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BACKGROUND AND AIMS: Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI. METHODS: Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries. RESULTS: Data for 840 consecutive patients treated in 2014-2021 at a median age of 29.2 (19.0-41.6) years were obtained. The most common diagnosis was conotruncal defect (70.5%), with a native or patched RVOT in 50.7% of all patients. Valve sizes were 20, 23, 26, and 29 mm in 0.4%, 25.5%, 32.1%, and 42.0% of patients, respectively. Valve implantation was successful in 98.5% [95% confidence interval (CI), 97.4%-99.2%] of patients. Median follow-up was 20.3 (7.1-38.4) months. Eight patients experienced infective endocarditis; 11 required pulmonary valve replacement, with a lower incidence for larger valves (P = .009), and four experienced pulmonary valve thrombosis, including one who died and three who recovered with anticoagulation. Cumulative incidences (95%CI) 1, 3, and 6 years after TPVI were as follows: infective endocarditis, 0.5% (0.0%-1.0%), 0.9% (0.2%-1.6%), and 3.8% (0.0%-8.4%); pulmonary valve replacement, 0.4% (0.0%-0.8%), 1.3% (0.2%-2.4%), and 8.0% (1.2%-14.8%); and pulmonary valve thrombosis, 0.4% (0.0%-0.9%), 0.7% (0.0%-1.3%), and 0.7% (0.0%-1.3%), respectively. CONCLUSIONS: Outcomes of SAPIEN 3 TPVI were favourable in patients with CHD, half of whom had native or patched RVOTs.

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Sepsis is defined as a life-threatening organ dysfunction caused by a dysregulated host response to infection with a high mortality rate. Septic shock is a subset of sepsis with manifest circulatory dysfunction (use of vasopressors and persistent elevation of lactic acid) . As stated in literature, in addition to the use of empiric antibiotics and control of the infectious focus, intravenous fluid therapy is an essential intervention to promote hemodynamic stabilization. However, the literature also describes harmful outcomes related to fluid overload. Hemodynamic management in critically ill patients has traditionally focused on maintaining adequate cardiac output and arterial blood pressure by relying on fluid administration and/or vasopressor/inotropic support. However, organ perfusion is affected by other important factors, such as venous pressure, which can be overlooked. The evaluation of lung congestion with point of care ultrasound (POCUS), as a signal of extravascular fluid, and, more recently, a venous excess Doppler ultrasound (VExUS) grading system, are parameters for the assessment of the fluid status of the patient and organ congestion. Our main hypothesis is that adding a modified lung ultrasound score to the VExUS protocol could provide higher sensitivity and earlier identification of fluid overload, guiding the clinician in the decision of fluid administration in patients with sepsis.

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In patients with a patent foramen ovale (PFO) who have had a cryptogenic ischemic stroke, percutaneous closure reduces its recurrence risk. However, its role in spinal cord infarction (SCI) is less well-established. A few case reports describe the putative causative role of PFO in SCI. We present a case of a teenager with cryptogenic anterior SCI in the setting of a deep vein thrombosis and a high risk-PFO who underwent successful percutaneous closure.

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AIMS: Aortic arch atresia (AAA) is one of the rarest obstructive defects. The presence of this anomaly in adult age is uncommon. The typical anatomic feature consists of a complete occlusion of the membranous obstruction resulting in an acquired atresia without flow continuity between the proximal and distal segments. This feature is important in determining the feasibility of percutaneous intervention. The aim of the present study was to share long-term follow-up data of adult patients with AAA requiring percutaneous interventions for the management of this rare anomaly involving five different centres. METHODS AND RESULTS: Retrospective data of 19 patients (12 males, 63.2%, mean age 32.2±18.9 years) diagnosed with AAA treated in five different centres between 1999 and 2017 were collected. All patients underwent percutaneous recanalisation by (1) radiofrequency (RF) system (five patients, 26.3%), (2) extra-stiff guidewire (12 patients, 63.2%), and (3) transseptal needle (two patients, 10.5%). All procedures were subsequently followed by covered stent implantation. Two patients developed complications during the procedure and one of them died. Over a median follow-up of 4.94 years, four (21%) patients were able to be weaned from medications for hypertension. All the patients underwent reassessment for recurrence or restenosis during the follow-up. Seven (36.8%) patients underwent successful stent dilatation with a balloon. After the intervention, one patient experienced a late complication; however, one patient died due to an unknown cause believed to be unrelated to the previous recanalisation procedure. CONCLUSIONS: Percutaneous treatment of AAA is feasible with good long-term survival. This study reports the largest case series so far available in the literature.

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The benefits of patent foramen ovale (PFO) closure for cryptogenic stroke secondary prevention are still debated. The Risk of Paradoxical Embolism (RoPE) study developed a score to improve patient selection for this procedure. We proposed to assess the validity of this score to assess the prognostic impact of PFO closure.From 2000 to 2014, all consecutive patients submitted to PFO closure were included in a prospective registry in a university center. The primary endpoint was recurrent ischemic cerebrovascular events and the secondary endpoints were all-cause, neurological, and cardiac mortality rates and new-onset atrial fibrillation (NOAF) rates. In total, 403 patients were included in the study (women: 52.1%; mean age: 44.7 ± 10.9 years). The mean follow-up period was 6.4 ± 3.7 years. Immediate success was achieved in 97% patients. There were 23 (5.8%) ischemic cerebrovascular events, 8 (2.0%) deaths, and 17 (4.3%) NOAFs. The mean RoPE score was 6.10 ± 1.79. Smoker status, coronary artery disease, lower RoPE score, and higher left atrial dimensions were predictors of the primary endpoint. However, a lower RoPE score and coronary artery disease remained independent predictors in multivariate analysis.RoPE score was shown to be an independent predictor of recurrent ischemic cerebrovascular events, and a score of ≤ 6 was shown to identify patients with significantly higher risk of mortality and recurrent ischemic events.

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BACKGROUND: Guidelines allow percutaneous pulmonary valve implantation (PPVI) in conduits above 16mm diameter. Balloon dilatation of a conduit to a diameter>110% of the original implant size is also not recommended. We analyzed patients undergoing PPVI in such conditions. METHODS AND RESULTS: Nine patients (May 2008-July 2016) from 8 institutions underwent PPVI in conduits <16mm diameter. Five patients with 16-18mm conduit diameter underwent PPVI after over-expansion of the conduit>110%. Mean age and weight of the 14 patients was 12.1 (7.7 to 16) years and 44.9 (19 to 83) kg. Median conduit diameter at PPVI was 12 (10 to 17) mm. Median systolic right ventricular pressure was 70 (40 to 94) mmHg. Procedure was successful in all cases. A confined conduit rupture occurred in 7 patients (50%) and was treated with covered stent in 6. One patient experienced dislocation of 2 pulmonary artery stents that were parked distally. The post-implantation median systolic right ventricular pressure was 36 (28 to 51) mmHg. A fistula between right-ventricle outflow and aorta was found in one patient, secondary to undiagnosed conduit rupture. This was closed surgically. After a median follow-up of 20.16 (6.95 to 103.61) months, all the patients are asymptomatic with no significant RVOT stenosis. CONCLUSIONS: PPVI is feasible in small conduits but rate of ruptures is high. Although such ruptures remain contained and can be managed with covered stents in our experience, careful selection of patients and high level of expertise are necessary. More studies are needed to better assess the risk of PPVI in this population.

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A 39-year-old woman underwent uneventful percutaneous occlusion of an ostium secundum atrial septal defect (ASD) with a 22 mm Ultrasept ASD Occluder®. Transesophageal echocardiography (TEE) performed two years after implantation revealed a de novo residual left-to-right shunt through the correctly implanted device. Three-dimensional transesophageal echocardiography (3D TEE) further clarified this finding by showing a perforation of the device membrane coating. The patient underwent transcatheter closure of the residual shunt with a 20 mm Ultrasept PFO® device. The procedure was guided by fluoroscopy and real-time 3D TEE. At the end of the procedure 3D TEE documented correct device deployment with complete defect coverage and absence of residual shunt.

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OBJECTIVE: A case series on different vascular malformations (VM) treated with percutaneous occlusion in children and adults is presented. BACKGROUND: Percutaneous occlusion is usually the preferred treatment method for VM. Previous series have mostly focused on single types of devices and/or VM. METHODS: Retrospective analysis of all patients who underwent percutaneous occlusion of VM in a single center, from 1995 to 2014, excluding patent ductus arteriosus. Clinical and angiographic data, procedural details, implanted devices, and complications were assessed. Procedural success was defined as effective device deployment with none or minimal residual flow. Predictors of procedural failure and complications were determined by multivariate analysis. RESULTS: A total of 123 VM were intervened in 47 patients with median age of 12 years (25 days-76 years). The VM included 55 pulmonary arteriovenous fistulae, 39 aortopulmonary collaterals, 10 systemic venovenous collaterals, 8 peripheral arteriovenous fistulae, 5 Blalock-Taussig shunts, 4 coronary fistulae, and 2 Fontan fenestrations. The 143 devices used included 80 vascular plugs, 38 coils, 22 duct occluders, and 3 foramen ovale or atrial septal defect occluders. Median vessel size was 4.5 (2.0-16.0) mm and device/vessel size ratio was 1.4 (1.1-2.0). Successful occlusion was achieved in 118 (95.9%) VM, including three reinterventions. Four (3.3%) clinically relevant complications occurred, without permanent sequelae. Lower body weight was independently associated with procedural failure and complications. CONCLUSION: To our knowledge, this is the largest series on different VM occluded percutaneously in children and adults, excluding patent ductus arteriosus. Percutaneous occlusion was effective and safe, using different devices.

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INTRODUCTION: Hypoplastic left heart syndrome (HLHS) is a major cause of cardiac death during the first week of life. The hybrid approach is a reliable, reproducible treatment option for patients with HLHS. Herein we report our results using this approach, focusing on its efficacy, safety and late outcome. METHODS: We reviewed prospectively collected data on patients treated for HLHS using a hybrid approach between July 2007 and September 2014. RESULTS: Nine patients had a stage 1 hybrid procedure, with seven undergoing a comprehensive stage 2 procedure. One patient completed the Fontan procedure. Five patients underwent balloon atrial septostomy after the hybrid procedure; in three patients, a stent was placed across the atrial septum. There were three deaths: two early after the hybrid procedure and one early after stage two palliation. Overall survival was 66%. CONCLUSIONS: In our single-center series, the hybrid approach for HLHS yields intermediate results comparable to those of the Norwood strategy. The existence of dedicated teams for the diagnosis and management of these patients, preferably in high-volume centers, is of major importance in this condition.

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INTRODUCTION: Infantile hemangioma (IH) is one of the most common childhood tumors. There are various medical or surgical therapeutic options, all with suboptimal results. Recently, the successful use of propranolol for involution of IH was described. We report the results of a single-center experience with this therapeutic option. OBJECTIVE: To prospectively assess the efficacy and safety of propranolol in children with infantile hemangioma. METHODS: We performed a prospective analysis of clinical data of all patients with IH referred to a pediatric cardiology center for baseline cardiovascular assessment prior to propranolol therapy. Propranolol was given at a starting dose of 1 mg/kg/day and titrated to a target dose of 2-3 mg/kg/day according to clinical response. Efficacy was assessed through a photograph-based severity scoring scale. Safety was assessed by collecting data regarding significant side effects. RESULTS: Starting in 2010, 30 patients (15 female) were referred for propranolol treatment of IH, at a median age of six months (1-63 months). The mean target propranolol dose was 2.8 mg/kg/day, with a mean duration of therapy of 12 months. All patients experienced significant reduction of IH size and volume. There were no side effects. CONCLUSIONS: In our experience propranolol appears to be a useful and safe treatment option for severe or complicated IH, achieving a rapid and significant reduction in their size. No adverse effects were observed, although until larger clinical trials are completed, potential adverse events should be borne in mind and consultation with local specialists is recommended prior to initiating treatment.

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BACKGROUND: The neonatal arterial switch operation (ASO) is now the standard of care for children born with transposition of the great arteries. Stenosis of the neopulmonary artery on long-term follow up is a known complication. METHODS: We performed a retrospective analysis of eleven patients who underwent a cardiac magnetic resonance imaging (MRI) due to echocardiographic evidence suggestive of stenosis of the neopulmonary artery or its branches (mean estimated Doppler gradient 48 mmHg, min 30 mmHg, max 70 mmHg). A comprehensive evaluation of anatomy and perfusion was done by cardiac MRI. RESULTS: The branches of the neopulmonary artery (neo PA) showed decreased caliber in three patients unilaterally and in two patients, bilaterally. Magnetic resonance (MR) perfusion studies showed concomitant decreased flow, with discrepancy between the two lungs of 35/65% or worse, only in the three patients with unilateral obstruction, by two different MR perfusion methods. CONCLUSIONS: Cardiac MR can be used as a comprehensive non-invasive imaging technique to diagnose stenosis of the branches of the neopulmonary after the ASO, allowing evaluation of anatomy and function of the neoPA, its branches, and the differential perfusion to each lung, thus facilitating clinical decision making.

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This article, which shows a technically very challenging angioplasty of a near-atretic coarctation of the aorta, underlines the importance of an anticipated procedure planning and of the right selection of available technology. Additionally, the performance and clinical setting of utilization of a new small premounted stent--the Advanta Atrium stent--is highlighted.

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INTRODUCTION: Quadricuspid aortic valve is a rare malformation, with an estimated incidence of 0.003 to 0.043% of all congenital heart disease. It usually appears as an isolated congenital anomaly, but may also be associated with other malformations, the most common being coronary artery anomalies. Current technology enables noninvasive diagnosis in most cases. This entity's natural history is progression to valve regurgitation, which is rare before adulthood. OBJECTIVE: Case review of quadricuspid aortic valve patients diagnosed in the last 10 years in a tertiary pediatric cardiology center. METHODS: Retrospective chart review of patients diagnosed with quadricuspid aortic valve between January 2000 and December 2009. RESULTS: Over the past 10 years, four cases of quadricuspid aortic valve were diagnosed in children aged between 6 months and 8 years, two male. In three cases, the four leaflets were of similar size, which is the most common finding. Two of the valves functioned normally and two had minimal regurgitation. All patients had associated cardiac malformations (one atrial and two ventricular septal defects, one supravalvular aortic stenosis and one quadricuspid pulmonary valve). One patient was also diagnosed with Williams syndrome. During a median follow-up of 2 years (0-9), all patients remained asymptomatic and none required medical or surgical treatment of the aortic valve. CONCLUSION: Diagnosis of quadricuspid aortic valve is rare, especially in children, since most patients are asymptomatic and have normally functioning valves. In this study, half the patients had minimal aortic regurgitation. Contrary to what is described in the literature, all patients had concomitant cardiac malformations. We provide the first description of this entity's association with Williams syndrome. Clinical follow-up should be maintained in these patients in order to promptly detect the onset or worsening of functional alterations and to enable appropriate therapeutic intervention.

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Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart disease with a poor prognosis. It displays great anatomical diversity with varying degrees of right ventricular (RV) hypoplasia, which determine the therapeutic approach. Ideally, the goal is to establish a biventricular circulation. This may be achieved through surgical or percutaneous techniques. Pulmonary valve perforation using radiofrequency energy is indicated in patients with membranous atresia, moderate RV hypoplasia (bipartite or tripartite) and non-RV dependent coronary circulation. Arterial duct stenting is occasionally necessary to ensure adequate pulmonary flow. It is thus possible to treat some patients using exclusively percutaneous techniques. We report the first known case in Portugal of a neonate with PA/IVS treated by radiofrequency perforation and subsequent stenting of the arterial duct.

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The unilateral absence of one pulmonary artery is a rare congenital abnormality. The authors report a clinical case of a two-year-old boy with no previous medical history who was referred for evaluation after the detection of pulmonary asymmetry on the chest X-ray with a right mediastinal shift. The CT scan and pulmonary perfusion scintigraphy pointed to an absent right pulmonary artery, which was confirmed by right heart catheterization and cardiac magnetic resonance imaging. This is an important pathology because early diagnosis and timely correction can prevent future complications. Since at this time the patient is asymptomatic, the authors opted for careful clinical vigilance.

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INTRODUCTION: Coarctation of the aorta (CoA) is a stenosis usually located in the descending aorta. Treatment consists of surgical or percutaneous removal of the obstruction and presents excellent immediate results but significant residual problems often persist. OBJECTIVES: To describe the presentation, treatment and long-term evolution of a population of 100 unselected consecutive patients with isolated CoA in a single pediatric cardiology center. METHODS: This was a retrospective study of all patients with isolated CoA treated during4 the last 21 years (1987-2008). RESULTS: The patients (n=100, 68.3% male) were diagnosed at a median age of 94 days (1 day to 16 years). The clinical presentation differed between patients aged less or more than one year, the former presenting with heart failure and the latter being asymptomatic with evidence of hypertension (88 and 63%, respectively; p < 0.01). Treatment, a median of 8 days after diagnosis, was surgical in 79 cases (20 end-to-end anastomosis, 31 subclavian flap, 28 patch) and percutaneous in the remaining 21 (15 balloon angioplasty, 6 with stenting). The mean age of surgical patients was younger than in those treated percutaneously (3.4 vs. 7.5 years; p < 0.01). Immediate mortality was 2% and occurred in the surgical group. There was no late mortality, in a mean follow-up of 7.2 +/- 5.4 years. Recoarctation occurred in 8 patients (6 surgical, 2 percutaneous). There are 46 patients who currently have hypertension (19 at rest, 27 with effort), their median age at diagnosis being older than the others (23 vs. 995 days; p < 0.01). CONCLUSIONS: Isolated CoA has an excellent short-term prognosis but a significant incidence of long-term complications, and should thus no longer be seen as a simple obstruction in the descending aorta, but rather as a complex pathology that requires careful follow-up after treatment. Its potentially insidious presentation requires a high level of clinical suspicion, femoral pulse palpation during physical examination of newborns and older children being particularly important. Delay in treatment has an impact on late morbidity and mortality. Taking into account the data currently available on late and immediate results, the final choice of therapeutic technique depends on the patient's age, associated lesions and the experience of the medical-surgical team. Hypertension should be closely monitored in the follow-up of these patients, as well as its risk factors and complications.

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INTRODUCTION: Patients with congenital heart disease who undergo surgical implantation of a conduit between the right ventricle and the pulmonary artery (RV-PA conduit) may develop stenosis and/or insufficiency over time. These cases, which are associated with arrhythmias, RV dysfunction and sudden death, remain a challenge for surgical re-intervention, due to its complexity and associated morbidity and mortality. Percutaneous pulmonary valve implantation (PPVI) is therefore a valid alternative. OBJECTIVE: To report our center's initial experience with PPVI. METHODS: Prospective assessment of clinical, echocardiographic, magnetic resonance, hemodynamic and angiographic data from our series of PPVI. RESULTS: Six patients in NYHA functional class +/- II underwent PPVI. All had significant conduit dysfunction and five had stenosis. The procedure consisted of implanting a bare metal stent to reduce the risk of fracture of the Melody valved stents (Medtronic) that were then successfully deployed in all. The immediate hemodynamic results showed a reduction in RV pressure (94 +/-27 to 44 +/- 7 mmHg), RV/LV pressure ratio (94 +/-27 to 44 +/- 7%) and conduit gradient (65 +/- 28 to 11 +/- 4 mmHg), and no insufficiency. The median duration of the procedure was 180 minutes, with no major complications. Patients were discharged a median of two days after the procedure. After a median follow-up of 7.8 months, patients are in functional class I (5) or II (1), with no evidence of conduit dysfunction on non-invasive assessment. CONCLUSIONS: Our results are similar to the excellent results reported in larger series. PPVI is a valid therapeutic option in patients with conduit dysfunction.

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