Right disc thrombosis of the new Gore Cardioform ASD Occluder.

BACKGROUND: The GCA is a well performing device in terms of efficacy despite complex anatomies (aortic rim <5 mm and ASD diameter >17 mm) with a good safety profile. AIMS: To evaluate atrial septal defect (ASD) features impacting on right disc device thrombosis in patients who underwent Gore Cardioform ASD Occluder (GCA) implantation. METHODS: A total of 44 consecutive patients undergoing percutaneous ASD with GCA device from January 2020 to September 2022 at our tertiary care Center were evaluated. The minimum follow-up was 6 months. RESULTS: The patients were stratified in two groups according to a cut-off value of ASD diameter equal to 20 mm at sizing balloon, derived from ROC analysis (AUC = 0.894; p = 0.024). Baseline characteristics were comparable between groups in terms of age, sex, weight, height, and interatrial septum dimensions. Patients with ASD > 20 mm (n = 9) had a higher ASD/device dimension ratio, both at echocardiography (p = 0.009) and at sizing balloon (p = 0.001), longer fluoroscopic time (p = 0.022), and higher incidence of device thrombosis (0.006). Right disc thrombosis was observed in three patients of the ASD > 20 mm group, always in the inferior portion of the right disc. On univariate analysis, ASD diameter at sizing balloon (OR 1.360; p = 0.036) was the only positive predictor of device thrombosis. CONCLUSIONS: Right disc thrombosis of the GCA device may be under-recognized at follow-up, hence deserving clinical attention, especially in those patients with larger ASD diameters.

Regression of Cardiac Rhabdomyomas Producing a Severe Aortic Stenosis: Case Report and Discussion of the Literature.

We describe a case of a voluminous rhabdomyoma (R) detected by fetal echocardiography at 32 weeks' gestation (w.g.) obstructing the left ventricular inflow and aortic outflow tract, with a moderate aortic gradient at birth, not needing immediate surgery. At follow-up, the mass progressively regressed, leaving the aortic valve partly damaged, with a gradient that increased to a maximum of 100 mmHg at 9 years. The girl was then operated on successfully by a plasty of the aortic valve. The literature regarding R is discussed.

Characteristics and outcomes of fetuses with laterality defects are the current outcomes better? A single center study.

Objective: To analyze the more recent outcomes of fetuses with different laterality defects.Material and methods: Out of 1508 fetuses with congenital heart disease (CHD) diagnosed by echocardiography between January 1995 and December 2015, 94 (6.3%) presented abnormal visceroatrial situs: 42 till December 2002 (group 1, analyzed retrospectively) and 52 since then until December 2015 (group 2, analyzed prospectively). Twenty-one had left isomerism (Lisom), 22 right isomerism (Risom), 16 situs inversus- dextrocardia (SVI-dx), 5 situs inversus- levocardia (SVI-levo), and 30 situs solitus-dextrocardia (SSol-dx).Results: Seventy-six cases (81%) had CHD, more frequently complex atrioventricular defect. Eighteen cases showed normal heart: half of subgroups SVI-dx and SVI-levo, a third of SSol-dx. Postoperative mortality in continuing pregnancy in group 2 was 43.7 versus 40% in group 1, lower in SVs-dx (0 versus 50%) and Lisom (33.3 versus 66.7%), worse in Risom (71.4 versus 25%). Total mortality in group 2 was 48.3 versus 55% in group 1, better in Ssol-dx (37.5 versus 50%) and in Lisom (27.4 versus 71.4%). In isolated forms all but one case are alive.Conclusions: Our fetal cases presented a relevant mortality in both periods. The outcomes were slightly better in a more recent era in SSol and Lisom.What is known about this topic?Out of laterality defects, the heterotaxies (i.e. left and right isomerism) are known to be associated to a relevant mortality in fetal and neonatal cases.Heterotaxies have a variable position of the heart, but some authors evaluated only those with dextrocardia in their studies on different laterality defects.Controversial data are presented in the literature for the outcomes of fetuses with laterality defects. Many large studies analyzed the data of a very long time period in which it is opinable to compare the results of the treatment, because of a recent improved management and surgical techniques.What does this study add?Our study presents an experience of a single center with cases of different types of laterality defects observed during a routine fetal echocardiography, not only heterotaxies.The knowledge of different laterality defects and of their characteristics (association with cardiac and extracardiac anomalies or with normal heart) is useful for the prenatal counseling.We compare the postoperative and total mortality in more recent period (since 2003) with respect to the previous era (1995-2002).

Lombardy regional urgent reorganization for congenital cardiac patients following the Covid-19 pandemic.

OBJECTIVE: By the end of February 2020, the COVID-19 pandemic infection had spread in Northern Italy, with thousands of patients infected. In Lombardy, the most affected area, the majority of public and private hospitals were dedicated to caring for COVID-19 patients and were organized following the 'Hub-and-Spoke' model for other medical specialties, like cardiac surgery and interventional procedures for congenital cardiac disease (CHD). Here, we report how the congenital cardiac care system was modified in Lombardy and the first results of this organization. METHODS: We describe a modified 'Hub-and-Spoke' model - that involves 59 birthplaces and three specialized Congenital Cardiac Centers -- and how the hub center organized his activity. We also reported the data of the consecutive cases hospitalized during this period. RESULTS: From 9 March to 15 April, we performed: a total of 21 cardiac surgeries, 4 diagnostic catheterizations, 3 CT scans, and 2 CMR. In three cases with prenatal diagnosis, the birth was scheduled. The spoke centers referred to our center six congenital cardiac cases. The postop ExtraCorporeal Membrane Oxygenation support was required in two cases; one case died. None of these patients nor their parents or accompanying person was found to be COVID-19-positive; 2 pediatric intensivists were found to be COVID-19-positive, and needed hospitalization without mechanical ventilation; 13 nurses had positive COVID swabs (4 with symptoms), and were managed and isolated at home. CONCLUSION: Our preliminary data suggest that the model adopted met the immediate needs with a good outcome without increased mortality, nor COVID-19 exposure for the patients who underwent procedures.

Improving health perception through a transition care model for adolescents with congenital heart disease.

AIMS: The aim of this study was to assess the impact of a transition clinic model on adolescent congenital heart disease (CHD) patients' health perception outcomes. The transition clinic model consists of multidisciplinary standardized interventions to educate and support CHD patients and represents a key element in the adequate delivery of care to these individuals during their transition from childhood to adulthood. Currently, empirical data regarding the impact of transition clinic models on the improvement of health perceptions in CHD adolescent patients are lacking. METHODS: A quasi-experimental design was employed. Quality of life, satisfaction, health perceptions and knowledge were assessed at the time of enrolment (T0) and a year after enrolment (T1), respectively. During the follow-up period, the patients enrolled (aged 11-18 years) were involved in the CHD-specific transition clinic model (CHD-TC). RESULTS: A sample of 224 CHD adolescents was enrolled (60.7% boys; mean age: 14.84 ±â€Š1.78 years). According to Warnes' classification, 22% of patients had simple heart defect, 56% showed moderate complexity and 22% demonstrated severe complexity. The overall results suggested a good impact of the CHD-TC on adolescents' outcomes, detailing in T1 the occurrence of a reduction of pain (P < 0.001) and anxiety (P < 0.001) and an improvement of knowledge (P < 0.001), life satisfaction (P < 0.001), perception of health status (P < 0.001) and quality of life (P < 0.001). CONCLUSION: The CHD-TC seems to provide high-quality care to the patient by way of a multidisciplinary team. The results of the present study are encouraging and confirm the need to create multidisciplinary standardized interventions in order to educate and support the delivery of care for CHD adolescents and their families.

[The effect of the transition care model on health perception among adolescents with congenital heart disease: a quasi-experimental study]. / Gli effetti di un set standardizzato di interventi educazionali sulla percezione della salute di adolescenti con cardiopatia congenita (modello Transition): risultati preliminari di uno studio quasi-sperimentale.

BACKGROUND: The multidisciplinary standardized interventions to educate and support patients with congenital heart disease (CHD) are described as "Transition Clinic" (TC). TC represents a key element to deliver care for patients during the transition from childhood to adulthood. So far, there is a lack of empirical evidence regarding the impact of TC models on the improvement of health perception in adolescent patients with CHD (CHD-specific TC model). For this reason, the aim of this study is assess the impact of the TC model on CHD adolescent patients' health perception outcomes. METHODS: This study has a quasi-experimental design. Quality of life, satisfaction and health perception were assessed in T0 and after 1 year from enrollment (T1). During the follow-up period, the patients enrolled (aged 14 to 21 years) were involved in the CHD-specific TC model. RESULTS: The results are referred to the first 100 patients enrolled (mean age 14.79 ± 1.85 years; 60% male), as they have already completed the follow-up. The overall study is currently ongoing. According to Warnes' classification, 29% of patients had simple heart defects, 46% showed moderate complexity, and 25% showed severe complexity. The comparison between T0 and T1 showed statistically significant improvement in T1 regarding pain/discomfort, anxiety/depression and perception of health status (EQ-5D), general satisfaction and quality of life (LAS QoL). CONCLUSIONS: The preliminary results showed in this study are encouraging, and confirm the need to create a multidisciplinary standardized intervention of education and support to deliver care for adolescent patients with CHD.

Biventricular Heart Remodeling After Percutaneous or Surgical Pulmonary Valve Implantation: Evaluation by Cardiac Magnetic Resonance.

PURPOSE: The aim of this study was to evaluate the impact of percutaneous pulmonary valve implantation (PPVI) and surgical pulmonary valve replacement (SPVR) on biventricular and pulmonary valve function using cardiac magnetic resonance. MATERIALS AND METHODS: Thirty-five patients aged 20±8 years (mean±SD) underwent PPVI, whereas 16 patients aged 30±11 years underwent SPVR. Cardiac magnetic resonance examinations were performed before and after the procedures with an average follow-up interval of 10 months. Cine steady-state free precession sequences for cardiac function and phase-contrast sequences for pulmonary flow were performed. The right ventricle (RV) and left ventricle (LV) functions were evaluated using a dedicated software. RESULTS: The RV end-diastolic volume index (mL/m) decreased significantly after PPVI and SPVR, from 74 to 64 (P=0.030) and from 137 to 83 (P=0.001), respectively. The RV ejection fraction increased significantly after SPVR, from 47% to 53% (P=0.038). The LV end-diastolic volume index increased significantly after PPVI, from 66 to 76 mL/m (P<0.001). The LV stroke volume index increased significantly after PPVI, from 34 to 43 mL/m (P=0.004). The analysis of bivariate correlations showed that in patients undergoing SPVR the RV changes after the procedure were positively correlated to LV changes in terms of end-systolic volume index (r=0587; P=0.017) and ejection fraction (r=0.681; P=0.004). CONCLUSIONS: A RV volumetric reduction and a positive effect on ventricular-ventricular interaction were observed after both PPVI and SPVR. After PPVI, a positive volumetric LV remodeling was found. No LV remodeling was found after SPVR. After both procedures, the replaced pulmonary valve functioned well.

Surgical mitral valve replacement with the Melody valve in infants and children: the Italian experience.

AIMS: Paediatric patients with mitral valve (MV) disease have limited options for prosthetic MV replacement. Based on long experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, we aimed to test the use of the Melody valve as a surgical implant in the mitral position in a small group of infants with congenital mitral disease. METHODS AND RESULTS: Eight patients, aged from 3 months to 6.2 years, with congenital MV dysplasia underwent Melody valve implantation in the mitral position between March 2014 and October 2015. Once sutured to the MV annulus, the Melody valve was expanded through a balloon catheter to achieve the best diameter (12-20 mm). Two patients needed ECMO assistance after traditional MV repair and a Melody valve was successfully implanted as possible bail-out. However, neither of them recovered from multi-organ failure and both died, in spite of the well-functioning Melody prosthesis. During follow-up, only one patient underwent catheter-based balloon expansion of the valve. CONCLUSIONS: The medium-term results of this procedure are encouraging. The Melody valve opens up the opportunity to carry out MV replacement in more children at an earlier time point, and it has potential to revolutionise the treatment of MV disease.

Percutaneous management of failed bioprosthetic pulmonary valves in patients with congenital heart defects.

AIMS: We reviewed our center experience in the field of transcatheter pulmonary valve-in-valve implantation (TPViV), that is emerging as a treatment option for patients with pulmonary bioprosthetic valve (BPV) dysfunction. METHODS: Between April 2008 and September 2015, a total of six patients with congenital heart disease (four men) underwent TPViV due to stenosis of preexisting BPV. Four patients received a Melody Medtronic Transcatheter Pulmonary Valve and two an Edward Sapien Valve. RESULTS: No procedural-related complications occurred. After valve implantation, right ventricular systolic pressure (RVSP, 80.5 ±â€Š25.3-41.2 ±â€Š8.35 mmHg, P < 0.05), right ventricular outflow tract (RVOT) gradient (55.3 ±â€Š23.4-10.6 ±â€Š3.8 mmHg, P < 0.05), and RVSP-to-aortic pressure (0.75 ±â€Š0.21-0.38 ±â€Š0.21, P = 0.01) fell significantly. Echocardiograms at follow-up revealed a significant reduction in estimated RVSP (88.7 ±â€Š22-21.7 ±â€Š4.7 mmHg, P < 0.05), in RVOT (76.2 ±â€Š17.9-25.7 ±â€Š6.1 mmHg, P = 0.005), and in mean RVOT (40.7 ±â€Š9.9-15.5 ±â€Š4.8 mmHg, P < 0.05) gradients. Cardiac magnetic resonance showed no significant change in biventricular dimensions and function. Symptomatic patients reported improvement of symptoms, although cardiopulmonary exercise did not show any significant differences. CONCLUSION: TPViV is an effective and well tolerated treatment for BPV dysfunction, improving freedom from surgical reintervention. Long-term studies will redefine the management of dysfunctional RVOT, either native or surrogate.

PFO "angioplasty": The preparation of a very stiff and long tunnel for device closure.

INTRODUCTION: Patent foramen ovale (PFO) closure is a widely performed procedure. Understanding the anatomy is mandatory in order to perform the procedure successfully. The degree of overlap between the septum primum and secundum is called the tunnel. In particular, long and stiff tunnels may prevent the correct positioning of devices that have a fixed distance between the right and left atrial discs. PATIENTS AND METHODS: We evaluated retrospectively 361 patients with PFO closure between January 2012 and June 2014 in our institution and were treated due to previous history of ischemic stroke. By TEE, the overlap between septum primum and secundum is usually better seen on the bicaval view that is obtained at 75-90° midesophageal views. Tunnel length and PFO opening were measured. Twelve subjects (median age 35 years; range 30-58 years; three females) showed a long (median 22 mm; range 15-32 mm) and rigid tunnel (median opening 2 mm; range 1-4 mm). RESULTS: Angioplasty of the tunnel was performed by using peripheral angioplasty balloons with a length ranging from 4 to 8 cm and a diameter between 8 and 12 mm. After angioplasty, the tunnel appeared shorter (12 mm; range 8-16 mm) and less rigid (median opening 8 mm; range 7-12 mm). The following devices were implanted: 25 mm Amplatzer PFO occluder in three patients; 25 mm Gore septal occluder in nine subjects. Median fluoroscopy time was 8 min (range 6-10 min). No complications occurred. At a median follow-up of 12 months (range 2-18 months), no problems occurred and all subjects but one (trivial residual shunting) showed a complete closure. CONCLUSIONS: Angioplasty of a PFO rigid and stiff tunnel is a feasible and safe option. © 2016 Wiley Periodicals, Inc.

Partial abnormal drainage of superior and inferior caval veins into the left atrium: two case reports.

Abnormal connection of the right superior caval vein to the left atrium is an uncommon systemic vein drainage anomaly, with only a few cases reported among congenital heart disease (CHD), around 20 cases published in the medical literature. The inferior vena cava connection with the left atrium, also very rare, can appear directly or in heterotaxy. Clinical suspicion arises due to the presence of cyanosis in the absence of other specific clinical signs (without other associated CHD). We present the cases of two children with abnormal superior and inferior systemic venous return. The first case is an abnormal connection of right superior vena cava to the left atrium associated with persistent left superior vena cava draining into the right atrium through the coronary sinus. The second case is an interruption of the inferior vena cava with hemiazygos continuation, drained into the left superior vena cava, which drained into the left atrium. The diagnosis was imagistic - echocardiography and angiography. Surgical treatment solutions vary from one case to another, usually following anatomic correction. Hypoxia accompanied by cyanosis must bring into question the pathology of systemic venous drainage anomaly, after other common causes have been excluded. Surgery is indicated in all cases due to the risk associated with the presence of right-to-left shunt.

Coronary-cameral fistulas: indications and methods for closure.

Coronary-cameral fistulas (CCF) are anomalous connections between one or more coronary arteries and a cardiac chamber, most commonly the right ventricle or right atrium. The major indications for closure are: significant left to right shunt, myocardial ischaemia, prevention of endoarteritis or rupture. Nowadays, the first option for treatment is transcatheter closure. According to the morphology of the fistulas the most appropriate occluder device should be selected: coils (e.g., Gianturco coils, controlled-release coils, PFM coils), vascular plugs or a patent ductus arteriosus (PDA) device or muscular ventricular septal defect (VSD) device. The way to deploy the occluders could be direct arterial or venous through an arteriovenous loop, according to the anatomy of the fistulas assessed by multiple angiograms in different projections. A test occlusion of the fistula with balloon catheter and simultaneous coronary angiogram is recommended for choosing the proper device type and size and the best position for deployment to achieve complete occlusion of the fistula without compromising the flow in coronary side branches.

Tetralogy of Fallot With Left Pulmonary Artery Discontinuity and Totally Anomalous Pulmonary Venous Drainage to the Azygos Vein.

We describe a 13-month-old patient with tetralogy of Fallot, left pulmonary artery discontinuity, and supracardiac totally anomalous pulmonary venous drainage to the azygos vein. The diagnosis and the management of this rare association are briefly discussed.

Treatment of right ventricular outflow tract dysfunction: a multimodality approach.

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.

The Edwards Valeo lifestents in the treatment and palliation of congenital heart disease in infants and small children.

BACKGROUND: Intravascular stenting is the procedure of choice in the treatment of vascular stenoses. However, in infants and small children large sheaths are needed, and adult-size stents cannot be implanted. The Valeo Biliary Lifestent (Edwards Lifesciences Irvine, CA) is low profile and can be dilated up to 18-20 mm. We aimed to report on early and mid-term results with the use of Valeo stents in infants and children with congenital heart disease. METHODS: Twenty-five subjects were treated in our experience. Mean age and weight were 39 ± 35 months (range 1-132) and 10.4 ± 6.7 kg (range 3-30), respectively. Two groups of patients were: Group A: patients with pulmonary artery stenosis (21 subjects); Group B subjects in whom stenting was applied in other vessel or heart sites (four subjects). RESULTS: A total of 35 stents were successfully used. Fluoroscopy time was 32 ± 11 min. No intra-operative death or hospital mortality was recorded. Stent post dilatation was performed in nine patients. The incidence of complication was 12% (3/25) (two subjects developed transient hypotension and bradycardia which required inotropic treatment, 1 patient developed mild lung bleeding). There was a significant improvement of angiographic appearance and RV pressure (RV/AO systolic pressure ratio before 1 ± 02 (range 0.8-1.5) versus after the procedure 0.6 ± 0.2 (range 0.4-0.9) P < 0.001). In group B stents were successfully implanted in aortic recoarctation, interatrial septum and in two modified BT shunts. At a median follow-up of 18 months (range 1-24 months) results remained stable and no complications occurred. In particular no stent fractures were seen. Furthermore, redilation was performed safely and successfully in three subjecs up to 18 months after the first implantation. CONCLUSIONS: In our series, Valeo Lifestents have proven to be effective and with low incidence of complication in various anatomical settings, in low weight infants and in early post-operative course. Large series and longer follow-up are mandatory.

Echocardiographic assessment after surgical repair of tetralogy of fallot.

Surgical correction of tetralogy of Fallot is still one of the most frequently performed intervention in pediatric cardiac surgery, and in many cases, it is far from being a complete and definitive correction. It is rather an excellent palliation that solves the problem of cyanosis, but predisposes the patients to medical and surgical complications during follow-up. The decision-making process regarding the treatment of late sequel is among the most discussed topics in adult congenital cardiology. In post-operative Fallot patients, echocardiography is used as the first method of diagnostic imaging and currently allows both a qualitative observation of the anatomical alterations and a detailed quantification of right ventricular volumes and function, of the right ventricular outflow tract, and of the pulmonary valve and pulmonary arteries. The literature introduced many quantitative echocardiographic criteria useful for the understanding of the pathophysiological mechanisms involving the right ventricle and those have made much more objective any decision-making processes.

Evaluation of right ventricular function in adults with congenital heart defects.

The right ventricle (RV) is of lesser importance in acquired heart disease, but its role is of increasing importance in congenital heart disease (CHD). Despite major progress being made, precise measurements of the RV are challenging because of its peculiar anatomical structure that is not adaptable to any planar geometrical assumption. This is particularly true in adult patients with CHD where the RV shape eludes any standardization, it may assume various morphologies, and its modality of contraction depends on previous surgical treatment and/or pathophysiological conditions. However, reliable and repeatable quantification of RV dimensions and function for these patients are essential to provide appropriate timing for intervention to optimize outcomes. In this population, echocardiographic evaluation should not be limited to an observational and subjective functional assessment of the RV but must provide quantitative values repeatable and clinically reliable to help the decision-making process. The aim of this review was to discuss the echocardiographic approach to the RV in adult patients with CHD in general and in specific cases of pressure or volume overload.

Partially uncovered Cheatham platinum-covered stent to treat complex aortic coarctation associated with aortic wall aneurysm.

Percutaneous treatment of aortic coarctation is a widely used option. Covered stents have increased the profile of efficacy and safety of this procedure. Here we report on a 32-year-old woman with significant aortic recoarctation associated with aortic wall aneurysm and close proximity of both lesions to the origin of both the subclavian arteries. It was decided to manually and partially uncover the proximal part of the stent to have a hybrid stent that could act as a bare stent at the level of the origin of the subclavian arteries and as a covered stent at the level of the aneurysm.