Holography-guided procedural planning for modifying Venus P-valve implantation technique in patients with left pulmonary artery stents: a case-series.

Background: Venus P-valve™ (Venus Medtech, Hangzhou, China) is a self-expandable bioprosthetic valve that can be transcatheter-implanted in native right ventricular outflow tract (RVOT) patients. Valve implantation is technically challenging. Due to the implantation technique, left pulmonary artery (LPA) stents represent a relative contraindication to Venus P-valve. In this case series, we describe our experience in implanting Venus P-valve in patients with previous LPA stents and the use of holographic models to facilitate procedural planning. Methods and results: From January to October 2023, 17 patients were scheduled for Venus P-Valve implantation. 16/17 (94%) patients were successfully implanted. 3/16 (18.7%) patients underwent Venus P-valve implantation with LPA stents. All patients underwent pre-operative CT scan. CT data set were employed to create three-dimensional (3D) holographic models (Artiness, Milan, Italy) of the entire heart, which were useful to plan valve implantation with a modified technique. Procedural success rate was 100%. No procedural complications occurred. All three patients presented good haemodynamic and angiographic results at discharge and follow-up visits. Conclusion: This case-series underscores the feasibility of Venus P-valve implantation in patients with previous LPA stents. The use of holographic models facilitated procedural planning in these challenging anatomical scenarios.

The Appraisal of Adults with Congenital Heart Disease: Lesson from Comparison of Surgical Outcomes.

The population of adults with congenital heart disease (ACHD) is constantly growing. There seems to be a consensus that these patients are difficult to manage especially if compared to patients with acquired heart disease. The aim of this study is to compare outcomes and results of cardiac surgery in ACHD patients with a reference population of adults with acquired cardiac disease. Retrospective study of 5053 consecutive patients older than 18 years hospitalized for cardiac surgery during a 5-years period in our Institution. Two groups of patients were identified. Group I: 419 patients operated for congenital heart disease; Group II: 4634 patients operated for acquired heart disease. In each Group were identified low, medium, and high-risk patients, according to validated scores. Right ventricular outflow tract surgery was the most frequent procedure in Group I, while coronary artery by-pass grafting was the most common in Group II. Patients with ACHD were younger (37.8 vs. 67.7 years), with higher number of previous operations (32.1% vs. 6.9%), had longer post-ICU hospital stay (11 vs. 8 days) but had lower ICU stay (1 vs. 2 days), shorter assisted mechanical ventilation (12 vs. 14 h) and lower surgical mortality (1 vs. 3.7%) (all p < 0.001). No differences were found in term of post-operative complications (12.4 vs. 15%). The surgical treatment of ACHD patients can be done with excellent results and if compared with acquired cardiac disease patients they have better results with shorter ICU stay and lower mortality.

Ebstein's anomaly in children and adults: multidisciplinary insights into imaging and therapy.

Although survival has significantly improved in the last four decades, the diagnosis of Ebstein's anomaly is still associated with a 20-fold increased risk of mortality, which generally drops after neonatal period and increases subtly thereafter. With increasing age of presentation, appropriate timing of intervention is challenged by a wide spectrum of disease and paucity of data on patient-tailored interventional strategies. The present review sought to shed light on the wide grey zone of post-neonatal Ebstein's manifestations, highlighting current gaps and achievements in knowledge for adequate risk assessment and appropriate therapeutic strategy.A 'wait-and-see' approach has been adopted in many circumstances, though its efficacy is now questioned by the awareness that Ebstein's anomaly is not a benign disease, even when asymptomatic. Moreover, older age at intervention showed a negative impact on post-surgical outcome.In order to tackle the extreme heterogeneity of Ebstein's anomaly, this review displays the multimodality imaging assessment necessary for a proper anatomical classification and the multidisciplinary approach needed for a comprehensive risk stratification and monitoring strategy. Currently available predictors of clinical outcome are summarised for both operated and unoperated patients, with the aim of supporting the decisional process on the choice of appropriate therapy and optimal timing for intervention.

Modern tools in congenital heart disease imaging and procedure planning: a European survey.

AIMS: Congenital heart diseases (CHDs) often show a complex 3D anatomy that must be well understood to assess the pathophysiological consequences and to guide therapy. Three-dimensional imaging technologies have the potential to enhance the physician's comprehension of such spatially complex anatomies. Unfortunately, due to the new introduction in clinical practice, there is no evidence on the current applications. We conducted a survey to examine how 3D technologies are currently used among CHD European centres. METHODS: Data were collected using an online self-administered survey via SurveyMonkey. The questionnaire was sent via e-mail and the responses were collected between January and June 2022. RESULTS: Ninety-eight centres correctly completed the survey. Of these, 22 regularly perform 3D rotational angiography, 43 have the availability to print in-silico models, and 22 have the possibility to visualize holographic imaging/virtual reality. The costs were mostly covered by the hospital or the department of financial resources. CONCLUSION: From our survey, it emerges that these technologies are quite spread across Europe, despite not being part of a routine practice. In addition, there are still not enough data supporting the improvement of clinical management for CHD patients. For this reason, further studies are needed to develop clinical recommendations for the use of 3D imaging technologies in medical practice.

Overcoming Underpowering in the Outcome Analysis of Repaired-Tetralogy of Fallot: A Multicenter Database from the CMR/CT Working Group of the Italian Pediatric Cardiology Society (SICPed).

Background: Managing repaired tetralogy of Fallot (TOF) patients is still challenging despite the fact that published studies identified prognostic clinical or imaging data with rather good negative predictive accuracy but weak positive predictive accuracy. Heterogeneity of the initial anatomy, the surgical approach, and the complexity of the mechanism leading to dilation and ventricular dysfunction explain the challenge of predicting the adverse event in this population. Therefore, risk stratification and management of this population remain poorly standardized. Design: The CMR/CT WG of the Italian Pediatric Cardiology Society set up a multicenter observational clinical database of repaired TOF evaluations. This registry will enroll patients retrospectively and prospectively assessed by CMR for clinical indication in many congenital heart diseases (CHD) Italian centers. Data collection in a dedicated platform will include surgical history, clinical data, imaging data, and adverse cardiac events at 6 years of follow-up. Summary: The multicenter repaired TOF clinical database will collect data on patients evaluated by CMR in many CHD centers in Italy. The registry has been set up to allow future research studies in this population to improve clinical/surgical management and risk stratification of this population.

Left atrial remodeling in hypertrophic cardiomyopathy and Fabry disease: A CMR-based head-to-head comparison and outcome analysis.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) and Fabry disease cardiomyopathy (FD) are phenocopies, as they show left ventricular hypertrophy (LVH). The left atrium (LA) is emerging as a potential marker of disease severity in both cardiomyopathies. The present study compares HCM and FD cardiomyopathy with similar degree of LVH, exploring LA morpho-functional parameters and the correlates of clinical outcome. METHODS: We performed a comprehensive CMR-based comparison between 30 HCM and 30 FD patients matched on age, sex, BSA, LV mass and major cardiovascular risk factors affecting LA remodeling (arterial hypertension and diabetes). 30 healthy controls were also included. CMR feature tracking (CMR-FT) analysis, T1 mapping and conventional parameters were evaluated. Patients also underwent transthoracic echocardiography for LV diastolic function assessment. Clinical events at follow-up were collected (atrial and ventricular events, bradyarrhythmia, heart failure (HF) hospitalization and death). RESULTS: HCM patients showed greater LA remodeling compared to FD patients, namely higher LA end-systolic volume index (LAVi max), lower LA-ejection fraction (LA-EF) and worse reservoir (εs) and booster function (εa) (all p < 0.05). Accordingly, these parameters have demonstrated good potential for distinguishing between FD and HCM (AUC 0.68-0.73, all p < 0.05), with LAVi max being an independent predictor for HCM diagnosis (OR 1.07, 95%CI 1.011-1.132, p 0.02). Moreover, in HCM patients a significant association between εs and HF occurrence was observed at 2-year follow-up (OR 0.85, 95%CI 0.72-0.99, p 0.04). CONCLUSIONS: In HCM, LA remodeling is greater than in FD cardiomyopathy with similar LVH, and reservoir strain is associated with HF at follow-up.

Overview of Long-Term Outcome in Adults with Systemic Right Ventricle and Transposition of the Great Arteries: A Review.

The population of patients with a systemic right ventricle (sRV) in biventricular circulation includes those who have undergone an atrial switch operation for destro-transposition of the great arteries (d-TGA) and those with congenitally corrected transposition of the great arteries (ccTGA). Despite the life expectancy of these patients is significantly increased, the long-term prognosis remains suboptimal due to late complications such as heart failure, arrhythmias, and premature death. These patients, therefore, need a close follow-up to early identify predictive factors of adverse outcomes and to implement all preventive therapeutic strategies. This review analyzes the late complications of adult patients with an sRV and TGA and clarifies which are risk factors for adverse prognosis and which are the therapeutic strategies that improve the long-term outcomes. For prognostic purposes, it is necessary to monitor sRV size and function, the tricuspid valve regurgitation, the functional class, the occurrence of syncope, the QRS duration, N-terminal pro B-type natriuretic peptide levels, and the development of arrhythmias. Furthermore, pregnancy should be discouraged in women with risk factors. Tricuspid valve replacement/repair, biventricular pacing, and implantable cardioverter defibrillator are the most important therapeutic strategies that have been shown, when used correctly, to improve long-term outcomes.

The Effect of Holographic Heart Models and Mixed Reality for Anatomy Learning in Congenital Heart Disease: An Exploratory Study.

In this paper, we present an exploratory study on the potential impact of holographic heart models and mixed reality technology on medical training, and in particular in teaching complex Congenital Heart Diseases (CHD) to medical students. Fifty-nine medical students were randomly allocated into three groups. Each participant in each group received a 30-minute lecture on a CHD condition interpretation and transcatheter treatment with different instructional tools. The participants of the first group attended a lecture in which traditional slides were projected onto a flat screen (group "regular slideware", RS). The second group was shown slides incorporating videos of holographic anatomical models (group "holographic videos", HV). Finally, those in the third group wore immersive, head-mounted devices (HMD) to interact directly with holographic anatomical models (group "mixed reality", MR). At the end of the lecture, the members of each group were asked to fill in a multiple-choice questionnaire aimed at evaluating their topic proficiency, as a proxy to evaluate the effectiveness of the training session (in terms of acquired notions); participants from group MR were also asked to fill in a questionnaire regarding the recommendability and usability of the MS Hololens HMDs, as a proxy of satisfaction regarding its use experience (UX). The findings show promising results for usability and user acceptance.

The predictive role of right ventricular late gadolinium enhancement in patients with tetralogy of Fallot undergoing pulmonary valve replacement.

BACKGROUND: Our purpose was to evaluate the correlations between right ventricular (RV) late gadolinium enhancement (LGE) at cardiac magnetic resonance (CMR) in patients with tetralogy of Fallot (ToF) scheduled for pulmonary valve replacement (PVR) and post-PVR functional data. METHODS: We retrospectively reviewed ToF patients scheduled for PVR who underwent two CMR examinations at our institution, one before the procedure (CMR-0), including contrast-enhanced sequences, and one after the procedure (CMR-1). Functional left and RV data were obtained by segmenting short-axis stacks on both CMR examinations, and normalised variations were calculated by dividing differences between CMR-1 and CMR-0 by the intercurring time interval, whereas the RV scar burden was assessed on CMR-0 LGE sequences both semiquantitatively and quantitatively. Data were reported as median and interquartile range, differences were appraised with the Mann-Whitney U test, while correlations were assessed with Spearman's ρ. RESULTS: Fifteen patients with a median age of 25 years (16-29), including 9 (60%) males, with a median time interval between CMR-0 and CMR-1 of 17 months (12-23), were retrospectively reviewed. The semiquantitative LGE score at CMR-0 was 7 (6-9), and LGE volume was 4.49 mL (3.70-5.78), covering 5.63% (4.92-7.00) of the RV. RV LGE score showed a moderate positive correlation with the normalised variation of RV stroke volume (ρ = 0.662, p = 0.007) and a borderline moderate positive correlation with the normalised variation of RV end-diastolic indexed volume (ρ = 0.513, p = 0.050). CONCLUSIONS: The assessment of RV LGE before PVR may provide insights on post-PVR functional data, potentially facilitating a patient-tailored treatment pathway.

Dynamic changes of repolarization abnormalities in takotsubo cardiomyopathy.

OBJECTIVE: This study analyses dynamicchangesin dispersion of ventricularrepolarization over the time course of takotsubo cardiomyopathy (TC), and their relationships with clinical features and life-threatening arrhythmias. METHODS AND RESULTS: All consecutive patients admitted to our division between January 2008 and December 2011 with a diagnosis of TC were analysed. Patients with prior myocardial infarction, symptoms-onset-to-admission time greater than 12 hours, an implanted pacemaker, or under treatment with drugs affecting QTinterval, were excluded. Standard 12-lead ECG recordings during the acute, subacute and chronic phases were collected for each patient. Twenty-four patients (23 women, 63 +/- 14 years) were includedin our analysis. Only one patient experienced ventricular arrhythmias (4.2%). Significant increases were observed in QT and QTc intervals (from 420 ?423 to 505 +/- 66 ms, P < 0.00001, and from 479 +/- 33 to 551 +/- 51 ms, P < 0.00001, respectively), QTdispersion (from 59 +/- 18 to 100 +/- 44 ms, P=0.0006), Tpeak-to-Tend (from 82 +/- 20 to 123 +/- 39 ms, P=0.00006) and Tpeak-to-Tend/QT (from 0.20 +/- 0.33 to 0.26 +/- 0.57, P=0.0003) during the subacute phase. All these parameters returned to baseline values in the chronic phase and did not show any significant differences between the acute and chronic phases. CONCLUSIONS: A marked increase in QTc, QT dispersion, Tpeak-to-Tend and Tpeak-to-Tend/QT was observed during the subacute phase; this increase was transient and reverted in allpatients before hospital discharge. Ofnote, these findings were not associated with an increased risk of life-threatening arrhythmias.

Acute electrocardiographic differences between Takotsubo cardiomyopathy and anterior ST elevation myocardial infarction.

BACKGROUND: The aim of this study was to compare ECG findings between anterior ST elevation myocardial infarction (STEMI) and Takotsubo cardiomyopathy (TC) in a similar sample of postmenopausal women. METHODS: Between 2008 and 2011, 27 patients with TC were retrospectively enrolled and matched with 27 STEMI patients with the same age and sex taken from the prospective database of our laboratory. RESULTS: The absence of abnormal Q waves, the ST depression in aVR and the lack of ST elevation in V1 were significantly associated with TC (respectively: 52% vs 18%, p=0.01; 47% vs 11%, p=0.01; 80% vs 41%, p=0.01). The combination of these ECG findings identified TC with a specificity of 95% and a positive predictive value of 85.7%. CONCLUSIONS: The ECG on admission may be useful to distinguish TC from anterior STEMI. The combination of three ECG findings identifies patients with TC with high specificity and positive predictive value.