosteroids and colchicine. Therapy is generally based on NSAID with or without steroids with the adjunct of colchicine for recurrences. Only a few randomized controlled trials (RCTs) in adults support NSAID therapy. Efficacy of steroids has been proved only in small paediatric works, whereas no studies are available for colchicine. Studies furthermore presented some limitations: not univocal endpoints (not allowing for a meta-analysis), a limited sample size, scarce attention to confounders (such as the underlying cardiac disease and diuretic/analgesic regimen). Moreover, different agents were not assessed, nor when to start therapy. More evidence (two wide RCT plus a meta-analysis) supports the role of colchicine for prophylaxis in adults. Prophylaxis with NSAID/corticosteroids instead failed to have significant advantage in children, whereas a few data are available for adults. Evidence for the treatment of postsurgical pericardial syndromes is incomplete, making it difficult to understand when to treat and which agent to employ, especially in children.

Echocardiographic nomograms for ventricular, valvular and arterial dimensions in caucasian children with a special focus on neonates, infants and toddlers.

BACKGROUND: A quantitative echocardiographic assessment is often essential for the management of congenital heart disease, especially in the first months of life. Despite this, pediatric echocardiographic nomograms are limited and heterogeneous, particularly for neonates and infants. The aim of this study was to establish reliable echocardiographic nomograms in a broad population of healthy Caucasian children. METHODS: Two-dimensional and M-mode measurements of 22 cardiovascular structures were performed. Models using linear, logarithmic, exponential, and square root relationships were tested. Heteroscedasticity was tested using the White and Breusch-Pagan tests. Age, weight, height, and body surface area (BSA; calculated using seven different formulas) were used as the independent variables in different analyses to predict the mean value of each echocardiographic measurement. Structured Z scores were then computed. RESULTS: A total of 445 consecutive Caucasian Italian healthy subjects (age range, 0 days to 36 months; 49% female subjects) with BSAs ranging from 0.12 to 0.67 m(2) were prospectively enrolled. The calculation of BSA using the Haycock formula provided the best results, while other formulas either underestimated (DuBois, Mosteller, Dreyer, and Meban) or overestimated (Boyd and Gehan) BSA. The Haycock formula has been used when presenting data as predicted values (mean ± 2 SDs) for a given BSA and within equations relating echocardiographic measurements to BSA. For all the measurements, there was no significant intraobserver or interobserver variability. CONCLUSIONS: The investigators report new, reliable echocardiographic Z scores derived from a large population of Caucasian neonates, infants, and toddlers calculated using a rigorous statistical design. These nomograms represent a valid diagnostic tool for echocardiographic quantification in this age group.

Residual shunt after patent foramen ovale closure: preliminary results from Italian patent foramen ovale survey.

BACKGROUND: Percutaneous patent foramen ovale (PFO) closure is accepted as treatment for cryptogenic ischemic stroke/transient ischemic attack in young subjects. However, a thorough evaluation of residual right-to-left shunt (rRLS) after PFO closure is needed. Our aims were to analyze the characteristics related to PFO diagnosis and closure, focusing on rRLS and clinical recurrences until 24-month follow-up. Data were extrapolated from the 12-month Italian PFO Survey. METHODS: In all, 1035 patients were included. PFO diagnosis and right-to-left shunt (RLS) were assessed by contrast-enhanced transesophageal and/or transthoracic echocardiography and/or transcranial Doppler. RESULTS: PFO diagnosis with RLS data were available in 894 of 1035 (86.4%) patients. rRLS was investigated in 49.6% (6 months), 27.1% (12 months), and 3.5% (24 months), and observed in 19.5% (6 months) and 18.2% (12 months) of subjects. Large permanent rRLS was observed in less than 3% of RLS-positive patients after 1 year. Eleven of 14 and 3 of 14 neurological recurrences were observed in 10 of 444 (2.25%) and 2 of 243 (0.8%) patients within the 6- and 12-month follow-up, respectively. Among these, no large rRLS was reported. There were no neurological events at 2-year follow-up. Forty of 444 subjects had non-neurological complications, mostly cardiac arrhythmias within the sixth month. CONCLUSIONS: PFO closure is a safe procedure. rRLS is not uncommon but large rRLS is rare. Clinical complications, mostly related to cardiac arrhythmias, are not unusual. Evaluation of the data of the whole survey is underway.

Melody transcatheter pulmonary valve implantation. Results from the registry of the Italian Society of Pediatric Cardiology.

BACKGROUND AND AIMS: Percutaneous implantation of pulmonary valve has been recently introduced in the clinical practice. Our aim was to analyze data of patients treated in Italy by using the Melody Medtronic valve. METHODS: Prospective, observational, multi-centric survey by means of a web-based database registry of the Italian Society of Pediatric Cardiology (SICP). RESULTS: Between October 2007 and October 2010, 63 patients were included in the registry (median age: 24 years; range 11-65 years). Forty subjects were in NYHA class I-II while 23 were in NYHA class III-IV. Patients included had a history of a median three previous surgeries (range 1-5) and a median of one previous cardiac catheterization (range 0-4). A cono-truncal disease was present in 39 patients, previous Ross operation in 9, and other diagnosis in 15. Indication to valve implantation was pure stenosis in 21 patients, pure regurgitation in 12, association of stenosis and regurgitation in 30. Implantation was performed in 61 subjects (97%). Pre-stenting was performed in 85% of cases. Median procedure time was 170 minutes (range 85-360). No significant regurgitation was recorded after procedure while the trans-pulmonary gradient reduced significantly. Early major complications occurred in seven subjects (11%). One death occurred in the early post-operative period in a severely ill subject. At a median follow-up of 30 months (range 12-48 months), three patients died due to underlying disease. Major complications occurred in six patients during follow-up (external electric cardioversion: one patient; herpes virus encephalitis: two patients; Melody valve endocarditis needing surgical explant: two patients; major fractures of the stent and need second Melody valve implantation: two patients). Freedom from valve failure at latest follow-up was 81.4% ± 9%. CONCLUSION: Early results of the SICP registry on transcatheter Melody pulmonary valve implantation show that the procedure is safe and successful. Major concerns are related to the occurrence of stent fracture and bacterial endocarditis. Longer follow-up and larger series are needed.