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.

Pulmonary arterial hypertension related to congenital heart disease with a left-to-right shunt: phenotypic spectrum and approach to management.

Patients with pulmonary hypertension associated with a left-right shunt include a wide spectrum of pathophysiological substrates, ranging from those characterized by pulmonary over-circulation to those with advanced pulmonary vascular disease. The former group may benefit from shunt repair in carefully selected cases but, when advanced pulmonary vascular disease has developed, defect closure should be avoided, and pulmonary vasodilators may be used to improve effort tolerance and hemodynamics. There is a paucity of evidence, however, to support decision-making in the care of these patients. We discuss the principles of management in patients with pulmonary hypertension and a predominant left-right shunt. The recommendations and statements made in this paper are based on pathophysiological considerations and expert opinion.

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.

Transcatheter closure of atrial septal defect using intracardiac echocardiography: a two-center, retrospective study.

AIMS: We present the experience and long-term results of intracardiac echocardiography (ICE)-guided closure of ostium secundum atrial septal defects (ASDs) in two Italian centers and investigate its systematic applicability as the gold standard in routine clinical practice. METHODS: We retrospectively evaluated all consecutive patients who underwent an ASD percutaneous closure procedure from March 2008 to February 2020. All patients underwent a preprocedural transesophageal echocardiography (TEE) evaluation. The closures were carried out under fluoroscopic and ICE guidance. A follow-up visit was performed at 1, 3 and 12 months, followed by telephone evaluations approximately every 2 years. RESULTS: Sixty-six patients (29% male individuals), mean age 43 ±â€Š16 years, were treated. In 15 cases, the TEE defect diameter was less than 10 mm, and in 8 of these patients, the ICE intraprocedural sizing increased the maximum diameter by more than 5 mm. Sizing balloon of the defect was performed in 51 cases; 2 patients received an ASD 38 mm device. Eight patients had multiple defects; in three of these, it was necessary to apply two devices. Four patients showed nonsignificant residual shunt; no complications related to the use of ICE were observed. One patient presented the migration of the ASD device into the abdominal aorta, percutaneously retrieved with a snare. No major complications were recorded during the entire follow-up period. CONCLUSION: This study confirms that ICE monitoring during ASD percutaneous closure is well tolerated and effective; it might be achievable as a routine gold standard by operators willing to use ICE systematically in all transcatheter closure interventions of interatrial communications.

Morbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life.

AIMS: To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking. METHODS AND RESULTS: Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35-48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0-15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4-10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04-1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75-10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23-7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05-70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis. CONCLUSION: Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment.

This study sheds light on the complex medical journey of adults living with the outcomes of Fontan surgery­a procedure performed in early childhood. These individuals have reached the milestone of their forties and beyond, yet they confront an array of significant health challenges that necessitate lifelong, individualized congenital heart disease care. The key findings are as follows: While adults with Fontan circulation are living longer, they are at high risk of death, mainly due to heart failure. They also face a host of other health issues, including the need for additional surgeries or interventions. Nearly two-thirds have experienced some form of heart rhythm problem, and a substantial number eventually require evaluation for a heart transplant.Heart transplants within this group were rare, which may be linked to the various barriers to transplantation in the Fontan population. Moreover, those with multiple indicators of advanced disease have a heightened risk of life-threatening events, reinforcing the critical need for personalized and continuous specialist care designed to meet their distinct health requirements.

Long-term effects of physical training on cardiopulmonary exercise parameters in young patients with congenital heart diseases.

INTRODUCTION: Physical activity is recognised as an important intervention in patients with CHD. However, more data on the actual magnitude of physical training impact on functional capacity in this group of patients are still warranted. We aim to assess effort tolerance in a contemporary cohort of patients with congenital heart disease, regularly following a training programme, in comparison with a matched control group. METHODS: Patients with CHD followed at the sports medicine department, who had undergone cardiopulmonary exercise test between 2011 and 2019, were included. Variables recorded were maximum workload, absolute and indexed maximum oxygen consumption, maximum heart rate, absolute and indexed maximum O2 pulse, ventilatory equivalent of CO2 and oxygen consumption/Work. Trend of cardiopulmonary parameters was analysed over time. Maximal workload, maximum oxygen consumption and ventilatory equivalent of CO2 were compared with a control group of patients with a more sedentary lifestyle, matched for diagnosis, gender, age, and body mass index. RESULTS: Among one hundred and eleven patients, 73 males (66%) were analysed. Median age was 14 (12-17) years. Twenty-nine patients (27%) were practising sports at competitive level. Maximum oxygen consumption and oxygen consumption % of maximum predicted were not significantly different at follow-up as compared with baseline. Follow-up of maximum oxygen consumption was 38.2 ± 9 ml/kg/min versus 38.6 ± 9.2 ml/kg/min (p = NS) and follow-up of %oxygen consumption was 88 ± 20 versus 87 ± 15 (p = NS). Ventilatory equivalent of CO2 significantly improved in the last test as compared with the baseline: 30 ± 4 versus 33 ± 5 (p = 0.002). As compared with the control group, trained patients displayed a significantly higher maximum workload and oxygen consumption, while ventilatory equivalent of CO2 was not significantly different. CONCLUSIONS: In our cohort, patients following a regular training programme displayed a significantly higher functional capacity as compared with not trained control group, irrespective of NYHA class. Objective functional capacity was stable over a median follow-up of 3 years.

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.

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.

Holographic Techniques as a Novel Method for Intervention Planning: A Tertiary Centres Experience.

Advanced holographic visualization techniques are becoming increasingly important in clinical practice, not only for diagnostic purposes but also in the planning of interventional or surgical procedures. The traditional approach for visualizing anatomic structures is based on standard imaging modalities such as echocardiography, cardiac magnetic resonance (CMR) and cardiac CT scan (CCT) which, however, can only provide two-dimensional (2D) images thus limiting 3D perception. Many recent studies have shown that the use of 3D imaging modalities such as augmented reality, virtual reality, mixed reality and holography improve the short and long-term outcome of percutaneous or surgical procedures. In this article, we report our experience on the use of the hologram in different clinical scenarios and in the field of university education.

Acute myocarditis as first presentation of severe ulcerative colitis in a young man. Multidisciplinary management and long-term follow-up.

Myocarditis is a rare extra-intestinal complication of inflammatory bowel disease (IBD), in particular, ulcerative colitis.We report a case of acute myocarditis as first manifestation of severe ulcerative colitis. A 22-year-old man was admitted with fever, bloody diarrhea, and fatigue. He had suffered from frequent bloody diarrhea, abdominal pain, fatigue, and weight loss for one month. A 12-lead-electrocardiogram showed sinus rhythm with QRS fragmentation and T waves inversion. High sensitivity troponin-I was elevated and the echocardiogram showed a mild pericardial effusion and inferior hypokinesia with normal ejection fraction. Cardiac magnetic resonance disclosed late enhancement in the inferior wall, corroborating the hypothesis of myocarditis. One week later, a colonoscopy revealed severe ulcerative extensive colitis (Mayo subscore 3). 5-aminosalicylic acid (mesalazine) and systemic steroid were started with good clinical and biochemical response. The following days the patient developed mesalazine hepatic and pancreatic induced toxicity requiring drug discontinuation and strict multi-disciplinary follow-up. At 7 months follow-up intestinal symptoms were well controlled with complete normalization of liver and pancreatic enzymes. Transthoracic echocardiography showed normal biventricular function and pericardial effusion resolution.This case underscores the importance of a high suspicion for extra intestinal involvement in patients with IBD. These complications may be multifactorial and need multidisciplinary management. Learning objective: •When a patient was first-time diagnosed with a severe form of inflammatory bowel disease or has a disease relapse, bear in mind myocarditis as possible extra intestinal manifestation.•Multidisciplinary management is crucial to ensure the best level of care and follow-up in a such challenging and insidious clinical picture.

First case reported of COVID-19 infection in an adult patient with Ellis-van Creveld Syndrome.

Ellis-van Creveld syndrome (EVC) is a rare autosomal recessive disorder, the features of the syndrome are: chondral and ectodermal dysplasia characterized by short ribs, polydactyly, growth retardation resulting in dwarfism, teeth and craniofacial abnormalities and heart defects (mostly endocardial cushions and atrial septal defects). We describe the first case reported of COVID-19 infection in a 24-years-old girl, diagnosed with EVC syndrome. The patient suffered only from a mild illness, she remained stable with normal saturation without need of neither respiratory support nor specific therapy and she was rapidly discharged. This case appraises the pathophysiological interplay between different specific prognostic variable in a syndromic patient with congenital heart disease and COVID-19. In patients with congenital heart disease, comorbidities related to syndromic picture may affect the clinical course of COVID-19 infection regardless of the anatomic complexity.

Early Diagnosis of Acute Myocarditis in the ED: Proposal of a New ECG-Based Protocol.

The diagnosis of acute myocarditis (AM) is based on a multi-parametric assessment including clinical presentation, ECG, imaging and biomarkers. Fragmented QRS (fQRS) might be an additional diagnostic sign in patients with proven AM. The main objective of this study was to assess the diagnostic yield of fQRS in patients with suspected AM presenting to the emergency department (ED). Patients admitted between January 2016 and March 2021 with a proven diagnosis of AM, according to clinical, cardiac magnetic resonance (CMR) and/or histologic criteria, were included in the analysis. In total, 51 patients were analyzed (41 men, 78%), with a median age of 36 (29-45) years. Thirty-three (65%) patients had prodromal flu-like symptoms. Patients presented to the ED mostly complaining of chest pain (68%) and palpitations (21%). Seven (14%) patients experienced cardiac arrest, one of whom died. At presentation, 40 patients (78%) displayed fQRS, and 10 (20%) presented ventricular arrhythmias. All the surviving patients underwent CMR and displayed late gadolinium enhancement (LGE). ECG leads showed that fQRS matched the LGE distribution in 38 patients (95%). The presence of fQRS is a simple clinical bedside tool to support the initial suspect of AM in the emergency department and to guide the most appropriate clinical workup.

A case report of late physiologic repair of congenitally corrected transposition of the great arteries and pulmonary stenosis in a severely cyanotic patient: better late than never.

BACKGROUND: Patients with congenitally corrected transposition of great arteries (ccTGA) not infrequently seek medical attention for the first time late in life. Optimal management of natural history ccTGA is debated and must be tailored. CASE SUMMARY: A 38-year-old male patient was referred to our centre because of severe cyanosis and worsening dyspnoea. Investigations disclosed situs solitus, mesocardia, double discordance, large ventricular septal defect (VSD), severe pulmonary stenosis, and no significant atrio-ventricular valves regurgitation. The patient underwent physiologic repair: VSD closure, placement of a left ventricle to pulmonary artery conduit, and epicardial atrio-biventricular pacemaker implantation. The conduit was intentionally undersized to promote tricuspid valve continence. Post-operative course was uneventful, transthoracic echocardiography showed good biventricular function without significant tricuspid regurgitation. At 1 month after discharge, the patient is in New York Heart Association Class II. DISCUSSION: Management of late presenter patients with ccTGA depends on the associated lesion and estimation of surgical risk. In selected patients markedly symptomatic physiologic repair is a rationale option, providing a normal saturation and biventricular circulation with a significantly lower surgical risk as compared with an anatomic repair.

Oncogenesis in patients with congenital heart disease: A possible role of the neural crest.

Patients with congenital heart disease (CHD) seem to have a higher risk for specific malignancies. We hypothesize a pathogenetic link between particular congenital heart defects and cancer originating from specific cellular lineages. We report a series of patients, followed in two high-volume referral centers, with CHD involving neural crest-derived structures who developed cancer later in life. Fourteen patients (five female) developed neoplasia with a cellular origin embryologically linked to the neural crest between 2010 and 2020. If confirmed on larger datasets, this observation might support the hypothesis of common embryogenetic pathway suggesting tailored surveillance of a specific subset of patients.