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Purpose:Transposition of great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is uncommon. We reviewed operations performed in patients with TGA + IVS + LVOTO in the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD).Methods:All 109 patients with a diagnosis of TGA + IVS + LVOTO in ECHSA-CD who underwent cardiac surgery during a 21-year period (01/2000-02/2021, inclusive) were included. Preoperative variables, operative data, and postoperative outcomes were collected.Results:These 109 patients underwent 176 operations, including 37 (21.0%) arterial switch operations (ASO), 26 (14.2%) modified Blalock-Taussig-Thomas shunts (MBTTS), 11 (6.2%) Rastelli operations, and 13 (7.3%) other palliative operations (8 superior cavopulmonary anastomosis[es], 4 Fontan, and 1 other palliative procedure). Of 37 patients undergoing ASO, 22 had a concomitant procedure.There were 68 (38.6%) reoperations, including 11 pacemaker procedures and 8 conduit operations. After a systemic-to-pulmonary artery shunt, reoperations included shunt reoperation (n = 4), Rastelli (n = 4), and superior cavopulmonary anastomosis (n = 3).Overall Operative Mortality was 8.2% (9 deaths), including three following ASO, two following “Nikaidoh, Kawashima, or LV-PA conduit” procedures, and two following Rastelli. Postoperative complications occurred after 36 operations (20.4%). The most common complications were delayed sternal closure (n = 11), postoperative respiratory insufficiency requiring mechanical ventilation >7 days (n = 9), and renal failure requiring temporary dialysis (n = 8).Conclusion:TGA + IVS + LVOTO is rare (109 patients in ECHSA-CD over 21 years). ASO, MBTTS, and Rastelli are the most common operations performed for TGA + IVS + LVOTO. Larger international studies with long-term follow-up are needed to better define the anatomy of the LVOTO and to determine the optimal surgical strategy.

A 6-month-old baby girl, with a history of 2 months of intensive care management and two episodes of cardiac arrest, was transferred from another European country to initiate the “Giessen approach” for end-stage heart failure in children. At the admission, left ventricular ejection fraction was 20%. Severe mitral valve regurgitation and severe left atrial dilatation were present. Right ventricular function was preserved, and tricuspid valve regurgitation was mild. As a result, the patient underwent surgical pulmonary artery banding. Additionally, unloading of the left atrium was achieved by implanting an 8 mm atrial flow regulator device through a hybrid per-atrial approach. Two months after the procedure, the patient was progressively weaned from the inotropes and transferred to the ward.

For decades, bovine jugular vein conduits (BJV) and classic cryopreserved homografts have been the two most widely used options for pulmonary valve replacement (PVR) in congenital heart disease. More recently, decellularized pulmonary homografts (DPH) have provided an alternative avenue for PVR. Matched comparison of patients who received DPH for PVR with patients who received bovine jugular vein conduits (BJV) considering patient age group, type of heart defect, and previous procedures. 319 DPH patients were matched to 319 BJV patients; the mean age of BJV patients was 15.3 (SD 9.5) years versus 19.1 (12.4) years in DPH patients (p = 0.001). The mean conduit diameter was 24.5 (3.5) mm for DPH and 20.3 (2.5) mm for BJV (p p = 0.45). The rate of freedom from endocarditis was significantly lower for BJV patients (87.1 vs. 96.5%, p = 0.006). Freedom from explantation was significantly lower for BJV at 10 years (81.7 vs. 95.5%, p = 0.001) as well as freedom from any significant degeneration at 10 years (39.6 vs. 65.4%, p p = n.s.). DPH showed 20% higher freedom from explantation and degeneration in this subgroup (p = 0.232). Decellularized pulmonary homografts exhibit superior 10-year results to bovine jugular vein conduits in PVR.

Objectives: The surgical technique for right partial anomalous pulmonary venous return (PAPVR) depends on the location of the anomalous pulmonary veins (PVs). With this in mind, we sought to evaluate the impact of 3D heart segmentation and reconstruction on preoperative surgical planning. Methods: A retrospective study was conducted on all patients who underwent PAPVR repair at our institution between January 2018 and October 2021; three-dimensional segmentations and reconstructions of all the heart anatomies were performed. A score (the PAPVR score) was established and calculated using two anatomical parameters (the distance between the most cranial anomalous PV and the superior rim of the sinus venosus defect/the sum of the latter and the distance between the PV and the azygos vein) to predict the type of correction. Results: A total of 30 patients were included in the study. The PAPVR score was found to be a good predictor of the type of surgery performed. A value > 0.68 was significantly associated with a Warden procedure (p < 0.001) versus single/double patch repair. Conclusions: Three-dimensional heart segmentations and reconstructions improve the quality of surgical planning in the case of PAPVR and allow for the introduction of a score that may facilitate surgical decisions on the type of repair required.

OBJECTIVES Early results from the prospective ESPOIR Trial have indicated excellent results for pulmonary valve replacement using decellularized pulmonary homografts (DPH). METHODS A 5-year analysis of ESPOIR Trial patients was performed to provide an insight into the midterm DPH performance. ESPOIR Trial and Registry patients were matched with cryopreserved homografts (CH) patients considering patient age, type of heart defect and previous procedures to present the overall experience with DPH. RESULTS A total of 121 patients (59 female) were prospectively enrolled (8/2014–12/2016), median age 16.5 years (interquartile range 11.2–29.8), and median DPH diameter 24 mm. One death (73 year-old) occurred during a median follow-up of 5.9 years (5.4–6.4), in addition to 2 perioperative deaths resulting in an overall mortality rate of 2.5%. One case of endocarditis in 637 patient-years was noticed, resulting in an incidence of 0.15% per patient-year. At 5 years, the mean peak gradient was 19.9 mmHg (9.9), mean regurgitation 0.9 (0.6, grade 0–3) and freedom from explantation/any reintervention 97.5% (1.5). The combined DPH cohort, n = 319, comprising both Trial and Registry data, showed significantly better freedom from explantation for DPH 95.5% (standard deviation 1.7) than CH 83.0% (2.8) (P CONCLUSIONS The 5-year data of the prospective ESPOIR Trial show excellent performance for DPH and low rates of adverse events. ESPOIR Registry data up to 15 years, including a matched comparison with CH, demonstrated statistically significant better freedom from explantation.

Introduction ExtraCorporeal Membrane Oxygenation (ECMO) in pediatric patients with COVID-19 has a survival rate similar to adults. Occasionally, patients may need to be cannulated by an ECMO team in a referring hospital and transported to an ECMO center. The ECMO transport of a COVID-19 patient has additional risks than normal pediatric ECMO transport for the possible COVID-19 transmissibility to the ECMO team and the reduction of the ECMO team performance due to the need of wearing full personal protective equipment. Since pediatric data on ECMO transport of COVID-19 patients are lacking, we explored the outcomes of the pediatric COVID-19 ECMO transports collected in the EuroECMO COVID_Neo/Ped Survey. Methods We reported five European consecutive ECMO transports of COVID-19 pediatric patients collected in the EuroECMO COVID_Neo/Ped Survey including 52 European neonatal and/or pediatric ECMO centers and endorsed by the EuroELSO from March 2020 till September 2021. Results The ECMO transports were performed for two indications, pediatric ARDS and myocarditis associated to the multisystem inflammatory syndrome related to COVID-19. Cannulation strategies differed among patients according to the age of the patients, transport distance varied between 8 and 390 km with a total transport duration between 5 to 15 h. In all five cases, the ECMO transports were successfully performed without major adverse events. One patient reported a harlequin syndrome and another patient a cannula displacement both without major clinical consequences. Hospital survival was 60% with one patient reporting neurological sequelae. No ECMO team member developed COVID-19 symptoms after the transport. Conclusion Five transports of pediatric patients with COVID-19 supported with ECMO were reported in the EuroECMO COVID_Neo/Ped Survey. All transports were performed by an experienced multidisciplinary ECMO team and were feasible and safe for both the patient and the ECMO team. Further experiences are needed to better characterize these transports and draw insightful conclusions.

Background: The major limitation to the Ross operation is a progressive autograft dilation, possibly leading to reoperations. A murine model was created to evaluate pulmonary artery graft (PAG) adaptation to pressure overload. Methods: Lewis rats (n = 17) underwent heterotopic surgical implantation of a PAG, harvested from syngeneic animals (n = 17). A group of sham animals (n = 7) was used as a control. Seriated ultrasound studies of the PAG were performed. Animals were sacrificed at 1 week (n = 5) or 2 months (n = 15) and the PAG underwent mechanical and histopathological analyses. Results: Echography showed an initial increase in diameter (p < 0.001) and a decrease in peak systolic velocity (PSV). Subsequently, despite no change in diameter, an increase in PSV was observed (p < 0.01). After 1 week, the stiffness of the PAG and the aorta were similar, while at 2 months, the PAG appeared more rigid (p < 0.05). PAG’s histological analysis at 2 months revealed intimal hyperplasia development. The tunica media showed focal thinning of the elastic lamellae and normally distributed smooth muscle cells. Conclusions: We demonstrated a stiffening of the PAG wall after its implantation in systemic position; the development of intimal hyperplasia and the thinning of the elastic lamellae could be the possible underlying mechanism.

Objective: To propose and evaluate an adapted NYHA classification for children with congenital heart disease (CHD) as a feasible clinical tool for classifying patients’ fitness, cardiorespiratory efficiency and functional limitations during their ordinary daily activities, which are also characterized by vigorous and competitive physical exercise among peers. Methods: This cross-sectional investigation analyzed 332 patients (13.1 ± 3.01 y/o) who underwent surgical repair of CHD and performed Cardiopulmonary Exercise Testing (CPET). Patients were divided into NYHA class I, IIA and IIB by specific questioning regarding functional limitation and performance compared to peers and at strenuous intensity. Class IIA was characterized by slight exercise limitation only for strenuous/competitive activities, whereas IIB for already ordinary physical activities. These NYHA classes were compared with maximal CPET on treadmill. Results: Patients’ exercise capacity (exercise time, METs), aerobic capacity (VO2peak) and chronotropic response were found progressively impaired when NYHA class I was compared with IIA and IIB. Indeed, ventilatory-perfusion mismatch (PETCO2, VE/VCO2) significantly worsened from NYHA class I to IIA, while no difference was found between IIA and IIB. Conclusion: This adapted NYHA-CHD classification could allow regular functional evaluations and accurate assessments by clinicians, leading to facilitated clinical management and timely medical interventions.

OBJECTIVES Many centres have recently adopted pulmonary valve (PV) preservation (PVP) during tetralogy of Fallot (ToF) repair. We sought to identify the midterm functional outcomes and risk factors for pulmonary regurgitation after this procedure. METHODS All patients undergoing PVP during transatrial–transpulmonary repair for ToF with PV stenosis at our institution between January 2007 and December 2020 were reviewed. RESULTS Overall, 73 patients were included. At the index surgery, the body surface area was 0.31 ± 0.04 m2, the age was 4.9 ± 2.9 months and the preoperative PV z-score was -3.02 ± 1.11. At a mean follow-up of 5.3 ± 2.7 years, the fractional area change of the right ventricle (RV) was 47.1 ± 5.2%, and the tricuspid annular plane systolic excursion z-score was -3.31 ± 1.89%. The 5-year freedom from moderate/severe PV regurgitation was 61.3% [95% confidence interval (CI): 48, 73%]. There was a significant correlation between RV function and moderate/severe PR at follow-up (R2: 0.08; P = 0.03). A comparison with a group of patients undergoing a transannular patch procedure (N = 33) showed superior outcomes for patients with PVP. The preoperative PV z-score and the degree of PR at discharge were risk factors for the early development of moderate/severe PR at follow-up [hazard ratio (HR): 0.64; 95% CI: 0.48, 0.86, P = 0.01 and HR: 2.31; 95% CI: 1.00, 5.36, P = 0.04, respectively]. A preoperative PV annulus z-score ≤ -2.85 was found to be predictive for moderate/severe PR at 5 years after PVP (HR: 2.56; 95% CI: 1.31, 5.01, P = 0.002). CONCLUSIONS A pulmonary valve preservation strategy during tetralogy of Fallot repair should always be attempted. However, a preoperative PV annulus z-score < -2.85 and moderate/severe regurgitation upon discharge are risk factors for midterm pulmonary regurgitation.

The Ross operation for aortic valve disease has regained new interest due to its outstanding long-term results. Nonetheless, when employed as freestanding root replacement, the possible dilation of the pulmonary autograft and subsequent aortic regurgitation is described. Several animal models have been proposed. However, these are usually limited to ex-vivo models or in-vivo experiments with relatively expensive large animal models. In this study, we sought to establish a rodent model of pulmonary artery graft (PAG) implantation in a systemic position. A total of 39 adult Lewis rats were included. Immediately after euthanasia, the pulmonary root was harvested from a donor animal (n=17). Syngeneic recipient (n=17) and sham-operated (n=5) rats were sedated and ventilated. In the recipient group, the PAG was implanted with an end-to-end anastomosis in infra-renal abdominal aortic position. Sham-operated rats underwent only transection and re-anastomosis of the aorta. Animals were followed with serial ultrasound studies for two months and post-mortem histological analysis. The median PAG diameter in the native position was 3.20 mm (IQR=3.18-3.23). At follow-up, the median diameter of the PAG was 4.03 mm (IQR=3.74-4.13) at 1 week, 4.07 mm (IQR=3.80-4.28) at 1 month, and 4.27 mm (IQR=3.90-4.35) at 2 months (p0.01). Peak systolic velocity was 220.07 mm/s (IQR=210.43-246.41) at 1 week, 430.88 mm/s (IQR=375.28-495.56) at 1 month, and 373.68 mm/s (IQR=305.78-429.81) at 2 months (p=0.02) and did not differ from the sham-operated group at the end of the experiment (p=0.5). Histological analysis did not show any sign of endothelial thrombosis. This study showed that rodent models may allow for the evaluation of the long-term adaptation of the pulmonary root to a high-pressure system. A systemically placed syngeneic PAG implantation represents a simple and feasible platform for the development and evaluation of novel surgical techniques and drug therapies to further improve the outcomes of the Ross operation.

OBJECTIVES The aim of this study was to evaluate if a ‘protective’ (low-tidal/low-frequency) ventilation strategy can shorten the postoperative ventilation time and minimize acute lung injury in children with congenital heart disease (CHD) undergoing repair with cardiopulmonary bypass (CPB). METHODS This is a single-centre prospective, interventional study, including children with CHD under the age of 5 years, undergoing open-heart surgery with a CPB >60 min, in hypothermia, haemodynamically stable, and without evident genetic abnormalities. Assist-control ventilation (tidal volume of 4 ml/kg, 10 breaths/min, positive end-expiratory pressure 5 cmH2O and FiO2 0.21) was applied in a cohort of patients during CPB. We compared clinical outcomes and in fully ventilated versus non-ventilated (control) patients. Propensity score was used to weigh ventilated and control groups to correct for the effect of other confounding clinical variables. Clinical and ventilation parameters and lung inflammatory biomarkers in tracheal aspirates were measured. The primary outcome was the postoperative intubation time of more or less than 48 h. RESULTS We included 140 children (53 ventilated, 87 non-ventilated) with different CHD. There were no deaths or adverse events in ventilated patients. Using a weighted generalized linear model, we found no sufficient evidence for an effect of intraoperative ventilation on postoperative intubation time [estimate 0.13 (95% confidence interval, –0.08; 0.35), P = 0.22]. CONCLUSIONS Continuous low-tidal/low-frequency mechanical ventilation during CPB is safe and harmless. However, no significant advantages were found when compared to non-ventilated patients in terms of postoperative ventilation time.

OBJECTIVES A conclusive interpretation of the role of ventricular dominance in outcomes after Fontan palliation has not been formulated yet. We conducted a systematic review and meta-analysis of scientific literature to give an insight into the impact of ventricular morphology in single-ventricle palliation, focusing on its influence on survival, morbidities, ventricular performance and functional capacity. METHODS A systematic review of PubMed, Web of Science and Scopus databases was performed. A random-effect meta-analysis was conducted, and survival data were reconstructed using the published Kaplan–Meier survival curves. RESULTS Twenty-seven studies were selected, for a total of 4529 left-dominant versus 4844 right-dominant patients. Estimated survival at 1, 5, 10, 20 and 30 years of follow-up was 0.99 [95% confidence interval (CI) = 0.98–0.99], 0.95 [95% CI = 0.94–0.96], 0.92 [95% CI = 0.91–0.93], 0.86 [95% CI = 0.84–0.88] and 0.68 [95% CI = 0.65–0.83] for left-dominant patients and 0.94 [95% CI = 0.93–0.95], 0.89 [95% CI = 0.88–0.9], 0.85 [95% CI = 0.83–0.87], 0.69 [95% CI = 0.63–0.75] and 0.59 [95% CI = 0.5–0.69] for right-dominant patients, respectively. Survival was statistically lower for right-dominant patients (P CONCLUSIONS According to our meta-analysis, the morphology of the dominant ventricle has a significant impact on outcomes after Fontan palliation. Right-dominant patients experience an inferior long-term survival when the anatomical right ventricle is included in the systemic circulation.

Pediatric heart failure (HF) is an important clinical condition with high morbidity and mortality. Compared to adults, pediatric HF shows different etiologies characterized by different physiology, a different clinical course, and deeply different therapeutic approaches. In the last few years, new drugs have been developed and new therapeutic strategies have been proposed with the goal of identifying an individualized treatment regimen. The aim of this article is to review the new potential drugs and non-pharmacological therapies for pediatric heart failure in children.

Aims Autoimmune congenital heart block (CHB) is a severe manifestation of neonatal lupus syndrome. There is lack of consensus regarding treatment of pregnant women with anti-SSA/SSB autoantibodies. To evaluate the effectiveness of the combined protocol therapy (oral steroid, plasmapheresis, and IVIG) for the CHB treatment. Methods and results All cases of CHB from 2000 to 2020 were retrospectively enrolled. All the patients underwent foetal echocardiography for the evaluation of CHB, ventricular rate and main related foetal complications: cardiomegaly, pericardial and pleural effusion, foetal hydrops, dilated cardiomyopathy, and heart failure. Moreover, postnatal major adverse cardiovascular, such as death and pacemaker implantation, were recorded. For statistical analysis, the population was divided into two cohorts: a protocol group receiving in utero combined therapy and a control group receiving other therapies or not treated. Among 252 pregnancies with anti-SSA/SSB antibodies, 36 developed CHB. At birth, complete CHB treated with protocol therapies showed a significantly higher ventricular rate (P = 0.042), a significant reduction in intrauterine or postnatal mortality (P = 0.018), and a lower rate of pacemaker implantation (P = 0.049). Conclusions The combined treatment protocol has proven effective in improving foetal and neonatal short- and long-term survival.

Aims To describe the current application of exercise stress echocardiography (ESE) in our Paediatric Cardiology Departments. Methods and results Baseline and under stress symptoms, vital parameters, ECG, and echocardiograms from patients who underwent ESE in our centres, were retrospectively analysed and compared, as well as clinical management plans formulated based on ESEs results. Forty-five patients from Centre 1 (median age 16 years), including 87% of patients with congenital heart diseases (CHDs), and 20 patients from Centre 2 (median age 11 years), mainly tested to rule out myocardial ischemia, were included. Among patients from Centre 1, 28 had previously been treated surgically, 6 percutaneously, and 11 were under follow-up. Indications for ESE/patients’ native diagnosis are illustrated in the picture. Centre 1: Exercise was maximal in 17 patients, with 2 of them having symptoms at the peak of exercise. It was stopped beforehand in 28 patients because of dyspnoea (3) and muscle fatigue (25). No arrhythmia was detected. ESE was considered as positive in 14 patients; after that, 3 patients underwent percutaneous interventions, 2 underwent cardiac surgery, 3 received indication for cardiac catheterization, 4 for advanced cardiac imaging, and 2 for exercise restriction and medical therapy. Centre 2: 15 patients (75%) completed the exercise, and none of them developed symptoms. In the remaining 25%, exercise was stopped because of muscle exhaustion. No arrhythmia was detected, and all the ESEs were negative. Comparing the cohorts, no differences in terms of ejection fraction were noticed at rest and under stress. Patients in cohort 1 were older (P = 0.002), they achieved lower average maximal heart rate (P = 0.0001), performed less lasting exercise (P = 0.05), and ESE was maximal less frequently (P = 0.005). Lastly, they had significant changes in clinical decisions (P = 0.004). Conclusions In paediatric cardiology, particularly in children with CHDs, ESE is a promising technique and could influence significantly clinical management plans.

Background: Extracorporeal membrane oxygenation (ECMO) implantation for neonates with severe cardiorespiratory life-threatening conditions is highly effective. However, since ECMO is a high-risk and complex therapy, this treatment is usually performed in centers with proven expertise. Methods: A retrospective review of neonates, from January 2014 to January 2020, presenting with life-threatening conditions and treated by means of Hub and Spoke (HandS) ECMO in peripheral (spoke) hospitals. Data were retrieved from our internal ECMO registry. Protocols and checklists were revised and shared with all spoke hospitals located in North-Eastern Italy. Results: Eleven neonates receiving maximal respiratory and cardiovascular support at a spoke hospital underwent HandS ECMO management. All but three patients were affected by life-threatening meconium aspiration syndrome (MAS). The median ECMO support duration and hospitalization were four (range 2&ndash, 32) and 30 days (range 8&ndash, 50), respectively. All but two patients (with congenital diaphragmatic hernia), were weaned off ECMO and discharged home. At a mean follow up of 33.7 &plusmn, 29.2 months, all survivors were alive and well, without medications, and normal somatic growth. All but one had normal neuropsychological development. Conclusion: HandS ECMO model for neonates with life-threatening conditions is effective and successful. A specialized multidisciplinary team and close cooperation between Hub and Spoke centers are essential for success.

The neonatal Hub and Spoke (HandS) ECMO approach provides emergent ECMO implantation to support neonates in severe cardio-respiratory life-threatening conditions at secondary (Spoke) centers, and a safe transfer to the ECMO center (Hub). We report a retrospective review of 11 neonates fom January 2014 to January 2020, presenting with life-threatening conditions and treated by HandS ECMO team in Spoke hospitals. Protocols and checklists were arranged by a local group of ECMO experts and shared with all the hospitals located in North-Eastern Italy. At the emergent call, all patients were cared for at the Spoke hospitals, receiving maximal respiratory and cardiovascular support (including high frequency oscillatory ventilation, inhaled nitric oxide, and major inotropic intravenous infusion). All but 3 patients were affected by life-threatening meconium aspiration syndrome. Median duration of ECMO support and hospitalization were 4 days (range 2-32) and 30 days ( range 8-50), respectively. All but 2 patients with congenital diaphragmatic hernia, were weaned off ECMO and discharged home without complications. At a median follow up of 14.4 months (1.3-74.8), all survivors were alive and in good clinical conditions, without medications, and normal somatic growth. All but one had normal neuropsychological development. We conclude that HandS ECMO model for treating neonates in life-threatening conditions is effective and successful. A specialized multidisciplinary team and common organizational protocols shared between Hub and Spoke centers are the key-points for success. While excellent results occur with MAS (100% survival), treatment was not successful in CDH, in which other that HandS ECMO issues are implied. Lessons learned from our experience are presented and discussed in this article.

Troponin-I (Tn-I) is a well-recognized early postoperative marker for myocardial damage in adults and children. The present prospective study was undertaken to investigate whether a postoperative Tn-I value higher than 35 microg/l is able to predict long-term outcome as it does in early postoperative course, after surgery for congenital heart defects (CHD).Five hundred and twenty patients (median age 11 months; male 54.7%: 284 patients) undergoing congenital heart defect repair on cardiopulmonary by-pass were prospectively updated in our database including postoperative Tn-I values. Seventy of them (13.4%) (mean age 2.6+/-5.8 months) (70/520) experienced low output syndrome in the early postoperative period. According to the complexity of their malformations, we have arbitrarily divided these patients into two groups: group A included atrial and ventricular septal defects (13 patients), while group B included hypoplastic left heart syndrome, atrio-ventricular canal, transposition of great vessels, tetralogy of Fallot, double outlet right ventricle, truncus arteriosus, total anomalous venous return, and other combined diseases (57 patients). These patients are the object of our study. We reviewed clinical, laboratory, and echocardiographic data performed in the immediate postoperative course (within 24 h) and in the follow-up.In this study, 13 patients died (13/70 patients; 18.5%), 12 in group B and 1 in group A. In deceased patients, mean Tn-I value was 130+/-175 microg/l (CK-MB 570+/-280 microg/l). Conversely, survivors showed a lower mean Tn-I value (25.5+/-28.9 microg/l; CK-MB 76+/-86 microg/l). Overall, Tn-I peak value was higher than 35 microg/l in 19 patients (19/70; 27.2%); among these, 9 died (median Tn-I was 163+/-186 microg/l), whereas in survivors it was 73.4+/-37 microg/l (p=0.37). The remaining four patients who died had a median Tn-I value of 21 microg/l. When Tn-I exceeded 35 microg/l (100 microg/l in two cases), at echocardiogram a severely depressed cardiac function was evident. Nevertheless, at long-term follow-up (12+/-6 months), the echocardiogram showed an enhanced cardiac performance with an ejection fraction of 70+/-8.5% in all; none of these patients presented with worsened ventricular function.Cardiac Tn-I is a specific and sensitive marker of myocardial injury after cardiac surgery and it may predict early in-hospital outcomes. However, by long-term echocardiographic analysis, cardiac Tn-I value looses its prognostic significance and therefore it is not a predictor of long-term ventricular dysfunction.

Tetralogy of Fallot is a common complex congenital heart disease, nowadays amenable to safe surgical repair with good early and long-term outcomes. Notwithstanding this, surgical repair is not a definite treatment, and the heart of patients with tetralogy of Fallot remains anatomically, physiologically and electrically abnormal. Indeed, long-term survival of patients with repaired tetralogy of Fallot differs from that of the general population. Major long-term complications include supraventricular and ventricular arrhythmias, with risk of sudden cardiac death, and chronic pulmonary regurgitation, which gradually leads to right ventricular dilation and dysfunction. Thus, the primary aims of clinical follow-up for adult patients with repaired tetralogy of Fallot should be to assess ventricular morphology and function, to stratify the arrhythmic risk and to define the optimal surgical or interventional timing.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease (CHD), whose surgical repair is associated with high mortality and reoperation rates. We sought to identify predictors of early and late outcomes.Data from medical records of patients who underwent surgical repair for TAPVC from 1989 to 2012 were included. The patients were divided in two groups, according to absence or presence of associated major CHDs.Forty-six patients were included (M/F: 26/20, median age 26 days, interquartile range 15 to 59, median weight 3.350 kg, interquartile range 1800 to 4470). Anatomic types of TAPVC were: supracardiac in 48%, intracardiac in 20%, infracardiac in 20%, and mixed in 12%; TAPVC was obstructive in 33%; TAPVC was isolated in 63%, complex in 37%. Single ventricle physiology was present in 11 patients, heterotaxy in eight patients. Overall operative mortality was 19.6% (9/46): 6.9% in isolated TAPVC, 41.2% in complex type (p-value: 0.002). It was associated with low weight at intervention (3 kg, p = 0.027), single ventricle physiology (p = 0.047), and aortic cross-clamp time 60 minutes (p = 0.097). At a median follow-up of 2.97 years (range 43 days to 22 years, 91% complete), there were nine late deaths (24.3%); 15 patients (40.5%) had major events (including late death). Multivariate analysis of event-free survival showed worse outcome in the complex group (p = 0.001).Surgical mortality and morbidity remain consistent in patients with TAPVC and associated major CHD, while the isolated type shows excellent outcomes. Pulmonary vein obstruction is a rare but highly lethal complication. doi: 10.1111/jocs.12399 (J Card Surg 2014;29:678-685).

Balloon aortic valvuloplasty has become in many centers the treatment of choice for neonates with critical or severe aortic stenosis. Usual approaches both antegrade and retrograde can be problematic in preterms extremely low birth weight babies. We describe a novel approach for dilating the aortic valve in an 890 grams baby.