Aims:We aim to explore whether biomechanical simulations can identify coronary compression sites and support pre-operative assessment as a proof-of-concept approach in subjects with anomalous aortic origin of a coronary artery. Methods and results:Patient-specific computer-aided engineering models of the aortic root and coronary arteries were developed from computed tomography scans of subjects with anomalous aortic origin of coronary arteries, retrospectively evaluated to include variants with and without an intramural segment. The fixed and dynamic components of stenosis were assessed by simulating blood pressure from rest to extreme effort and by retrieving the percentage of narrowing of the proximal anomalous lumen with reference to the distal segment. Patients were classified as potential candidates for surgery if the degree of stenosis was >50% in any of the simulated pressure scenarios. A total of 31 patients (19 with interarterial course and intramural segment, 12 with non-intramural course) were simulated. Simulations showed promising trends of agreement with retrospective clinical treatment decisions (13 aligned with surgery, 11 aligned with non-surgical management). This concordance with surgical decisions should not be interpreted as validation for predicting myocardial ischaemia. Conclusion:Computational models of the anomalous aortic origin of a coronary artery have not yet been routinely introduced into the clinical setting. Our method demonstrates potential as a proof-of-concept tool for identifying coronary compression-prone regions and estimating stenosis severity, but further studies are needed to validate its diagnostic and clinical utility.
Background In 2014 a professional collaboration was established between IRCCS Policlinico San Donato, Milan, Italy (PSD) and the Institute for Cardiovascular Disease Dedinje, Belgrade, Serbia (ICDD) to support and improve the care of adult patients with congenital heart disease (ACHD) in the country. Since 2018, this collaboration has become more structured, with a specific focus on surgery for adults with complex congenital heart disease (CHD). Aim of the study was to evaluate the outcomes of this collaboration. Methods We conducted a retrospective study on all consecutive adult patients (aged over 18 years) with CHD who underwent surgery at the Institute for Cardiovascular Disease Dedinje (ICDD) over a ten-year period, from January 2014 to December 2024. Collected data included number of cases, mean age at surgery, types of surgical procedures performed, number of previous operations, distribution of cases based on complexity, patient outcomes, and follow-up. Results Between 2014 and 2024, a total of 244 ACHD patients underwent surgery at ICDD. Eighty-eight of these patients (88/244 = 36%) required complex procedures and were referred to the visiting PSD cardiac surgeon. Mean age at surgery was 34.8 years (±14 years). The most common diagnosis was right ventricular outflow tract (RVOT) pathology, accounting for 89% of cases. Seventy patients (80%) underwent reoperations following previous procedures. In-hospital mortality was 4.5% (4 patients). No additional deaths or reoperations occurred during a mean follow-up of 58 months (2-122 months). Conclusions This ten-year international partnership demonstrates that high-quality ACHD surgical care can be delivered sustainably in countries with limited case volumes and expertise. By combining local access with external specialist support, the model has achieved outcomes comparable to high-volume Western European centres. Increased access to ACHD surgery in their home country and reduced financial burden suggests that this model of care may potentially serve in other countries facing similar healthcare challenges.
Recent advances in congenital heart disease management reflect a paradigm shift towards less invasive, physiology-preserving, and durable interventions. Transcatheter pulmonary valve implantation has become an established therapy for right ventricular outflow tract dysfunction, supported by multiple valve systems tailored to increasingly complex anatomies. These devices have demonstrated high procedural success and sustained hemodynamic performance, although infective endocarditis and arrhythmic complications remain clinical concerns. In parallel, transcatheter edge-to-edge repair for atrioventricular valve regurgitation (AVVR) has expanded from acquired disease to congenital populations, particularly in patients with single-ventricle physiology or systemic AVVR where surgical risk is prohibitive. Early experiences with devices such as MitraClip™, TriClip™, and Pascal have shown promising results in improving valve competence and functional status. From a surgical perspective, the Ross procedure continues to offer a unique, physiological alternative for aortic valve replacement in selected young adults, achieving excellent long-term outcomes when performed in high-volume centres with reinforcement strategies to prevent autograft dilation. Similarly, percutaneous correction of sinus venosus atrial septal defect using covered stents has emerged as a safe and effective alternative to conventional repair, providing anatomical reconstruction with low complication rates. The modern Fontan circulation exemplifies the evolution of single-ventricle palliation, emphasizing conduit optimization, pro-active re-intervention, and individualized surgical planning to sustain function over time. Collectively, these innovations underscore the growing integration of transcatheter and surgical therapies, re-defining long-term management paradigms and improving survival and quality of life in patients with congenital heart disease.
Background Ebstein’s anomaly (EA) exhibits significant anatomical and clinical heterogeneity, warranting a systematic approach to risk stratification. While the Carpentier classification (CC) is commonly employed for qualitative disease assessment, there is increasing interest in quantitative imaging parameters to personalise monitoring strategies and therapeutic interventions. We sought to evaluate the association between cardiovascular magnetic resonance (CMR) parameters, CC and symptoms of heart failure (HF). Also, we investigated whether CMR-derived markers may predict the need for bidirectional cavopulmonary anastomosis (BCPA) and the occurrence of haemodynamic complications or significant right ventricular (RV) dysfunction in the postoperative stay.Methods This retrospective study evaluated consecutive patients diagnosed with EA undergoing 1.5T CMR acquisition at a tertiary care centre. CC and quantitative indices were extracted from steady-state free precession sequences.Results In a total population of 60 patients (53% male, median age 22 years), CMR parameters most strongly associated (p<0.001) with CC included indexed displacement of the septal (SLDi) and inferior (ILDi) leaflets, Ebstein valve rotation angle, functional RV ejection fraction, indexed atrialised RV end-diastolic volume and the ratio of either atrialised or functional RV to anatomical RV. Decreasing left ventricular ejection fraction (OR 0.85, 95% CI 0.75 to 0.97, p=0.01) and increasing ILDi (OR 1.05, 95% CI 1.00 to 1.10, p=0.04) emerged as the most prominent variables associated with HF symptoms. Additionally, ILDi was significantly linked to the need for BCPA (OR 1.15, 95% CI 1.03 to 1.28), the occurrence of haemodynamic complications (OR 1.09, 95% CI 1.01 to 1.18) and significant RV dysfunction in the postoperative stay (OR 1.08, 95% CI 1.01 to 1.17).Conclusions Quantitative CMR indices proved to be effective in distinguishing between Carpentier classes and they may be valuable in an integrated CMR-based approach to assess EA severity. Among these, ILDi reflects both the extent of tricuspid valve abnormality and RV atrialisation and may serve as a useful metric in guiding personalised therapeutic strategies.
The extracardiac Fontan procedure is the most widely adopted surgical approach for definitive palliation of single-ventricle anomalies, offering improved hemodynamics and reduced arrhythmogenic risk. However, limited access to the pulmonary venous atrium during follow-up poses significant challenges for electrophysiologic and interventional procedures. We describe a simple technical modification of the extracardiac Fontan designed to preserve these advantages while facilitating future percutaneous access.
OBJECTIVES:Testing for high-sensitivity cardiac troponin (hs-cTn) often occurs following pediatric cardiac surgery, although evidence regarding its utility remains heterogeneous. This study aimed to assess the prognostic value of postoperative hs-cTnT patterns detected within 48 h after cardiac surgery. METHODS:Serum hs-cTnT (Roche Diagnostics, 5th generation assay) concentrations were measured post-operatively at three time points: upon pediatric intensive care unit (PICU) admission (T1), 13 h after admission (T2), and 24 h after T2 (T3). Surgical and postoperative variables were recorded. The outcome was a composite of 30-day mortality and/or PICU stay >10 days. Multivariable logistic regression and model discrimination were evaluated. RESULTS:Over 15 months, 154 patients (56.5 % male) with a median age of 3.8 (25th-75th percentile: 1.2-7.8) months and a median Risk Assessment for Congenital Heart Surgery score of 2, were included in this study. The outcome occurred in 24 % of the population (with the 30-day mortality rate being 7.8 %). The time point of the highest recorded hs-cTnT concentration, and not the concentration, was significantly associated with the outcome (p=0.001). Patients, whose peak hs-cTnT concentration occurred later after surgery had a sixfold higher risk of the adverse outcome. Urgent/emergent procedures were associated with a 3.6-fold increase in relative risk, and each additional minute of cardiopulmonary bypass (CPB) time conferred a 1.6 % incremental increase in risk. The multivariate model including time to troponin peak, CPB duration, need for urgent/emergent procedures showed a good discriminatory ability (c-statistic=0.84; 95 %CI: 0.78-0.90). CONCLUSIONS:Timing of hs-cTnT elevation post cardiac surgery is a valuable prognostic marker in children.
Neonates and infants undergoing cardiac surgery are prone to cerebral injuries. Neuromonitoring with continuous conventional electroencephalography (cEEG) and/or amplitude integrated electroencephalography (aEEG) is able to detect subclinical abnormal cerebral patterns, especially seizures, which have been found to correlate with medium-long term neurocognitive deficits. The endpoints of this study are (i) to define a risk model for electrographic subclinical epileptic seizures (ES) and (ii) to investigate the relationship between ES and neurologic and non-neurologic outcomes. Retrospective analysis of 373 neonates and infants < year undergone cardiac surgery and receiving a pre/postoperative combined cEEG/aEEG exam. The risk factors for postoperative ES were investigated and pooled in a predictive model, and postoperative ES were assessed for association with neurologic and non-neurologic (major morbidity) postoperative patterns. The independent risk factors for postoperative ES were the complexity of surgery (RACHS-2 score), an open sternum, and the hematocrit at the arrival in the intensive care unit. The predictive model based on these factors yielded a good discrimination (c-statistics 0.81). Postoperative ES were significantly associated with major non-neurologic morbidity, but once corrected for other confounding factors they lost significance. Conversely, postoperative ES were the only predictor of cerebral injuries, with an odds ratio of 8.0 (95
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.
Neonates with complex congenital heart disease (CHD) frequently require early and staged surgical interventions, which carry a high risk of postoperative complications. The coexistence of an interrupted aortic arch, ventricular septal defect (VSD), and mitral valve stenosis represents a rare and particularly high-risk constellation. This case is noteworthy for the integration of multistage cardiac surgery, prolonged extracorporeal membrane oxygenation (ECMO), and an innovative wound management strategy combining negative pressure wound therapy (NPWT) with topical oxygen-enriched gel. We report the case of a one-month-old Romanian female infant diagnosed with type A interrupted aortic arch, VSD, and mitral valve stenosis. The initial surgical procedure included aortic arch reconstruction, VSD closure with a bovine pericardial patch, and mitral valve repair. Severe residual mitral regurgitation necessitated immediate initiation of veno-arterial ECMO, followed by mitral valve replacement with a Melody bioprosthesis. On postoperative day 19, thrombotic obstruction of the prosthetic valve required a third surgical intervention for clot removal, after which ECMO was discontinued. Delayed sternal closure was performed on day 26, but wound dehiscence developed on day 33. Management included NPWT combined with topical oxygen-enriched gel for 33 days, followed by gel monotherapy for 38 additional days. Complete secondary intention healing was achieved after 73 days. Despite developing Pseudomonas aeruginosa sepsis and late-onset Staphylococcus haemolyticus bacteremia, the patient recovered fully without neurological deficits or wound-related sequelae. This case highlights the feasibility of combining advanced surgical management with innovative wound care strategies in critically ill neonates with complex CHD. The successful integration of NPWT and topical oxygen therapy promoted wound healing after delayed sternal closure, even in the context of severe infection and prolonged ECMO support. These findings suggest that tailored, multidisciplinary approaches may optimize outcomes in high-complexity neonatal cardiac cases.
Background/Objectives: Congenital heart malformations (CHDs) are not rare diseases, and, in many cases, their pathogenic mechanisms are well established. Several conotruncal defects are associated with genetic syndromes such as DiGeorge syndrome and RASopathies, reflecting shared developmental pathways affecting cardiac outflow tract formation. However, even common CHDs may occur within complex syndromic contexts, making early diagnosis essential for optimal management. This review aims to provide a unifying framework linking ciliary dysfunction to CHD phenotypes. Methods: We performed an integrative narrative review of genetic, experimental, and developmental studies focusing on the role of primary and motile cilia in cardiac morphogenesis. Particular attention was given to signaling pathways regulated by cilia and their contribution to disease phenotypes. Results: Emerging evidence indicates that primary and motile cilia act as central regulators of cardiac development, integrating morphogen gradients and mechanical cues into transcriptional programs. Dysfunctions in ciliary structure or signaling are increasingly recognized as important contributors to selected complex CHD phenotypes, particularly in syndromic forms and laterality-associated defects. This cilia-centered model may help explain part of the phenotypic heterogeneity observed in CHD and highlights shared mechanisms across distinct clinical entities. Conclusions: Understanding cilia-dependent mechanisms provides a unifying conceptual framework linking genetic defects to disrupted morphogenesis. This perspective may refine disease interpretation and support future development of precision diagnostics and pathway-informed therapeutic strategies in CHD.
Background Cardiac surgery-associated acute kidney injury (CSA-AKI) is a common feature in pediatric patient population and is associated with major morbidity and mortality. Its definition and grading are still unclear, and 3 different adjudication criteria exist. The aim of this study is to find the best definition for clinically relevant CSA-AKI. Methods prospective observational study on 174 pediatric patients < 20 kgs. CSA-AKI was adjudicated based on the Pediatric Risk, Injury, Failure, Loss of Function, and End Stage (pRIFLE), modified Kidney Disease Improving Global Outcomes (mKDIGO) and pediatric reference change value optimized for AKI children (pROCK) definitions. The 3 definitions were compared for association and prediction of a composite outcome. Results The incidence of CSA-AKI was different for the different definitions. 43.7% according to the pRIFLE, 14.9% for the mKDIGO and 13.8% for the pROCK. The composite outcome had a rate of 18.4%. At a univariate analysis, the odds ratio for experiencing the composite outcome was 3.0 (95% confidence interval 1.35–6.75, P = 0.007) for the pRIFLE, 4.4 (95% confidence interval 1.79–1.11, P = 0.001) and 5.2 (95% confidence interval 2.06–13.1) for the pROCK. The 3 definitions were significantly discordant. When corrected for other confounders, only the pROCK remained independently associated with the composite outcome (odds ratio 4.29, 95% confidence interval 1.23–14.9, P = 0.022). Prediction of the composite outcome was not significantly better for the pROCK. Conclusions the pRIFLE overestimates the incidence of clinically relevant CSA-AKI. The best definition for a clinically relevant CSA-AKI, as well as for its prediction, is obtained by the pROCK.
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, classically characterized by right ventricular outflow tract obstruction, ventricular septal defect, overriding aorta, and right ventricular hypertrophy. Recent advances in molecular and genomic research indicate that TOF is part of a phenotypic continuum encompassing Trilogy, Tetralogy, and Pentalogy of Fallot, in which the variability of anatomical presentation reflects shared genetic and epigenetic mechanisms with highly variable penetrance and expressivity. Variants in NOTCH1, FLT4, KDR, GATA6, and TBX1 highlight key pathways in conotruncal development and endothelial-mesenchymal transition, yet these well-known genes explain only a fraction of the genetic landscape. Emerging studies have identified additional candidate genes and networks involved in cardiac morphogenesis, including transcriptional regulators, signaling mediators, chromatin-remodeling factors, and splicing-associated genes such as PUF60 and DVL3. Epigenetic mechanisms, including DNA methylation, histone modifications, and non-coding RNA expression, further modulate phenotypic expressivity and contribute to variability along the Trilogy-Tetralogy-Pentalogy spectrum. This review integrates current genomic and clinical evidence to provide a comprehensive overview of the molecular architecture of Fallot-type conotruncal malformations, emphasizing the interplay between genetic and epigenetic mechanisms, genotype-phenotype correlations, and implications for diagnosis, risk stratification, counseling, and personalized management in the era of precision cardiology.
Background. Neonates and infants undergoing cardiac surgery are prone to cerebral injuries. Neuromonitoring with continuous conventional electroencephalography (cEEG) and/or amplitude integrated electroencephalography (aEEG) is able to detect subclinical abnormal cerebral patterns, especially seizures, which have been found to correlate with medium-long term neurocognitive deficits. The endpoints of this study are (i) to define a risk model for epilectic seizures (ES) and (ii) to investigate the relationship between ES and neurologic and non-neurologic outcomes. Methods . Retrospective analysis of 373 neonates and infants < year undergone cardiac surgery and receiving a pre/postoperative combined cEEG/aEEG exam. The risk factors for postoperative ES were investigated and pooled in a predictive model, and postoperative ES were assessed for association with neurologic and non-neurologic (major morbidity) postoperative patterns. Results. The independent risk factors for postoperative ES were the complexity of surgery (RACHS-2 score), an open sternum, and the hematocrit at the arrival in the intensive care unit. The predictive model based on these factors yielded a good discrimination (c-statistics 0.81). Postoperative ES were significantly associated with major non-neurologic morbidity, but once corrected for other confounding factors they lost significance. Conversely, postoperative ES were the only predictor of cerebral injuries, with an odds ratio of 8.0 (95% confidence interval 2.25-28.3, P=0.003). Conclusions. Postoperative ES are associated with an hemodynamic compromise and low arterial oxygen content, with a consequent inadequate oxygen delivery to the brain and other organs. In presence of these factors, EEG monitoring is useful for the early diagnosis of cerebral injuries in patients sedated and paralyzed.
BACKGROUND:Weaning from cardiopulmonary bypass in pediatric cardiac surgery is challenging, especially after prolonged procedures. Delayed chest closure may be necessary in cases of low cardiac output syndrome (LCOS) to support hemodynamic recovery. Although near-infrared spectroscopy is standard for neuromonitoring, amplitude-integrated electroencephalography (aEEG) and continuous electroencephalography (cEEG) are emerging tools. CASE SUMMARY:We report the case of a 6-month-old infant with transposition of the great arteries who required delayed chest closure after surgical repair. LCOS recurred after chest closure on postoperative day 3 despite stable near-infrared spectroscopy values. Retrospective aEEG/cEEG analysis, unintentionally initiated before closure, showed early EEG abnormalities preceding clinical signs, which were resolved after chest reopening. DISCUSSION:This case highlights the potential of aEEG/cEEG to detect early cerebral compromise due to LCOS, even when conventional monitoring appears normal, an association not well established in the literature. TAKE-HOME MESSAGE:Cerebral distress may be detected earlier with aEEG compared with standard monitoring, supporting timely LCOS identification and management.
The primary aim of this study was to evaluate the impact of the polyhexamethylene biguanide (PHMB) care bundle on the occurrence rates of surgical site infections (SSIs) in paediatric and neonatal cardiac surgery, addressing a critical gap in paediatric-specific infection prevention protocols. A retrospective cohort study included patients under 18 years old who underwent cardiac surgery at IRCCS Policlinico San Donato. Cohort A (n = 117) received the PHMB care bundle from April to December 2023, while Cohort B (n = 801) received conventional care from September 2020 to March 2023. The 1:1 propensity score matching was used to balance covariates between cohorts, resulting in two comparable cohorts (Cohort A = 114 patients and Cohort B = 112). The study found a significant reduction in SSIs among patients receiving the PHMB care bundle compared with those receiving conventional care (1.8% vs. 7.1%, p = 0.048). The comprehensive nature of the PHMB care bundle, including educational programs, preoperative and postoperative antimicrobial treatments, and consistent application of best practices, was instrumental in achieving these outcomes. Implementing antimicrobial care bundles could significantly reduce SSIs in paediatric cardiac surgery. Future research is needed to refine the tested bundle with prospective approaches.
Ebstein's anomaly is a rare congenital heart defect characterized by tricuspid valve malformation and right ventricular myopathy. The "cone procedure," introduced by Dr. José Pedro da Silva, represents a major advancement in the repair of Ebstein's anomaly, achieving near-anatomical tricuspid valve reconstruction. We describe the case of a 23-year-old-female with Carpentier type B Ebstein's anomaly, severe tricuspid regurgitation and Wolff-Parkinson-White syndrome, who underwent cone reconstruction combined with right ventricular resection. Detailed preoperative imaging guided the procedure, ensuring precise leaflet mobilization, delamination and cone creation. The atrialized right ventricular portion was reduced via triangular resection, preserving right ventricular geometry. An annuloplasty ring stabilized the repair. Postoperatively, the patient demonstrated excellent recovery, with trivial regurgitation and preserved right ventricular function confirmed at the one-year follow-up. This case highlights the reproducibility and effectiveness of the cone repair, supporting its role as the primary surgical approach for Ebstein's anomaly. Key factors for success include meticulous leaflet delamination, ensuring a complete 360° cone structure and maintaining RV geometry. Although long-term outcomes require further evaluation, the cone procedure offers superior valve competence and symptom relief compared to traditional repairs, minimizing the need for reoperation.
AIMS:Anomalous Aortic Origin of Coronary Arteries (AAOCA) is associated with myocardial ischaemia and sudden cardiac death, particularly in young athletes. Although inducible myocardial ischaemia investigation is generally recommended, there is no clear indication of the most appropriate stress test, as the ECG exercise stress test presents low diagnostic accuracy. Dobutamine-stress cardiac magnetic resonance (dsCMR) has been proposed as a promising diagnostic tool, but its application has been limited to paediatric populations. This study aimed to evaluate the presence of inducible myocardial ischaemia assessed by dsCMR in AAOCA patients, regardless of age, and to identify factors associated with inducible myocardial ischaemia. METHODS AND RESULTS:In this single-centre observational study, 100 AAOCA patients who underwent dsCMR were enrolled from 2015 to 2024. dsCMR resulted positive in 14 cases, all showing perfusion abnormalities, with only one presenting segmental wall motion abnormalities. dsCMR was found to be safe without major adverse events. Factors significantly associated with inducible ischaemia included intramural course, the presence of ischaemic late gadolinium enhancement (LGE), and a history of acute coronary syndrome (ACS). Nine patients (9%) showed small areas of ischaemic LGE within the AAOCA territory, and they were older with a higher incidence of arterial hypertension, smoking habits and previous ACS episodes. CONCLUSION:dsCMR is a reliable test for the evaluation of myocardial ischaemia in AAOCA, especially for those with intramural course, ischaemic LGE, and previous ACS episodes. Although rare, older patients may have a myocardial ischaemic scar without alteration of ventricular function.
Background:Survival rates for patients with congenital heart disease (CHD) have improved, but complications like pulmonary regurgitation (PR) often require re-interventions. Transcatheter pulmonary valve implantation (TPVI) with self-expandable valves, such as the Venus P-Valve, has broadened treatment options. Accurate procedural planning, particularly valve sizing, remains a significant challenge. Mixed Reality (MxR) technology enables a patient-specific approach that enhances procedural planning accuracy. Aim:To evaluate the use of MxR in planning Venus-P valve implantation. Materials and methods:This study included patients undergoing Venus P-Valve implantation with holographic models generated from CT data using ARTICOR® software from January 2023 to June 2024. Two independent operators used these models for procedural planning. Concordance between operators was assessed. Predictions were compared with implanted valve dimensions to evaluate concordance. Results:Of 29 eligible patients, 26 underwent successful Venus-P valve implantation. Concordance between the operators reached 60 % (n = 15/26) for valve diameter and over 88 % (n = 23/26) for valve length. Holographic models achieved 96 % (n = 25/26) concordance in predicting valve length, type of the approach (92 %) and 50 % (n = 13/26) concordance for diameter. Discussion:Holographic models enhanced procedural planning, enabling better visualization and collaborative decision-making. While highly effective for valve length predictions, and type of the approach limitations in predicting valve diameter highlight the need for improved methods, such as computational modelling or machine learning. Conclusion:Patient-specific holographic models are promising tools for TPVI planning. Advancements in technology and interdisciplinary collaboration are critical to overcoming current limitations and advancing procedural planning and related outcomes in interventional cardiology. Condensed abstract:This study assesses the use of mixed reality (MxR) technology for procedural planning in transcatheter pulmonary valve implantation (TPVI) with the Venus P-Valve. Holographic models were created from CT data to aid in valve sizing and implantation strategies, with two operators comparing measurements for valve diameter and length. Among 26 patients who underwent successful implantation, concordance between operators was 60 % for valve diameter and 88 % for valve length. Predictions using holographic models showed 96 % concordance for implanted valve length, type of the approach (92 %) and 50 % concordance for valve diameter. These results highlight the potential of MxR for improving TPVI planning, though the limited accuracy for valve diameter suggests a need for further advancements, such as computational modeling or machine learning, to optimize procedural outcomes in interventional cardiology.