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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., Competing Interests: Conflict of interest: A.C. has received educational grants, personal fees, and non-financial support from Janssen-Cilag Limited. K.D. has received non-financial support from Janssen-Cilag Limited and has been a consultant to and received grants and personal fees from Janssen-Cilag Limited, Pfizer, GlaxoSmithKline, and Bayer/MSD. All the remaining authors have no relationships with industry to declare., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Congenital heart disease (CHD) is often comprised of complex three-dimensional (3D) anatomy that must be well understood to assess the pathophysiological consequences and guide therapy. Thus, detailed cardiac imaging for early detection and planning of interventional and/or surgical treatment is paramount. Advanced technologies have revolutionized diagnostic and therapeutic practice in CHD, thus playing an increasing role in its management. Traditional reliance on standard imaging modalities including echocardiography, cardiac computed tomography (CT) and magnetic resonance imaging (MRI) has been augmented by the use of recent technologies such as 3D printing, virtual reality, augmented reality, computational modelling, and artificial intelligence because of insufficient information available with these standard imaging techniques. This has created potential opportunities of incorporating these technologies into routine clinical practice to achieve the best outcomes through delivery of personalized medicine. In this review, we provide an overview of these evolving technologies and a new approach enabling physicians to better understand their real-world application in adult CHD as a prelude to clinical workflow implementation., (© The Author(s) 2022. Published by Oxford University Press on behalf of European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Atrial septal defects (ASDs) represent the most common congenital heart defect diagnosed in adulthood. Although considered a simple defect, challenges in optimal diagnostic and treatment options still exist due to great heterogeneity in terms of anatomy and time-related complications primarily arrhythmias, thromboembolism, right heart failure and, in a subset of patients, pulmonary arterial hypertension (PAH). Atrial septal defects call for tertiary expertise where all options may be considered, namely catheter vs. surgical closure, consideration of pre-closure ablation for patients with atrial tachycardia and suitability for closure or/and targeted therapy for patients with PAH. This review serves to update the clinician on the latest evidence, the nuances of optimal diagnostics, treatment options, and long-term follow-up care for patients with an ASD., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: journals.permissions@oup.com.)

Adult congenital heart disease (ACHD) patients represent a growing population with increasing use of acute emergency department (ED) care. Providing comprehensive ED care necessitates an understanding of the most common clinical scenarios to improve morbidity and mortality in this population. The aim of this position document is to provide a consensus regarding the management of the most common clinical scenarios of ACHD patients presenting to the ED., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: journals.permissions@oup.com.)

Chloracne, also known as metabolizing acquired dioxin-induced skin hamartomas (MADISH), is a rare disfiguring disease related to dioxin exposure. There is a paucity of literature on the clinical manifestations and pathogenesis of chloracne/MADISH. The aim of this study was to assess the clinical features of this very unusual acneiform eruption and to explore the pathogenesis of the disease. This was a retrospective, observational report study was conducted on five patients belonging to the same nuclear family (father, mother and three children) and a relative (father's brother) living in the same house. Histopathological, immunohistochemical, laboratory and toxicological analyses were performed for all patients. The results suggest that CYP1A1 in human skin is a diagnostic biomarker in chloracne, and was positive for all the patients in our sample. Tetrachlorodibenzo-p-dioxin is the most investigated dioxin responsible for chloracne; however, several other agonists, whether dioxin-like or not, can activate the aryl hydrocarbon receptor. To our knowledge, this Italian case series is the first study to suggest polychlorinated biphenyls as a possible cause of an overstimulation of aryl hydrocarbons causing the consequent acneiform eruption., (© 2021 British Association of Dermatologists.)

We are witnessing an unparalleled pandemic caused by the novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) associated with coronavirus disease 2019 (COVID-19). Current data show that SARS-CoV-2 results in mild flu-like symptoms in the majority of healthy and young patients affected. Nevertheless, the severity of COVID-19 respiratory syndrome and the risk of adverse or catastrophic outcomes are increased in patients with pre-existing cardiovascular disease. Patients with adult congenital heart disease (ACHD)-by definition-have underlying cardiovascular disease. Many patients with ACHD are also afflicted with residual haemodynamic lesions such as valve dysfunction, diminished ventricular function, arrhythmias or cyanosis, have extracardiac comorbidities, and face additional challenges regarding pregnancy. Currently, there are emerging data of the effect of COVID-19 on ACHD patients, but many aspects, especially risk stratification and treatment considerations, remain unclear. In this article, we aim to discuss the broad impact of COVID-19 on ACHD patients, focusing specifically on pathophysiology, risk stratification for work, self-isolation, hospitalization, impact on pregnancy, psychosocial health, and longer-term implications for the provision of ACHD care., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2020. For permissions, please email: journals.permissions@oup.com.)

Patent foramen ovale (PFO) is implicated in the pathogenesis of a number of medical conditions but to date only one official position paper related to left circulation thromboembolism has been published. This interdisciplinary paper, prepared with the involvement of eight European scientific societies, reviews the available evidence and proposes a rationale for decision making for other PFO-related clinical conditions. In order to guarantee a strict evidence-based process, we used a modified grading of recommendations, assessment, development, and evaluation (GRADE) methodology. A critical qualitative and quantitative evaluation of diagnostic and therapeutic procedures was performed, including assessment of the risk/benefit ratio. The level of evidence and the strength of the position statements were weighed and graded according to predefined scales. Despite being based on limited and observational or low-certainty randomised data, a number of position statements were made to frame PFO management in different clinical settings, along with suggestions for new research avenues. This interdisciplinary position paper, recognising the low or very low certainty of existing evidence, provides the first approach to several PFO-related clinical scenarios beyond left circulation thromboembolism and strongly stresses the need for fresh high-quality evidence on these topics., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: journals.permissions@oup.com.)

The presence of a patent foramen ovale (PFO) is implicated in the pathogenesis of a number of medical conditions; however, the subject remains controversial and no official statements have been published. This interdisciplinary paper, prepared with involvement of eight European scientific societies, aims to review the available trial evidence and to define the principles needed to guide decision making in patients with PFO. In order to guarantee a strict process, position statements were developed with the use of a modified grading of recommendations assessment, development, and evaluation (GRADE) methodology. A critical qualitative and quantitative evaluation of diagnostic and therapeutic procedures was performed, including assessment of the risk/benefit ratio. The level of evidence and the strength of the position statements of particular management options were weighed and graded according to predefined scales. Despite being based often on limited and non-randomised data, while waiting for more conclusive evidence, it was possible to conclude on a number of position statements regarding a rational general approach to PFO management and to specific considerations regarding left circulation thromboembolism. For some therapeutic aspects, it was possible to express stricter position statements based on randomised trials. This position paper provides the first largely shared, interdisciplinary approach for a rational PFO management based on the best available evidence., (The article has been co-published with permission in the European Heart Journal and EuroIntervention. All rights reserved. © The Author(s) 2018. The articles are identical except for minor stylistic and spelling differences in keeping with each journal's style. Either citation can be used when citing this article.)

Objectives: Tricuspid valve (TV) surgery in the adult with congenital heart disease (ACHD) is a frequently performed procedure. The aim of this study was to analyse postoperative and medium-term outcomes., Methods: We conducted a single-centre retrospective study of patients with ACHD who underwent TV surgery (January 2000-December 2016); patients with Ebstein's anomalies were excluded. Operative and clinical records were reviewed. Outcomes considered were survival, grade of insufficiency/stenosis and TV reoperation at follow-up., Results: A total of 128 patients with ACHD had TV surgery for functional regurgitation (n = 95), dysplasia (n = 23) and systemic TV (n = 10). Median age was 40.8 years [interquartile range (IQR) 25.3]; 55.5% were men. Preoperative regurgitation was classified as mild (n = 8), moderate (n = 47) and severe (n = 70). The TV was repaired in 109 as follows: ring annuloplasty (n = 43), de Vega annuloplasty (n = 29), Wooler annuloplasty (n = 13), commissural plasty (n = 9), Kay annuloplasty (n = 7) and others (n = 8). The TV was replaced in 19 patients with biological (n = 10) and mechanical (n = 9) prostheses. The median hospital stay was 12 days (IQR 10). The overall mortality rate was 8.6% (n = 11): 2 hospital deaths (1.6%) and 9 late deaths. Survival was 93% [95% confidence interval (CI) 85-97%] at 5 years and 83% (95% CI 70-91%) at 10 years. The median follow-up period was 4.95 years (IQR 7.7) with 1 TV reoperation. Echocardiographic assessment showed ≥moderate regurgitation in 34 (34.3%) patients. Suture plasty had a significantly higher incidence of TV regurgitation ≥moderate compared to ring annuloplasty (48.9% vs 26.3%; P = 0.033)., Conclusions: TV surgery in the ACHD is frequently associated with other main procedures. Stabilizing the TV annulus with a prosthetic ring guarantees lower recurrence of moderate to severe regurgitation compared to suture plasty repair., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: The population of ageing adults with congenital heart disease (ACHD) is increasing; surgery in these patients presents major difficulties in management. A great debate has developed about whether these patients should be cared for at an adult or paediatric hospital and by an acquired or congenital cardiac surgeon. We analysed data of the surgical treatment of ACHD from the Italian cardiac surgery centres in 2016, focusing on the type of surgery performed, where these patients were operated on and by whom., Methods: Ninety-two Italian cardiac surgery centres were contacted and 70 centres participated in this study. We collected data on the types of cardiac operations performed in congenital heart defect patients older than 18 years. In 2016, a total of 913 patients with ACHD were operated on: 440 by congenital cardiac surgeons (group I) in centres with paediatric and adult cardiac surgery units, and 473 by adult cardiac surgeons (group II) in centres with exclusively adult cardiac surgery units., Results: Pathologies of the right ventricular outflow tract were the most frequent diseases treated in group I and pathologies of the left ventricular outflow tract in group II. Group I included more complex and heterogeneous cases than group II. Surgery for ACHD represented 12% of the activity of congenital cardiac surgeons and only 1% of the activity of adult cardiac surgeons., Conclusions: In Italy, ACHD patients are operated on both by congenital and adult cardiac surgeons. Congenital cardiac surgeons working in centres with both paediatric and adult cardiac surgery are more involved with ACHD patients and with more complex cases., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Life expectancy of patients with repaired tetralogy of Fallot is normal until adulthood, when it becomes burdened by major complications such as cardiac arrhythmias and heart failure. Ventricular tachycardia (VT), associated with electrical conduction abnormalities in anatomical isthmuses, is the most common complication and is refractory to medical therapy. The arrhythmogenic sources can be surgically ablated during reoperations for pulmonary valve incompetence. The purpose of our study is to evaluate the safety and effectiveness of surgical radiofrequency ablation during PVR and its long-term impact on the occurrence of cardiac events., Methods: A total of 118 patients with repaired tetralogy of Fallot underwent reoperation for pulmonary valve regurgitation in IRCCS-Policlinico San Donato between January 2002 and February 2016. Twenty patients with risk factors for ventricular arrhythmia underwent EP testing and were found positive. The mean QRS duration was 178 ms. Right ventricular dysfunction was present in all the patients, and 20% had associated tricuspid regurgitation. The cohort (N = 20), with positive EP study, underwent pulmonary valve replacement and concomitant intraoperative ventricular surgical radiofrequency ablation, targeting the slow conducting isthmuses identified during preoperative electroanatomical mapping. All patients were reviewed at 1 month, 3 months and 6 months with clinical evaluation, echocardiography, ECG and implantable cardioverter-defibrillator interrogation. At 6 months, a postoperative electrophysiological study was performed in all patients, and a cardioverter defibrillator was implanted as primary preventive measure in patients with residual inducible VT., Results: Pulmonary valve replacement and surgical ventricular radiofrequency ablation were performed in all cases. One patient died while on V-V extracorporeal membrane oxygenation (ECMO) due to respiratory failure. There was no late mortality. Follow-up was complete with a median of 6.5 years. During the postoperative EPS study, inducible VT was found in 15.7% of patients who received an implantable cardioverter-defibrillator, whereas VT could no longer be induced in the remaining patients. No procedure-related complication was observed, and freedom from ventricular arrhythmia was 94% and 89.5% at 1 year and 5 years, respectively., Conclusions: Pulmonary regurgitation and right ventricular dysfunction correlate with VT and SCD, but valve replacement alone does not reduce the risk of ventricular tachyarrhythmias. Intraoperative ventricular ablation during reoperation for pulmonary regurgitation prevents the occurrence of ventricular arrhythmias in the early and medium term. Accordingly, this technique may be proposed as a safe associated procedure in selected patients., (© The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

The population of patients with congenital heart disease (CHD) is continuously increasing with more and more patients reaching adulthood. A significant portion of these young adults will suffer from arrhythmias due to the underlying congenital heart defect itself or as a sequela of interventional or surgical treatment. The medical community will encounter an increasing challenge as even most of the individuals with complex congenital heart defects nowadays become young adults. Within the past 20 years, management of patients with arrhythmias has gained remarkable progress including pharmacological treatment, catheter ablation, and device therapy. Catheter ablation in patients with CHD has paralleled the advances of this technology in pediatric and adult patients with structurally normal hearts. Growing experience and introduction of new techniques like the 3D mapping systems into clinical practice have been particularly beneficial for this growing population of patients with abnormal cardiac anatomy and physiology. Finally, device therapies allowing maintanence of chronotropic competence and AV conduction, improving haemodynamics by cardiac resynchronization, and preventing sudden death are increasingly used. For pharmacological therapy, ablation procedures, and device therapy decision making requires a deep understanding of the individual pathological anatomy and physiology as well as detailed knowledge on natural history and long-term prognosis of our patients. Composing expert opinions from cardiology and paediatric cardiology as well as from non-invasive and invasive electrophysiology this position paper was designed to state the art in management of young individuals with congenital heart defects and arrhythmias.

Aims: Supraventricular arrhythmias are a major cause of morbidity and mortality in adult patients with congenital heart disease (CHD). Intraoperative ablation offers an alternative for patients who failed ablation procedures or are requiring concomitant surgical intervention. We present our long-term results with the surgical treatment of arrhythmias in adults with CHD (ACHD) undergoing elective cardiac surgery and the clinical predictors for arrhythmia recurrence., Methods and Results: Between 2002 and 2013, 80 consecutive patients with CHD, mean age of 39 years, underwent intraoperative ablation with monopolar irrigated radiofrequency during cardiac surgery procedures. Significant clinical predictors of arrhythmia recurrence were determined by univariate analysis. We performed 47 right-sided Maze procedures, and 33 Cox-Maze III procedures. In 75 survivors, the ablation was effective immediately. Over an average follow-up period of 72 months (12-155 months), arrhythmias recurred in nine (20%) patients after right-sided Maze, and in six (19%) patients after Cox-Maze III. Eleven patients were controlled with medical therapy, three underwent catheter ablation of the arrhythmia, and one required a permanent pacemaker. Preoperative arrhythmia length ≥3 years (P ≤ 0.001), tetralogy of Fallot (P ≤ 0.006), and preoperative atrial fibrillation (P ≤ 0.016) were associated with recurrence arrhythmia. Conversely, NYHA class <3 (P ≤ 0.047) was associated with a lower risk of recurrence., Conclusion: Surgical treatment of unresponsive supraventricular arrhythmia during concomitant cardiac surgery in ACHD is a safe and effective procedure. Freedom from arrhythmias recurrence is 75% after 6 years of follow-up. Long-term recurrence of arrhythmia in these patients seems to be strongly correlated to preoperative arrhythmia duration., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.)

The success of cardiac surgery over the past 50 years has increased numbers and median age of survivors with congenital heart disease (CHD). Adults now represent two-thirds of patients with CHD; in the USA alone the number is estimated to exceed 1 million. In this population, many affected women reach reproductive age and wish to have children. While in many CHD patients pregnancy can be accomplished successfully, some special situations with complex anatomy, iatrogenic or residual pathology are associated with an increased risk of severe maternal and fetal complications. Pre-conception counselling allows women to come to truly informed choices. Risk stratification tools can also help high-risk women to eventually renounce to pregnancy and to adopt safe contraception options. Once pregnant, women identified as intermediate or high risk should receive multidisciplinary care involving a cardiologist, an obstetrician and an anesthesiologist with specific expertise in managing this peculiar medical challenge. This document is intended to provide cardiologists working in hospitals where an Obstetrics and Gynecology Department is available with a streamlined and practical tool, useful for them to select the best management strategies to deal with a woman affected by CHD who desires to plan pregnancy or is already pregnant.

The right timing to replace the pulmonary valve in a patient with dysfunction of the right ventricular outflow tract is unknown. Both percutaneous pulmonary valve and surgical prosthesis are suitable options. In every patient, the right ventricle (RV) remodels and recovers differently after pulmonary replacement. Therefore, it is difficult to identify the best treatment option and to predict the long-term results. In the last few years, we focused our research on optimizing the characterization of these patients through advanced cardiovascular imaging in order to find possible variables, parameters, and reproducible measurements that can help us in the decision-making process. The aim of the present article is to present our ongoing research lines that focus on the characterization and optimal treatment approach to the dysfunction of the RVOT.

Thanks to the improvement of surgical and interventional cardiac procedures, the majority of children with congenital heart defects (CHD) can now be expected to reach adulthood. The number of adults with congenital heart disease (ACHD) will inevitably increase, and recent data affirm that in Europe, we are actually faced with an estimated patient population of 2.3 million. These patients, particularly the adults with moderate and highly complex CHD, can be very difficult to manage, and should be treated in few experienced and specialized 'grown-up congenital heart disease' (GUCH) units, concentrating resources, patients, funding, and professional experiences. On 2000, we created a GUCH unit in our hospital that rapidly became a referral centre in Italy for number and complexity of patients treated. The present study is a presentation of how is organized our GUCH unit and a brief overview of our more recent experience with ACHD patients.

Motivation: The analysis of high-resolution proton nuclear magnetic resonance (NMR) spectrometry can assist human experts to implicate metabolites expressed by diseased biofluids. Here, we explore an intermediate representation, between spectral trace and classifier, able to furnish a communicative interface between expert and machine. This representation permits equivalent, or better, classification accuracies than either principal component analysis (PCA) or multi-dimensional scaling (MDS). In the training phase, the peaks in each trace are detected and clustered in order to compile a common dictionary, which could be visualized and adjusted by an expert. The dictionary is used to characterize each trace with a fixed-length feature vector, termed Bag of Peaks, ready to be classified with classical supervised methods., Results: Our small-scale study, concerning Type I diabetes in Sardinian children, provides a preliminary indication of the effectiveness of the Bag of Peaks approach over standard PCA and MDS. Consistently, higher classification accuracies are obtained once a sufficient number of peaks (>10) are included in the dictionary. A large-scale simulation of noisy spectra further confirms this advantage. Finally, suggestions for metabolite-peak loci that may be implicated in the disease are obtained by applying standard feature selection techniques.

Aim: To report the experience of 23 tertiary referral European Centres on transcatheter closure of congenital ventricular septal defects (VSD)., Methods and Results: Implantation of transcatheter devices was attempted in 430 patients (pts) with congenital VSDs until July 2005. The following anatomic types were present: 119 muscular, 250 perimembranous, 16 multiple, 45 residual post-surgery. Median VSD size was 7 mm (range 3-22), fluoroscopy time 33 min (range 3-146). Devices implanted were Amplatzer muscular or membranous devices in 364, PDA devices in 12, ASD devices in seven, Starflex in seven, and coils in nine patients. Procedure was successful in 410 cases (95%)., Complications: device embolization in five cases (surgery in two, catheter retrieval in three), aortic regurgitation in 14 cases (two of which requiring surgery), tricuspid regurgitation in 27 cases (no surgery was necessary), minor rhythm disturbances in 10 pts, death in one patient, complete heart block (cAVB) in 16 pts [perimembranous 12 of 250 (5%), muscular one of 119 (0.8%), residual post-surgery VSD three of 45 (6.7%)]. CAVB was transient in six patients, requiring permanent pace-makers in 10 cases (3.8%) (six early, four late). In the multivariate analysis, the only variable associated with a risk of the occurrence of complication was age (P=0.012) and weight (P=0.0035). In the univariate analysis, risk factors for the development of cAVB were, device type (P=0.03) and VSD location (P=0.05). After the multivariable Cox proportional hazards analysis, no risk factor was found., Conclusion: Transcatheter closure of congenital VSDs offers encouraging results. COMPLICATIONS are limited; the most relevant one seems to be the device related to cAVB in perimembranous VSD. More experience and long-term follow-up are mandatory to assess safety and effectiveness of this procedure as an alternative to conventional surgery.

Aims: The aim of the article is to study the safety, efficacy, and follow-up of percutaneous closure of muscular ventricular septal defect (mVSD) and perimembranous ventricular septal defect (pmVSD) in children., Methods and Results: Between January 2000 and June 2005, among 140 patients who underwent percutaneous closure of an mVSD or a pmVSD, 88 were aged lt;12. Two different Amplatzer devices were used: the mVSD occluder and the pmVSD occluder. Mean age and weight at procedure were 4.5+/-3.3 years and 18.7+/-11.2 kg, respectively. Percutaneous closure was successfully achieved in 83 subjects (94%). No deaths occurred. Thirteen patients (14.7%) had early complications: device embolization (n=4), vascular complications (n=3), and rhythm abnormalities (n=6). These were transient complications in all but one case [1.1% complete atrioventricular block (cAVB) needing pacemaker implantation]. During a median follow-up of 24 months, three subjects treated for a pmVSD needed pacemaker implantation due to the occurrence of cAVB. Multivariable analysis using Cox's proportional hazard regression showed that age was the only risk factor associated with the occurrence of cAVB (P=0.028; relative risk: 0.25)., Conclusion: Percutaneous closure of mVSD and pmVSD in children can be performed safely and successfully. The occurrence of cAVB is a major concern in young children with pmVSD.

Objective: Pulmonary regurgitation may cause progressive right ventricular dilatation and dysfunction in adult patients previously repaired for right ventricular outflow tract obstruction (RVOTO), and who require subsequent valve implantation for relief of these symptoms. Right ventricular recovery after pulmonary valve implantation (PVI) may be closely linked to the functional importance of the structural presence of an aneurysm or akinetic segment in the RVOT area. To test this concept, the impact of the right ventricular restoration with a new surgical ventriculoplasty technique is evaluated following pulmonary valve implantation in patients with severe pulmonary regurgitation and right ventricular dilatation., Methods: Sixteen patients with severe pulmonary valve regurgitation (PVR) and right ventricular dilatation with RVOT aneurysm underwent right ventricular remodelling since January 2002. Each underwent preoperative evaluation by Doppler echocardiography, magnetic resonance imaging (MRI), and right ventricular myocardial acceleration during isovolumic contraction (IVC). The surgical procedure included pulmonary valve implantation and RVOT restoration achieved by removal of the aneurysm tissue, coupled with a ventriculoplasty to reduce volume, accomplished by creating a satisfactory RVOT dimension by placing with 2-0 Gortex suture to allow acceptance of a 26 Hegar dilator to avoid restriction. Thirteen associate surgical procedures were added in nine patients., Results: All patients survived the operative procedure and underwent a 16-month follow-up interval. A reduction of cardio thoracic index and a clinical improvement occurred in each patient. Significant reduction of RVEDV and RVESV and increased right ventricular ejection fraction was observed, and IVC changed from 0.7+/-0.5 m/s2 to 1.3+/-0.6 m/s2 in the 13 patients that underwent MRI and IVC during the preoperative control interval and 6 months after the procedure., Conclusions: This preliminary database implies that the right ventricular restoration is a simple and effective procedure, and introduces a structural component that should be added during pulmonary valve implantation in patients with severe right ventricular dilatation and underlying aneurysm or akinesia of the right ventricular outflow tract.

Aims: Since the late 1980s, endovascular stents have been used in the treatment of several vascular lesions. In the last decades, stent implantation has been proposed as a reliable option for the treatment of coarctation of the aorta. In this setting, it seems to have some advantages, rendering it superior to angioplasty alone., Methods and Results: Between December 1997 and December 2004, 71 consecutive patients (44 males and 27 females) underwent cardiac catheterization for native or recurrent coarctation of the aorta. Seventy-four stents were implanted. All discharged patients were enrolled in a follow-up programme. Every patient underwent clinical evaluation, echo-colour Doppler studies, and exercise ECG at 1 and 6 months after the stent implantation. Peak systolic gradient dropped from 39.3 +/- 15.3 to 3.6 +/- 5.5 mmHg (P = 0.0041). The diameter of the coarcted segment increased from 8.3 +/- 2.9 to 16.4 +/- 3.8 mm (P = 0.037). In our series, one death occurred in a 22-year-old girl with a recurrent coarctation of the aorta, just after stent implantation. The rate of minor complications was <2%. Re-dilatation of a previously implanted stent was performed in three patients., Conclusion: In our experience (the largest reported to the best of our knowledge), stenting of a coarctation/re-coarctation of the aorta represents a safe alternative treatment without a significant mid-long-term complication.