BACKGROUND AND AIMS:The Fontan operation and its modifications have transformed the management of children with functional single ventricle physiology. While outcomes have improved, peri-operative complications remain common. This study aimed to assess early outcomes and complications following Fontan completion in a large international European cohort. METHODS:The EuroFontan registry is a multicentre retrospective study involving 21 congenital heart disease and transplant centres across Europe. Data were collected on patients who underwent Fontan surgery from January 1990 to January 2023. Analyses focused on the most recent 15-year period. A composite early adverse outcome (death within 30 days or in-hospital, low cardiac output syndrome, stroke, or Fontan takedown) was used. Predictors were assessed using multivariable mixed-effects logistic regression, with centre included as a random effect. Missing data were handled using multiple imputation with Rubin's rules, and sensitivity analyses were performed to assess robustness. RESULTS:Of 3510 patients, this analysis focused on 2075 individuals from the past 15 years, with a median age of 4.2 [interquartile range 3.2-6.2] years at Fontan completion. Early mortality was 1.0% (95% confidence interval .66%-1.54%). The composite adverse outcome occurred in 5.1% (95% confidence interval 4.2%-6.1%). On multivariable analysis, morphology other than tricuspid atresia was the only significant predictor of the composite outcome (odds ratio 2.2, 95% confidence interval 1.21-3.99, P = .01). CONCLUSIONS:Fontan surgery has evolved and, in contemporary practice, has low peri-operative morbidity and mortality. Morphology other than tricuspid atresia was associated with increased risk of early adverse outcomes, highlighting the importance of pre-operative risk stratification.

Neurological injury remains a major concern during neonatal aortic arch surgery. Near-infrared spectroscopy (NIRS) is commonly used to monitor cerebral oxygenation but does not provide direct quantitative information on cerebral blood flow. Transit-time flow measurement (TTFM) allows real-time volumetric flow assessment and may represent an additional tool for intraoperative monitoring of cerebral perfusion. We report the intraoperative use of TTFM for continuous monitoring of cerebral inflow during the repair of an interrupted aortic arch in a neonate. A perivascular flow probe was placed around the left common carotid artery to allow real-time measurement of carotid flow during cardiopulmonary bypass and selective cerebro-myocardial perfusion. Baseline flow in the brachiocephalic trunk was approximately 87 ml/min. During selective cerebro-myocardial perfusion, carotid flow remained stable at 45-50 ml/min. After aortic clamping, the carotid waveform changed from pulsatile to continuous, with a reduction in pulsatility index from 1.5 to 0.6. Flow values remained stable throughout arch reconstruction and were consistent with arterial pressure and NIRS monitoring. Transit-time flow measurement enabled direct real-time assessment of cerebral inflow during neonatal arch repair and provided quantitative information complementary to conventional monitoring. This technique may represent a useful adjunct for evaluating cerebral perfusion during complex neonatal cardiac surgery.

The arterial switch operation (ASO) is the standard surgical intervention to correct transposition of the great arteries (TGA). The aim of this study is to describe long-term outcomes after the ASO in patients with TGA, and to explore anatomical and clinical factors potentially associated with adverse outcomes, comparing simple and complex TGA. Since December 2001 to October 2023, 233 patients underwent arterial switch operation (158 simple TGA and 75 complex TGA). Kaplan–Meier analysis assessed survival and reoperation risk, Chi-square test and T-student test have been performed in order to analyse pre-, intra- and post-operative data among the two groups. In-hospital mortality was 1.7%. Overall survival rates were 97.9%, 96.8% and 96.8% at 5, 10 and 15 years, respectively. Freedom from surgical reoperation at 15 years was 98.3% in simple TGA and 91% in complex TGA at 15 years ( p = 0.039). Reinterventions were infrequent and mainly related to pacemaker implantation and right ventricular outflow tract obstruction. Coronary artery stenosis occurred in 5 patients (2.2%). Risk factors analysis suggested that anatomical complexity such as intramural coronary artery ( p = 0.003), aortic arch hypoplasia ( p = 0.004), aortic arch interruption ( p = < 0.001) and coronary reimplantation with button technique ( p = 0.018) are predictive factors for mortality. In addition, complex TGA ( p = 0.022) and post-operative aortic regurgitation ( p < 0.001) are identified as potential predictive factors for reintervention. Arterial switch operation provides excellent long-term survival and low reintervention rates in TGA. These results support tailored long-term surveillance, particularly for patients with complex anatomy or coronary anomalies.

BACKGROUND:Neonatal Ross procedure is rarely performed, especially in the setting of active infective endocarditis and prior pulmonary artery banding. CASE SUMMARY:We present a rare case of a 2.5-month-old boy with a history of complex congenital heart disease, including aortic coarctation, severe aortic stenosis, and a ventricular septal defect, who developed infective endocarditis on a dysplastic bicuspid aortic valve. DISCUSSION:This case highlights surgical decision-making and technical challenges in harvesting a pulmonary autograft after previous pulmonary artery banding in the presence of active infection. TAKE-HOME MESSAGES:Ross procedure can be feasible in neonates with complex anatomy and infection. Prior pulmonary artery banding increases technical complexity. Autograft resistance to reinfection supports its use in pediatric infective endocarditis.

Congenital heart defects (CHDs) are the leading congenital anomalies in humans, with approximately 1.3 million worldwide cases each year. In the past, they were one of the leading causes of infant mortality, and only 15% of patients in the 1940s and 1950s reached adulthood. Today, a profound shift in survival rate has occurred thanks to technological advancements, a deeper understanding of these conditions, early diagnosis capabilities, and neonatal surgery, with 90-95% of newborns with CHD reaching adulthood. The future of CHDs looks promising, with genetic and epigenetic discoveries enabling personalized treatments and improvements in the management of long-term complications. Tissue engineering and regenerative medicine could revolutionize treatment, with the creation of custom-made heart valves and vascular tissues, as well as cellular therapies to improve myocardial function, along with potential solutions for complete heart replacement in cases of myocardial failure. The future challenge remains ensuring that children born with CHDs not only have a long life but also a quality of life comparable to that of all other children.

Background: The most cost-benefit and affordable conduit to reconstruct right ventricle outflow tract (RVOT) is the pulmonary homograft, which is limited by the procurement and size availability. Xenograft is a good performance alternative, but the dysfunction due to progressive stenosis or regurgitation can be more frequently experienced. Aim of this study is to evaluate the mid-term outcome of Biopulmonic pulmonary conduits. Methods: From 08/2016 to 01/2021, 34 patients underwent RVOT reconstruction with Biopulmonic conduit, a porcine pulmonic bioprosthesis. We collected demographic data and clinical characteristics of patients and, for mid-term follow-up, freedom from conduit failure defined as stenosis or regurgitation resulting in surgical conduit change or percutaneous pulmonary valve implantation. Results: Mean follow-up was 14.65±14.1 months (IQR 3.8-19.8) with a freedom from conduit failure of 58,9% at 19 months. Mean gradient moved from 13.2±8.6 mmHg at 1 month to 26.3±19.6 mmHg at 12 months after surgery (p=0.0367). 11 patients (32.3%) required pulmonary valve replacement, 9 patients were treated with percutaneous valve implantation, 2 patients required surgery of whom one for endocarditis. Corticosteroid therapy in the post-operative period, used to treat pericardial effusion or no infective fever, showed to reduce the conduit dysfunction (p=0.0135). Conclusions: Biopulmonic conduit showed a high failure rate due to a suspected inflammatory reaction. This type of conduit should be used in selected patients and deep control of inflammatory markers is recommended.

Surgical implantation of a right ventricle to pulmonary artery (RV-PA) conduit is an important component of congenital heart disease (CHD) surgery, but with limited durability, leading to re-intervention. The present single-center, retrospective, cohort study reports the results of surgically implanted RV-PA conduits in a consecutive series of children and adults with CHD. Patients with CHD referred for RV-PA conduit surgical implantation (from October 1997 to January 2022) were included. The primary outcome was conduit failure, defined as a peak gradient above 64 mm Hg, severe regurgitation, or the need for conduit-related interventions. Longitudinal echocardiographic studies were available for mixed-effects linear regression analysis. A total of 252 patients were initially included; 149 patients were eligible for follow-up data collection. After a median follow-up time of 49 months, the primary study end point occurred in 44 (29%) patients. A multivariable Cox regression model identified adult age (>18 years) at implantation and pulmonary homograft implantation as protective factors (hazard ratio 0.11, 95% confidence interval [CI] 0.02 to 0.47 and hazard ratio 0.34, 95% CI 0.16 to 0.74, respectively). Fever within 7 days of surgical conduit implantation was a risk factor for early (within 24 months) failure (odds ratio 4.29, 95% CI 1.41 to 13.01). Long-term use of oral anticoagulants was independently associated with slower progression of peak echocardiographic gradient across the conduits (mixed-effects linear regression p = 0.027). In patients with CHD, the rate of failure of surgically implanted RV-PA conduits is higher in children and after nonhomograft conduit implantation. Early fever after surgery is a strong risk factor for early failure. Long-term anticoagulation seems to exert a protective effect.

Background: Adult with congenital heart defects (ACHD) constitute a heterogeneous and expanding group of patients. ACHD are characterized by complex cardiac anatomical features, precarious hemodynamic stability and associated multiorgan dysfunction, with higher risk for advanced heart failure requiring orthotopic heart transplantation (OHT). To stratify this risk, Seese et al. proposed an ACHD-OHT-Risk Score for 1-year mortality including age, comorbidities and laboratory values. This study evaluated early and long-term survival of ACHD underwent OHT in our unit and the adherence of the ACHD-OHT-Risk Score for our population. Methods: ACHD (age>16 years) underwent OHT between January 1997 and August 2024 were analyzed in the study. Kaplan-Meier analysis was performed to report the early and long-term survival. In addition, population was divided in two groups according to the ACHD-OHT-Risk Score: Low-risk (<2 points)-High-risk(≥2points). Groups’ survival at 1year was compared. Results: From 1997 to 2024, 33 ACHD underwent OHT. Mean age at transplant was 33,32 ± 12,60 years. Parameters used to determine the risk score are reported in fig.1. The overall survival at 1, 10,20 years was 84.8%, 77.7% and 64.8% respectively. Low-risk group included 13 patients (39,4%), High-risk group 20 patients (60,6%). One-year survival in low-risk group at 1,6,12 months was 100%,92%,92%, while in high risk group 95%,80% and 80%, respectively (log- rank=0.33). Conclusion: OHT in ACHD remains challenging and affected by high risk mortality in the first year of follow-up. The ACHD-OHT-Risk Score derived some risk factors of early mortality and could suggest a more aggressive approach to waiting list enrolment and progression to achieve OHT in optimal multiorgan conditions.

OBJECTIVES:Left pulmonary artery (LPA) or bifurcation stenoses at Fontan palliation can be very challenging to treat and may also require cardioplegia and aortic transection. Moreover, the low pressure of Fontan circulation and the bulkiness of the aorta increase the risk of a patch angioplasty collapse. Pre-Fontan LPA stenting of stenotic LPAs overcomes those drawbacks therefore the present study aimed to evaluate its advantageous impact on Fontan surgery. METHODS:A multicentre retrospective analysis was performed on 304 consecutive Fontan patients. The study population was divided into 2 groups (LPA stented, n = 62 vs not stented, n = 242); pre-and postoperative data were compared. RESULTS:LPA-stented patients had a higher prevalence of systemic right ventricle (P = 0.01), hypoplastic left heart syndrome (P = 0.042), complex neonatal palliations (Norwood/Damus-Kaye-Stansel) and surgical LPA patch repair at Glenn (P < 0.001). No differences were found in cross-clamp rates, early (P = 0.29) and late survival (94.6% vs 98.4, P = 0.2) or complications (P = 0.14). Complex palliations on ascending aorta/aortic arch (P = 0.013) and surgical LPA repair at Glenn (P < 0.001) proved to be risk factors for LPA stenting before Fontan at multivariable analysis. CONCLUSIONS:The LPA-stented group showed similar outcomes in terms of survival and complications rate compared to patients without LPA stenosis; however, they significantly differ in their higher preoperative risk profile and in their more complex anatomy. Complex neonatal palliations involving ascending aorta or aortic arch may increase the risk of pulmonary branches stenosis requiring stenting; therefore, preoperative stenting of LPA stenoses could help to reduce the surgical risk of complex Fontan procedure by avoiding the need for cross-clamp or complex mediastinal dissections to perform a high-risk surgical repair.

Background: Grown-up congenital heart disease patients (GUCHs) represent an expanding population characterized by higher early post heart transplant mortality compared with the population without congenital heart disease. Aim of our study was to identify potential preoperative risk factors and to develop a risk-score that could complement the pre-transplant assessment of GUCHs. Methods: From 2009 to 2024, 33 GUCHs underwent heart transplantation. Spearman’s correlation coefficient or Kendall’s tau were used to examine bivariate relationship between pre-transplant variables and mortality. Variables were explored using a correlation matrix: positive or negative correlations greater than 0.30 in absolute value were highlighted. ROC analysis was performed to evaluate predictive ability of the variables and score. Optimal cut-offs were identified by Youden’s J index. Moreover, ROC analysis was used to compare our score with the score proposed by Seese et al. Results: Seven predictors resulted associated with mortality: sodiemia (p=0.024), haemoglobin (p=0.013), haematocrit (p=0.007), FEV1 (p=0.068), systolic pulmonary artery pressure (PAP, p=0.037), diastolic-PAP (p=0.011) and mean-PAP (p=0.013). Overall survival in patients with Bologna’ score greater than 3 was 75%, 63% and 63%, while in patients with Bologna’ score lower than 3 was 88%, 83% and 83% at 1,5 and 10 years respectively(log-rank=0.037). ROC analysis comparing the two scores revealed a better accuracy of the Bologna score (AUC=0.730) than Seese’s score (AUC=0.488). Conclusions: Mortality in GUCHs is not only influenced by cardiac pathology but by the patient’s overall compensation picture. The developed population-specific risk-score showed to be predictive for mortality and could be used in the pre-transplant evaluation of GUCHs.

Despite the clinical results of the Fontan operation have certainly improved, it still presents with an inherent surgical risk of death and early morbidities. This is a retrospective clinical study of children undergoing Fontan operation in 9 congenital cardiac centers in Italy between 1990 and 2023. Clinical and surgical data were collected via a dedicated RedCap database. Primary outcome was cohort’s mortality, also considering different decades, while secondary outcomes were postoperative complications and reintervention. In the last 3 decades, there were 897 patients undergoing Fontan operation, M/F 512/384, median age: 4.5 years (IQR 3.3–6.4), median weight 16 kg (IQR 14–22). A first palliation was deemed necessary in 710 patients (80

Background and Aim: ASO is choice procedure for TGA correction. The transfer of coronary artery origins is the crucial part of this operation, especially in presence of coronary anomalies that increase complexity and have an effect on postoperative outcomes. The study’s aim is to analyze the relationship between coronary anomalies and postoperative outcome. Methods: We retrospectively reviewed 233 pts who underwent ASO from 01/2001 to 10/2023. At operation, median age was 18,28±49,85 days and median weight 3235,97±729,99 gr. The study included TGA simple (n=158) and complex (n=75). Coronary anatomy is classified according to the Yacoub classification. The surgical technique used for coronary transplantation is trap-door technique, sometimes button technique also. The median follow-up is 8,5±6,6 years. Results: 86 (36,9%) patients had coronary anomalies. REDO was performed in 31 (13,5%) pts, not related to the coronary arteries. 5 (2,6%) pts had a coronary stenosis, 1 required PCI. There were 4 (1.9%) in-hospital deaths (no post-discharge deaths). Overall survival was 98,1%, 97.9% and 96,8% at 1, 5 and 10 years. Coronary anatomy didn’t influence early postoperative mortality, instead intramural coronary artery and button technique coronary transplantation significantly increased mortality. Conclusions: ASO remains the choice procedure also for TGA with coronary anomalies with excellent in-hospital and post-discharge outcomes in terms of overall survival and freedom from reoperation. Improvements in preoperative imaging and increased surgeon experience decrease mortality associated with abnormal coronary patterns. Furthermore, pts with intramural coronary artery have higher risk for adverse postoperative outcome and must be monitored carefully.

Background: Heart failure in paediatric age represents a significant challenge, and Berlin Heart (BH) ventricular assist device is frequently the only option. We evaluated the effects of recent strategic changes in BH utilization in our centre. Methods: From January 2008 to March 2024, 24 patients underwent BH implant. We divided the population in first era (FE, 2008-2016; n=13; Age 5.3 years; weight 17 kg) and second era (SE, 2017-2024; n=11; Age 6.3 years; weight 18.5 kg), (FE, 2008-2016) and second era (SE, 2017-2024), the latter characterized by a more aggressive approach in order to avoid ECMO implantation before BH. Kaplan-Meier curves have been employed to compare mortality. Results: In-hospital mortality was 32%. All survived patients underwent orthotopic heart transplantation (OHT), except one still hospitalized in BH. Comparing the two era the need for pre-operative intubation (18.2%, p=0.001) and the use of ECMO (9.1%) were greatly reduced in the SE. No biventricular BH was implanted in the SE. Kaplan-Meier analysis revealed similar survival rates in BH in the two groups. Otherwise, survival after OHT in the FE was 90%, 70% and 70% at 6months, 1year and 4years respectively, in the SE was 100% all over the follow-up period. Overall survival rates at 5 years in patients in whom ECMO wasn’t used before BH implantation was 72%, while in patients with ECMO was 50%, (log- rank=0.307). Conclusions: Our analysis may suggest how an earlier and more aggressive approach in the BH implantation in pediatric patients with heart failure avoiding the use of ECMO may improve long-term survival. ECMO could be risk factor of mortality.

Double outlet right ventricle (DORV) is a malformation of the fetal heart in utero that affects the ventricular chambers. It usually presents with a displacement of the aorta and more than half the circumference of both arterial valves in the right ventricle. A peculiar characteristic is given by an interventricular septal defect (VSD), which allows communication between the left ventricle and the right with consequent mixing of oxygenated and non-oxygenated blood inside the cavities. A crucial question in assessing the degree of severity of functional dysfunction concerns the percentage of oxygenated blood that is ejected into the primary circulation via the aorta, a result that depends on the details of the vortex flow pattern within the two ventricular chambers. This study analyzes a complete DORV case through the use of numerical simulations that allow to identify the concentrations of oxygenated and non-oxygenated blood passing through this geometry. Results show that the VSD presents a significant impact on the fluid dynamic performance of the two ventricles. The analysis of blood concentration allowed to quantify the presence of oxygenated blood ejected into the pulmonary artery and of non-oxygenated blood into the aortic artery. The analysis of this specific case aims to demonstrate how the fluid dynamics analysis of this rare malformation, properly coupled with imaging technology, can provide information that could not be obtained otherwise and that are relevant for a careful clinical management including timely therapeutic intervention.

Background: A progressively increasing prevalence of congenital heart disease (CHD) in adulthood has been noticed in recent decades; CHD cases with a systemic right ventricle have a poorer outcome. Methods: Seventy-three patients with SRV evaluated in an outpatient clinic between 2014 and 2020 were enrolled in this study. Thirty-four patients had a transposition of the great arteries treated with an atrial switch operation; 39 patients had a congenitally corrected transposition of the great arteries (ccTGA). Results: Mean age at the first evaluation was 29.6 ± 14.2 years; 48% of the patients were female. The NYHA class at the visit was III or IV in 14% of the cases. Thirteen patients had at least one previous pregnancy. In 25% of the cases, complications occurred during pregnancy. Survival free from adverse events was 98.6% at one year and 90% at 6-year follow-up without any difference between the two groups. Two patients died and one received heart transplantation during follow-up. The most common adverse event during follow-up was the presence of arrhythmia requiring hospitalization (27.1%), followed by heart failure (12.3%). The presence of LGE together with lower exercise capacity, higher NYHA class and more dilated and/or hypokinetic RV predicted a poorer outcome. Quality of life was similar to the QoL of the Italian population. Conclusions: Long-term follow-up of patients with a systemic right ventricle is characterized by a high incidence of clinical events, prevalently arrhythmias and heart failure, which cause most of the unscheduled hospitalizations.

ObjectivesIsolated tricuspid valve dysplasia is a rare disease characterized by a wide spectrum of possible anomalies. We describe the use of the Cone concept to treat a patient with a double-orifice tricuspid valve with massive regurgitation and severe deficit of coaptation.MethodsThree adult patients with congenital non-Ebstein tricuspid valve anomaly characterized by severe coaptation deficiency underwent tricuspid valve repair applying the Cone technique. In particular, we describe the case of a symptomatic 21-year-old woman with a double-orifice tricuspid valve, with massive regurgitation and severe right ventricular dilatation. The tricuspid valve was transformed from a double-orifice valve into a single-orifice valve. The most superior orifice was opened, and the tissue surrounding the orifice was used to extend the leaflet of the inferior orifice. A Cone was created, and a ring annuloplasty was used to stabilize the result.ResultsThe patient was discharged home after 7 days with trivial residual tricuspid regurgitation and no significant antegrade gradient. The final coaptation height was 2.8 cm. The cardiothoracic ratio decreased from 0.77 to 0.59 after 2 months, and symptoms promptly improved.ConclusionsOver the past 2 years, we have applied the Cone creation concept to patients with a severely dysplastic tricuspid valve with excellent early results. One patient had a double-orifice tricuspid valve, and a Cone repair concept was adopted anyway. One orifice was sacrificed, and surrounding tissue was used to augment the leaflets of the other orifice. A Cone was created to improve central coaptation with a good initial result.

Objective: The object of the present study is to evaluate factors precluding heart transplantation (HTx) in adult congenital heart disease patients (ACHD) with end-stage heart failure (HF) referred for HTx evaluation. Methods: This retrospective cohort study enrolled consecutive ACHD patients considered for HTx in our institution between 2014 and 2020 and patients receiving HTx between 2001 and 2013. HTx refusal due to poor candidacy status for excess risk of mortality after transplantation served as the main study outcome. Results: Between 2014 and 2020, 46 ACHD patients were evaluated for HTx, 14 ACHD patients underwent HTx between 2001 and 2013 (final sample size 60 patients). We compared clinical, anatomical and demographic data of 41 patients suitable for transplantation with 15 patients refused after screening (excluding 4 patients with ongoing screening). Risk factors for refusal were: multiple high risk features (odds ratio [OR]: 3.6; 95% confidence interval [CI]: 1.1 to 12.9; p 0.048); anatomical factors (OR: 14.5; 95% CI: 3.1 to 68.4; p 0.001), out-of-center ACHD/HTx program referral (OR: 5.3; 95% CI: 1.5 to 19.0; p 0.01). HTx refusal identifies a high risk ACHD patient subgroup (hazard ratio for overall mortality: 3.1; 95% CI: 1.1 to 8.3; p 0.02). Conclusions: In our study risk factors for refusal from HTx are adverse anatomical features, multiple conventional HTx high risk factors and out-of-center referral. ACHD patients refused from HTx present shorter time to death. Efforts to increase HTx candidacy are strongly necessary for this growing population.

Background: Many studies have investigated ventricular arrhythmias (VA) risk factors in patients with repaired Tetralogy of Fallot (TOF), but none have evaluated the clinical presentation of VA before and after pulmonary valve replacement (PVR). Objectives: This study examines the current VA prevalence and clinical presentation in patients with repaired TOF before and after PVR. Methods: Retrospective analysis performed on adult patients with repaired TOF from 1995 to 2021. Medical and surgical history, ECG, transthoracic echocardiogram, cardiac magnetic resonance imaging (MRI), cardiopul-monary exercise test (CPET) and 24-hour ECG Holter were evaluated.Results: Before PVR (154 patients, mean age 26 +/- 11 years) 3.2 % of patients experienced sustained ventricular tachycardia (VT). Patients with VT were older at repair, presented more frequently with arrhythmic symptoms, QRS duration >= 180 ms, right ventricular outflow tract (RVOT) dysfunction with right ventricular (RV) dysfunction and complex premature ventricular complexes (PVC). All presented with monomorphic and he-modynamically tolerated VT, were referred after the VT episode and presented with longstanding RVOT dysfunction. After PVR (analysis performed on 85 patients, mean age 35 +/- 13 years, mean follow-up 9 +/- 1 years) VA incidence was not significantly different (6 %, equal to 0.7 % per year, p = .52). Two patients presented with resuscitated ventricular fibrillation (VF) related to short coupled PVC, while the others experienced mono-morphic VT related to the development of a new hemodynamic lesion, all these patients received PVR after longstanding RVOT dysfunction with RV dysfunction.Conclusion: Currently the incidence of VA in patients with repaired TOF is low. Patients with VA are older at repair and more frequently present with longstanding RVOT dysfunction with RV dysfunction. While mono-morphic VT are usually hemodynamically tolerated and rarely recurs after hemodynamic lesions resolution, VF related to short coupled PVC rarely can occur after PVR.