[Anomalous origin of right coronary artery from pulmonary artery associated to left main coronary stenosis]. / Anomalie de connexion de l'artère coronaire droite au niveau de l'artère pulmonaire associée à une sténose du tronc commun gauche.

We describe herein a case of a 16 years-old female patient referred to our department for further exploration of a chest pain. Color Doppler echocardiography revealed an abnormal flow at the level of the pulmonary artery. Exercise testing was abnormal leading to further imaging including computed cardiac tomography followed by coronary angiography which showed anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) associated with mid shaft left main stenosis. We discuss this uncommon association and therapeutic options.

Multicenter Experience for Early and Mid-Term Outcome of MyVal Transcatheter Pulmonary Valve Implantation.

Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has recently been reported to be implanted in pulmonary position. Myval transcatheter valve were implanted in patients with stenosed dysfunctional conduits, severe regurgitation from transannular patch or dysfunctional surgical pulmonary valves (Bioprosthesis). Procedural details and post-TPVI follow-up were analysed. Myval TPVI was used in Fifty three patients with median age of 15 years (IQR 12-19.5 years). Almost sixty percent of the patients were male, with a median weight of 50 kg (31-63 kg). Prestenting was used in more than 80 percent of patients (n = 45 patients), while 6 patients had a prior surgical valve implantation. After Myval TPVI implantation, the peak instantaneous gradient across the RVOT decreased from a median of 23.5 mmHg (IQR 10-53 mmHg) pre-procedure to 10 mmHg (IQR 5-16 mmHg) post-procedure. The median fluoroscopy time for the procedure was 35 min (IQR 23.5-44 min). The large sizes-mainly the 29-mm and 32 mm Myval (Navigator, Meril Life Sciences Pvt Ltd, India), were the most used size in 40% (n = 22) of the cases each. The median contrast volume used during the procedure was 247 mL (IQR 120-300 mL). Patients were followed for a median period of 360 days (IQR 164-525 days). At the last clinic follow-up, there was no tricuspid valve regurgitation. Moderate neo-pulmonary valve regurgitation was reported in three cases. Early experience of TPVI with MyVal is encouraging with procedural success in all patients and acceptable mid-term outcomes.

Transcatheter Closure of Superior Sinus Venosus Defects.

Superior sinus venosus defect is a communication between the right and left atrium located above the upper margin of the oval fossa, immediately inferior to the junction of the superior vena cava and the right atrium. It is systematically associated with partial anomalous pulmonary venous drainage, especially of the right upper pulmonary vein. Surgical repair has been the gold standard approach to close that defect. Introduced in 2014, percutaneous closure has gradually become a safe and effective alternative to surgery in carefully selected patients, although worldwide experience remains limited. This article provides an appraisal of the patients' selection process and a step-by-step description of the procedure as well as a comprehensive review of its outcomes.

Use of BeSmooth peripheral stent in paediatric cardiology.

I read with interest the article by Haddad et al concerning the bench testing of BeSmooth peripheral tests, and I would like to share some thoughts and highlight few points.BeSmooth stent is behaving like any pre-mounted stent available on the market. None can achieve the Holy Grail of being dilatable to adult size without losing their integrity. Until dedicated stents are available, pre-mounted stents are preferred to unmounted stents because of their better profile and deliverability. Technically, proper post-dilatation should be performed in order to avoid and extensive foreshortening and the formation of a ring that would limit further expansion of the stent. BeSmooth stent like other pre-mounted stents can also be used without restriction in situations where overexpansion is not required.BeSmooth stent platform with the knowledge of its intrinsic properties is, in our opinion, a good add-on to the armamentarium of pre-mounted stents used in paediatric cardiology.

Novel technique for transcatheter closure of sinus venosus atrial septal defect: The temporary suture-holding technique.

BACKGROUND: Transcatheter repair of sinus venosus atrial septal defect (SVASD) has become an alternative option to surgical repair. There are potential significant complications related to stent stability in the superior vena cava (SVC) and potential migration of the stent that need to be addressed. Therefore, the technique is still evolving. OBJECTIVES: To report results of a new modification "the suture technique" that improves safety profile of positioning and securing a covered stent in the SVC. METHODS: This is a descriptive, single center, retrospective review of patients who underwent SVASD closure using the suture technique at our institution between 02/2020 and 08/2022. RESULTS: Fourteen patients underwent transcatheter repair of SVASD using the suture technique. All procedures were successful. The suture technique allowed precise stent placement in all patients without any migration or complication. Six patients required additional stent placement at the level of the SVC. One patient had an additional covered stent placed to eliminate a tiny residual shunt. Two patients had negligible residual shunts at the time of the procedure. At follow-up, all patients clinically improved and had significant reduction in right heart size on echocardiography and/or magnetic resonance imaging. No arrhythmia was reported in any patient. None required re-intervention after a mean follow-up of 16.5 ± SD 10.5 months. CONCLUSIONS: The suture technique appears to be safe modification. Although our study involves small sample size with no comparative group, we believe our technique offers greater control over stent positioning, reducing the risk of stent embolization and residual shunting in transcatheter closure of SVASD.

Transcatheter interventions in patients with a Fontan circulation: Current practice and future developments.

The Fontan operation represents the last of multiple steps that are offered a wide range of congenital cardiac lesions with a single ventricle (SV) physiology. Nowadays this surgical program consists of a total cavopulmonary connection (TCPC), by anastomosing systemic veins to the pulmonary arteries (PAs), excluding the right-sided circulation from the heart. As a result of imaging, surgical, percutaneous, and critical care improvements, survival in this population has steadily increased. However, the Fontan physiology chronically increases systemic venous pressure causing systemic venous congestion and decreased cardiac output, exposing patients to the failure of the Fontan circulation (FC), which is associated with a wide variety of clinical complications such as liver disease, cyanosis, thromboembolism, protein-losing enteropathy (PLE), plastic bronchitis (PB), and renal dysfunction, ultimately resulting in an increased risk of exercise intolerance, arrhythmias, and premature death. The pathophysiology of the failing Fontan is complex and multifactorial; i.e., caused by the single ventricle dysfunction (diastolic/systolic failure, arrhythmias, AV valve regurgitation, etc.) or caused by the specific circulation (conduits, pulmonary vessels, etc.). The treatment is still challenging and may include multiple options and tools. Among the possible options, today, interventional catheterization is a reliable option, through which different procedures can target various failing elements of the FC. In this review, we aim to provide an overview of indications, techniques, and results of transcatheter options to treat cavopulmonary stenosis, collaterals, impaired lymphatic drainage, and the management of the fenestration, as well as to explore the recent advancements and clinical applications of transcatheter cavopulmonary connections, percutaneous valvular treatments, and to discuss the future perspectives of percutaneous therapies in the Fontan population.

Three centers experience with device closure of congenital Gerbode-type perimembranous ventricular septal defects.

OBJECTIVES: We aim to evaluate our experience with interventional closure of Gerbode-type perimembranous ventricular septal defects (pmVSDs). METHODS: We performed three-center retrospective data review of patients with congenital indirect Gerbode-type pmVSDs treated percutaneously between August 2017 and May 2021. Standard safety and latest follow-up outcomes were assessed. RESULTS: Ten patients (six females) were identified with a median age of 6.8 years (range: 2.5-54) and a median weight of 26.5 kg (range: 12-88). The median left ventricular defect size was 10 mm (range: 3-15.5). On baseline ultrasound, 6 patients had absent subaortic rim , 6 patients had trivial aortic regurgitation, and 3 patients had tear-drop-type (small) aortic cusp prolapse. The tricuspid regurgitation was graded II (n = 5) and III (n = 5). Five Lifetech Konar-Multifunctional occluders, four Amplatzer duct occluders II and one Amplatzer duct occluder I were implanted. The median fluoroscopy time was 10.4 min (range: 4.3-20.2). Pre-existing aortic regurgitations remained identical. One new aortic regurgitation was identified before discharge and remained trivial after 48 months of follow-up. No heart block or tricuspid stenosis was observed on a median follow-up of 17 months (range: 3-48). All patients are symptom-free with complete shunt closure and significant regression or resolution of tricuspid regurgitation. CONCLUSIONS: Despite anatomical challenges, interventional closure of congenital indirect Gerbode-type pmVSD appears to be feasible, safe, and most importantly clinically effective using different commercially available devices. Amplatzer duct occluder II and Lifetech Konar-Multifunctional occluder offer interesting specifications to retrogradely target this specific defect with success.

Multiscale modelling of Potts shunt as a potential palliative treatment for suprasystemic idiopathic pulmonary artery hypertension: a paediatric case study.

Potts shunt (PS) was suggested as palliation for patients with suprasystemic pulmonary arterial hypertension (PAH) and right ventricular (RV) failure. PS, however, can result in poorly understood mortality. Here, a patient-specific geometrical multiscale model of PAH physiology and PS is developed for a paediatric PAH patient with stent-based PS. In the model, 7.6mm-diameter PS produces near-equalisation of the aortic and PA pressures and [Formula: see text] (oxygenated vs deoxygenated blood flow) ratio of 0.72 associated with a 16% decrease of left ventricular (LV) output and 18% increase of RV output. The flow from LV to aortic arch branches increases by 16%, while LV contribution to the lower body flow decreases by 29%. Total flow in the descending aorta (DAo) increases by 18% due to RV contribution through the PS with flow into the distal PA branches decreasing. PS induces 18% increase of RV work due to its larger stroke volume pumped against lower afterload. Nonetheless, larger RV work does not lead to increased RV end-diastolic volume. Three-dimensional flow assessment demonstrates the PS jet impinging with a high velocity and wall shear stress on the opposite DAo wall with the most of the shunt flow being diverted to the DAo. Increasing the PS diameter from 5mm up to 10mm results in a nearly linear increase in post-operative shunt flow and a nearly linear decrease in shunt pressure-drop. In conclusion, this model reasonably represents patient-specific haemodynamics pre- and post-creation of the PS, providing insights into physiology of this complex condition, and presents a predictive tool that could be useful for clinical decision-making regarding suitability for PS in PAH patients with drug-resistant suprasystemic PAH.

Topsy-Turvy Heart with Aortopulmonary Window and Severe Airway Malacia: Prenatal Diagnosis and Review of the Literature.

The topsy-turvy heart is a very rare cardiac malformation that involves a global 90° clockwise rotation of the heart along its long axis. This rotation results in the displacement of the great arteries and severe elongation and stretching of the brachiocephalic arteries and the bronchi. We present an unusual case of topsy-turvy heart diagnosed prenatally with a large aorto-pulmonary window and. This case gives an insight into the morphological details and clinical presentation of this rare malformation and its associated complications. We also present a review of the literature of this rare anomaly showing only 15 live cases that have been published with only three cases diagnosed prenatally.

Correction of sinus venosus atrial septal defects with the 10 zig covered Cheatham-platinum stent - An international registry.

BACKGROUND: Covered stent correction of sinus venosus ASDs (SVASD) is a relatively new technique. Challenges include anchoring a sufficiently long stent in a nonstenotic superior vena cava (SVC) and expanding the stent at the wider SVC-RA junction without obstructing the anomalous right upper pulmonary vein (RUPV). The 10-zig covered Cheatham-platinum (CCP) stent has the advantage of being available in lengths of 5-11 cm and dilatable to 34 mm in diameter. METHODS: An international registry reviewed the outcomes of 10-zig CCP stents in 75 patients aged 11.4-75.9 years (median 45.4) from March 2016. Additional stents were used to anchor the stent in the SVC or close residual shunts in 33/75. An additional stent was placed in 4/5 (80%) with 5/5.5 cm CCPs, 18/29 (62%) with 6 cm CCPs, 5/18 (28%) with 7 cm CCPs, 5/22 (23%) with 7.5/8 cm CCPs and 0/1 with an 11 cm CCP. A "protective" balloon catheter was inflated in the RUPV in 17. RESULTS: Early stent embolization in two patients required surgical removal and defect repair and tamponade was drained in one patient. The CT at 3 months showed occlusion of the RUPV in one patient. Follow up is from 2 months to 5.1 years (median 1.8 years). QP:QS has reduced from 2.5 ± 0.5 to 1.2 ± 0.36 (p < .001) and RVEDVi from 149.1 ± 35.4 to 95.6 ± 21.43 ml/m2 (p < .001). CONCLUSIONS: Ten-zig CCPs of 7-8 cm appear to provide reliable SVASD closure with a low requirement for additional stents. Careful selection of patients and meticulous attention to detail is required to avoid complications.

Mid-Term Outcomes Following Percutaneous Pulmonary Valve Implantation Using the "Folded Melody Valve" Technique.

BACKGROUND: The folded valve is a manual shortening of the Melody device, which has been validated as a valuable therapeutic option for the management of dysfunctional right ventricular outflow tracts needing a short valved stent. In this article, we aimed to evaluate, in a multicenter cohort, the mid-term outcomes of patients in whom a percutaneous pulmonary valve implantation was performed using the folded valve technique. METHODS: A 2012 to 2018 retrospective multicenter study was performed in 7 European institutions. All patients who benefit from percutaneous pulmonary valve implantation with a folded Melody valve were included. RESULTS: A total of 49 patients (median age, 19 years [range 4­56], 63% male) were included. The primary percutaneous pulmonary valve implantation indication was right ventricular outflow tract stenosis (n=19; 39%), patched native right ventricular outflow tracts were the most common substrate (n=15; 31%). The folded technique was mostly used in short right ventricular outflow tracts (n=28; 57%). Procedural success was 100%. After a median follow-up of 28 months (range, 4­80), folded Melody valve function was comparable to the immediate postimplantation period (mean transvalvular peak velocity=2.6±0.6 versus 2.4±0.6 m/s, P>0.1; only 2 patients had mild pulmonary regurgitation). Incidence rate of valve-related reinterventions was 2.1% per person per year (95% CI, 0.1%­3.9%). The probability of survival without valve-related reinterventions at 36 months was 90% (95% CI, 76%­100%). CONCLUSIONS: The folded Melody valve is a safe technique with favorable mid-term outcomes up to 6.5 years after implantation, comparable with the usual Melody valve implantation procedure. Complications and reinterventions rates were low, making this technique relevant in selected patients.

A case series of transcatheter Potts Shunt creation in a pediatric population affected with refractory pulmonary artery hypertension: focus on the role of ECMO.

PURPOSE: Patients with suprasystemic idiopathic pulmonary hypertension (S-PAH) have a poor prognosis. Therapeutic options are limited. Reverse Potts shunt creation modifies physiology transforming patients with PAH into Eisenmenger physiology with a better outcome. Percutaneous transcatheter stent secured aortopulmonary connection (transcatheter Potts Shunt, TPS) is a feasible very high-risk procedural option in such patients. We report our experience with patients undergoing TPS at our institution requiring extracorporeal membrane oxygenation (ECMO) support. METHODS: A prospective observational study of patients with drug-refractory PAH, worsening NYHA class, and right ventricular failure undergoing TPS. Two patients required rescue ECMO for cardiac arrest during the procedure. Subsequently, "standby ECMO" was available in all the following cases and elective support was provided in patients with extremely poor conditions. RESULTS: Ten pediatric patients, underwent TPS at our institution. Two patients were rescued by ECMO after cardiac arrest during the shunt creation. This occurred as a result of the acute loading of the left ventricle (LV) after retrograde aortic arch filling through the Potts shunt. Following this, another two patients underwent elective ECMO after the uneventful induction of anesthesia. They all died postoperatively despite a successful TPS procedure. The causes of death were not related to the use of ECMO, but the complication of severe PAH. Six patients with successful TPS did not require ECMO and survived. CONCLUSIONS: TPS is a pioneering procedure offering the opportunity to treat high-risk idiopathic drug-refractory PAH patients. Acute LV failure is a complication of TPS in patients with S-PAH. Elective ECMO, an option to avoid circulatory arrest and acute profound hypoxia secondary to exclusive right-to left shunt systemic perfusion by Potts shunt and LV dysfunction with resulting pulmonary edema, may be used at the early stage of the learning curve, but it does not influence the prognosis of these patients which remains poor.

Aortic atresia with interrupted aortic arch and bilateral arterial ductus: a successful initial palliation.

A combination of aortic valve atresia and an interrupted aortic arch is a unique disease in which perfusion to the brain and myocardium depends on coexisting lesions or type of interruption. We report a case of aortic valve atresia with type B interrupted arch, bilateral arterial ductus in a neonate who was successfully palliated using a hybrid approach by placing stents in both arterial ductus and banding of branch pulmonary arteries.

Comparison of Two Percutaneous Atrial Septal Defect Occluders for Device Healing and Nickel Release in a Chronic Porcine Model.

AIMS: To investigate the healing process and nickel release of the Hyperion occluder (Comed BV, Netherlands), as compared to the Amplatzer septal occluder (ASO) (St. Jude Medical Inc., St. Paul, MN, USA) in a chronic swine model. BACKGROUND: Some long-term complications occurring after percutaneous atrial septal defect (ASD) closure may be partially associated with an inappropriate healing of the device and increased nickel release. There is no direct comparative study of different occluders for healing and nickel release. METHODS: After percutaneous ASD creation, 12 pigs were implanted with 15 mm Hyperion (n = 6) and 15 mm ASO (n = 6) devices. After 1 month (n = 3 for each device) and 3 months (n = 3 for each device) of follow-up, device explantation was performed and healing was assessed using histopathological workup. Systemic and tissular nickel release was performed. RESULTS: Implantation was successful in 100% without complications. Device coverage was observed as early as 1 month after implantation and was almost complete after 3 months. A granulation tissue with a predominantly mononuclear inflammatory reaction was observed in contact with nitinol wires while an inflammatory reaction was seen in contact with textile fibers. We found no statistically significant difference between the 2 devices whether for histological grading scores or systemic nickel release, regardless to follow-up duration. CONCLUSIONS: In this preclinical study, we demonstrated that Amplatzer septal occluder and Hyperion occluder were not significantly different for device healing and nickel release processes.

Tetralogy of Fallot Will be Treated Interventionally Within Two Decades.

Tetralogy of Fallot is considered a prototype congenital heart disease because of its embryological, anatomical, pathophysiological, and management aspects. Current management usually relies on a complete surgical repair that is electively performed between 3 and 6 months of age. With the advances of interventional cardiology especially in the fields of ventricular septal defect closure, stent, and pulmonary valve replacement, the question of complete repair of tetralogy of Fallot by interventional means can be discussed. Tetralogy of Fallot is a complex disease with multiple lesions, all individually amenable to transcatheter treatment. In this article, we will review current status of various aspects of tetralogy of Fallot focusing on interventional aspects, giving insights of what would be the ideal platform of a fully interventional repair.

Percutaneous closure of patent ductus arteriosus in premature infants: A French national survey.

BACKGROUND: Transcatheter closure of patent ductus arteriosus (PDA) in premature infants has been shown to be feasible in small series. Outcomes in larger series are currently lacking. MATERIAL: All premature infants (< 36 weeks GA) who underwent transcatheter PDA closure were included in a multicenter French national survey. Demographic data (gestational age [GA], birth weight [BW]) and procedural data (weight [PW], age at procedure [AP], procedural success, fluoroscopy time, and type of device) were collected. Outcomes and procedural complications were reviewed. RESULTS: Between September 2013 and June 2017, 102 patients were included. In 71 cases, PDA pharmacological closure had been attempted. Mean GA was 27 ± 2.9 weeks. Mean BW and PW were 1,040 ± 715 g and 1,543 ± 698 g, respectively. Mean AP was 39 ± 26 days. Number of premature infants <1 kg, between 1 and 2 kg, and > 2 kg was 21, 59, and 22, respectively. Mean fluoroscopic time was 6.5 min. Success rate was 99%. Device- or procedure-related complications were reported in nine patients (8.9%) including three LPA stenoses (requiring surgery in two and balloon dilatation in one), two neo-coarctations (one requiring subsequent surgery), and three instances of tricuspid valve regurgitation at follow-up. Seven deaths were reported, none being related to the procedure. Mean follow-up was 39.75 ± 13.1 months. CONCLUSION: In this large series of premature infants undergoing transcatheter PDA closure, it was demonstrated that this procedure can be performed successfully in the vast majority of patients with an acceptable complication rate. Future efforts should focus on minimizing complications, particularly device-related vascular stenoses.

Transcatheter ductus arteriosus stenting in paediatric cardiology: Indications, results and perspectives.

Stenting the arterial duct emerged in the early 1990s as an alternative to a variety of surgical interventions in neonates with a duct-dependent pulmonary or systemic circulation complex defect. Furthermore, palliative ductal stenting has been applied in older children with severe suprasystemic pulmonary arterial hypertension, as an alternative to surgical shunts, such as Potts anastomosis. Early results of this technique were discouraging, but by learning from the failures of the past, ductal stenting has become a reliable palliative therapy. In this review, we aim to describe the historical evolution of ductal stenting, its different clinical applications and outcomes, and future perspectives for this strategy in congenital cardiac catheterization.

Cardiac catheterisation in infants weighing less than 2500 grams.

OBJECTIVES: The aim of the study was to report the outcome of cardiac catheterisation in low-weight patients. BACKGROUND: Data regarding cardiac catheterisation in infants weighing <2500 g are scarce. METHODS: We reviewed all cardiac catheterisations performed in infants weighing <2500 g between January 2000 and May 2016. An analysis with respect to the type of procedure, the complexity of procedure (procedure type risk), and haemodynamic vulnerability index was finally carried out. We report the occurrence of deaths and complications using the adverse event severity score. RESULTS: A total of 218 procedures were performed on 211 patients. The mean age and weight were, respectively, 15 ± 26 days (range, 0-152) and 2111 ± 338 g (range, 1000-2500). Procedures were interventional and diagnostic, respectively, in 174 (80%) and 44 (20%) patients. Out of 218, 205 (94%) were successful. Eleven complications (5%) occurred - six with an adverse event severity score of 4 and five with an adverse event severity score of 3. Ten patients (91%) showed a favourable outcome, and one died (stent thrombosis few hours after patent ductus arteriosus stenting). No correlation was found between lower weight and occurrence of death (p = 0.68) or complications (p = 0.23). The gravity scores (procedure type risk and haemodynamic vulnerability index) were not predictive of complications. CONCLUSIONS: Cardiac catheterisation in infants weighing <2500 g appears feasible and effective with low risk. The weight should not discourage from performing cardiac catheterisation in this population.

A randomized, controlled, multi-center trial of the efficacy and safety of the Occlutech Figulla Flex-II Occluder compared to the Amplatzer Septal Occluder for transcatheter closure of secundum atrial septal defects.

AIMS: The aim of this study was to compare the efficacy and safety of the Occlutech Figulla Flex II Occluder (OFFII) with the Amplatzer Septal Occluder (ASO) in patients > 8kg undergoing transcatheter ASD closure. METHODS AND RESULTS: Randomized, controlled, multi-center prospective clinical trial with randomization 2:1 in favor of the OFFII. Primary efficacy endpoint was the rate of successful device placement and defect closure without major complications at hospital discharge. All data were assessed through a core laboratory. Interim analysis was performed when 70% of the patients were treated to evaluate for noninferiority. From a total of 176 randomized subjects, interim analysis was performed on the first 158 patients (65.2% female) (107 OFFII/51 ASO) undergoing device closure at a median weight of 42 kg (range 13-125 kg). Seventy-six percent (120 patients) completed 6-month follow-up. Successful device placement (first attempt) was achieved in 99.1% of the OFF group vs 90.2% of the ASO group (P < 0.05). Early efficacy success was achieved in 94.4% of the OFFII group vs 90.2% of the ASO group (P < 0.001). The incidence of major complications was 5.6% for the OFFII group compared to 9.8% for the ASO. CONCLUSIONS: The OFFII device was not inferior to the ASO with less complications and greater efficacy than the ASO.