Transcatheter Fontan completion: Creation of an extracardiac Fontan.

Patients with functionally univentricular hearts are usually palliated surgically. There have been several reports of successful attempts to complete the Fontan procedure without surgery. The pathways created at the time of the preconditioning were largely reminiscent of the lateral tunnel Fontan. However, this approach is still confidentially limited to a small number of centers. In 2013, we designed a circuit that mimics the actual surgical technique of extracardiac total cavopulmonary connection to allow for transcatheter completion in an animal study. A polytetrafluoroethylene conduit was connected between the pulmonary artery and the inferior vena cava (IVC). The superior anastomosis was occluded to avoid flow between IVC and superior vena cava (SVC). The conduit was connected to the right atrium (RA) and a large fenestration was created to allow free flow from the IVC to the RA. Extrapolating our approach, a center reported the successful transcatheter completion of an extracardiac Fontan in a 6-year-old child. However, this technique is not directly transposable to our population of patients who require preconditioning in infancy. We report here an innovative extension of this technique that may allow preparing patients in infancy, ideally at the time of the Glenn in the future, to receive an extracardiac Fontan at 2 years/11 kg without additional surgery.

Assessing the State of Training in Congenital Interventional Cardiology: A Global Survey of Program Directors.

This study aimed to evaluate the current state of congenital interventional cardiology training worldwide, with a focus on case volumes, competency assessment, and the need for ongoing mentorship during early career stages. A survey was conducted among program directors (PDs) of congenital interventional training programs across the globe. The survey gathered data on training pathways, case volumes, types of procedures performed, trainee competency assessment, and the role of ongoing mentorship. Of the 79 PDs who completed the survey, it was observed that training pathways and case volumes varied significantly, particularly between the United States and other countries. Most PDs reported an annual laboratory case volume of >500 congenital cardiac cases, with most cases being interventional. While trainees demonstrated competency in simple procedures (diagnostic cases, simple ASD closure), complex interventions (such as patent ductus arteriosus closure in premature infants) require ongoing mentorship for graduates. PDs recommended a minimum case volume of 400 total cases for trainees, including 250 interventional cases. In addition to case volumes, assessing trainee competency was deemed important, with clinical reasoning, judgment, skillset, teamwork, and complication management being key areas of evaluation. The study highlights the variability in congenital interventional cardiology training and the need for ongoing mentorship during the early career years. External mentorship programs, facilitated by national and international societies, are proposed to provide critical support for early career interventionalists thus enhancing patient care for congenital heart disease. Ultimately, the findings of this survey may serve as a framework for future training standards and guidelines in this specialized field.

Current status of transcatheter intervention for complex right ventricular outflow tract abnormalities.

Various transcatheter interventions for the right ventricular outflow tract (RVOT) have been introduced and developed in recent decades. Transcatheter pulmonary valve perforation was first introduced in the 1990s. Radiofrequency wire perforation has been the approach of choice for membranous pulmonary atresia in newborns, with high success rates, although complication rates remain relatively common. Stenting of the RVOT is a novel palliative treatment that may improve hemodynamics in neonatal patients with reduced pulmonary blood flow and RVOT obstruction. Whether this option is superior to other surgical palliative strategies or early primary repair of tetralogy of Fallot remains unclear. Transcatheter pulmonary valve replacement has been one of the biggest innovations in the last two decades. With the success of the Melody and SAPIEN valves, this technique has evolved into the gold standard therapy for RVOT abnormalities with excellent procedural safety and efficacy. Challenges remain in managing the wide heterogeneity of postoperative lesions seen in RVOT, and various technical modifications, such as pre-stenting, valve ring modification, or development of self-expanding systems, have been made. Recent large studies have revealed outcomes comparable to those of surgery, with less morbidity. Further experience and multicenter studies and registries to compare the outcomes of various strategies are necessary, with the ultimate goal of a single-step, minimally invasive approach offering the best longer-term anatomical and physiological results.

2024 Statement from Asia expert operators on transcatheter pulmonary valve replacement.

Transcatheter pulmonary valve replacement (TPVR), also known as percutaneous pulmonary valve implantation, refers to a minimally invasive technique that replaces the pulmonary valve by delivering an artificial pulmonary prosthesis through a catheter into the diseased pulmonary valve under the guidance of X-ray and/or echocardiogram while the heart is still beating not arrested. In recent years, TPVR has achieved remarkable progress in device development, evidence-based medicine proof and clinical experience. To update the knowledge of TPVR in a timely fashion, and according to the latest research and further facilitate the standardized and healthy development of TPVR in Asia, we have updated this consensus statement. After systematical review of the relevant literature with an in-depth analysis of eight main issues, we finally established eight core viewpoints, including indication recommendation, device selection, perioperative evaluation, procedure precautions, and prevention and treatment of complications.

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 perimembranous ventricular septal defect using a novel fully bioabsorbable occluder: multicenter randomized controlled trial.

Although the use of bioabsorbable occluder is expected to reduce the risk of metal occluder-related complications, it has not been approved due to incomplete degradation and new complications. Novel fully bioabsorbable occluders were designed to overcome such limitations. The aim of this study was to investigate the efficacy and safety of a fully biodegradable occluder in patients with ventricular septal defects. 125 patients with perimembranous ventricular septal defect (VSD) larger than 3 mm were screened from April 2019 to January 2020 in seven centers. 108 patients were enrolled and randomized into the bioabsorbable occluder group (n = 54 patients) and nitinol occluder group (n = 54). A non-inferiority design was utilized and all patients underwent transcatheter device occlusion. Outcomes were analyzed with a 24-month follow-up. All patients were successfully implanted and completed the trial. No residual shunt >2 mm was observed during follow-up. Transthoracic echocardiography showed a hyperechoic area corresponding to the bioabsorbable occluder which decreased primarily during the first year after implantation and disappeared within 24 months. Postprocedural arrhythmia was the only occluder-related complication with an incidence of 5.56% and 14.81% for the bioabsorbable and nitinol groups, respectively (P = 0.112). The incidence of sustained conduction block was lower in the bioabsorbable occluder group (0/54 vs. 6/54, P = 0.036) at 24-month follow-up. In conclusion, the novel fully bioabsorbable occluder can be successfully and safely implanted under echocardiography guidance and reduce the incidence of sustained postprocedural arrythmia. The efficacy and safety of this fully biodegradable occluder are non-inferior to that of a traditional nitinol one.

Unique challenges posed by a dysfunctional native right ventricular outflow tract for percutaneous pulmonary valve implantation using SAPIEN-S3 valve.

Dysfunctional right ventricular outflow tracts after a repair for tetralogy of Fallot using a transannular patch offer limited nonsurgical opportunities due to their large dimensions. A discrete subannular narrowing between a dilated right ventricle and the enlarged pulmonary trunk was a potential anatomical target for the creation of a landing zone using a prestent in a young male with severe pulmonary regurgitation and moderate stenosis. Asymmetric expansion of the prestent in the angulated outflow tract led to distal stent embolization that was stabilized by another telescoping stent before successful valve implantation. This manuscript details the unique challenges posed by a dilated regurgitant outflow tract for implanting a pulmonary valve.

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.

Hemodynamic and Structural Comparison of Human Fetal Heart Development Between Normally Growing and Hypoplastic Left Heart Syndrome-Diagnosed Hearts.

Congenital heart defects (CHDs) affect a wide range of societies with an incidence rate of 1.0-1.2%. These defects initiate at the early developmental stage and result in critical health disorders. Although genetic factors play a role in the formation of CHDs, the occurrence of cases in families with no history of CHDs suggests that mechanobiological forces may also play a role in the initiation and progression of CHDs. Hypoplastic left heart syndrome (HLHS) is a critical CHD, which is responsible for 25-40% of all prenatal cardiac deaths. The comparison of healthy and HLHS hearts helps in understanding the main hemodynamic differences related to HLHS. Echocardiography is the most common imaging modality utilized for fetal cardiac assessment. In this study, we utilized echocardiographic images to compare healthy and HLHS human fetal hearts for determining the differences in terms of heart chamber dimensions, valvular flow rates, and hemodynamics. The cross-sectional areas of chamber dimensions are determined from 2D b-mode ultrasound images. Valvular flow rates are measured via Doppler echocardiography, and hemodynamic quantifications are performed with the use of computational fluid dynamics (CFD) simulations. The obtained results indicate that cross-sectional areas of the left and right sides of the heart are similar for healthy fetuses during gestational development. The left side of HLHS heart is underdeveloped, and as a result, the hemodynamic parameters such as flow velocity, pressure, and wall shear stress (WSS) are significantly altered compared to those of healthy hearts.

The re-occurrence of dilated cardiomyopathy in propionic acidemia after liver transplantation requiring heart transplant, first case from Middle East.

Propionic acidemia is a rare autosomal recessive inborn error of metabolism. It is relatively common in Middle East. Dilated cardiomyopathy is one of the leading causes of morbidity and mortality for patients with propionic acidemia. Liver transplantation has been used for patient with frequent metabolic decompensations and was shown to be beneficial in propionic acidemia-related dilated cardiomyopathy. Up to our knowledge, there has been one reported case of recurrent dilated cardiomyopathy 3 years after liver transplantation. We report the first case, from Middle East, of recurrent dilated cardiomyopathy, 6 years after liver transplantation.

Safety of Occlutech Septal Occluder ACCELL Flex II for Transcatheter Closure of Secundum Atrial Septal Defects in Children: A Long-Term Follow-Up.

OBJECTIVES: To assess the long-term safety and efficacy of the Occlutech® ACCELL® Flex II device used for atrial septal defect (ASD) closure. This device differs from the regular device by having two very thin patches that are made of polyethylene terephthalate (PET). These patches enhance faster sealing of the defect. BACKGROUND: Transcatheter closure has become the method of choice to manage most patients with secundum ASDs. There are different types of devices. The regular Occlutech device used to close an ASD is called the Occlutech Figulla Flex II. A newer modification of this device (Occlutech® ACCELL® Flex II) has been designed to eliminate/reduce thrombus formation and to enhance faster sealing. METHODS: Thirty patients were followed up after occlusion of secundum ASD using the Occlutech® ACCELL® Flex II Device. The follow-up period ranged from 5.2-5.5 years with median of 5.3 years. Detailed history and full clinical examination, twelve-lead electrocardiogram (ECG), plain chest radiograph, and full 2D transthoracic echocardiography (TTE) were performed at discharge, at one month, six months, and yearly thereafter. RESULTS: The mean age of the study group at the last follow-up was 10.4 ± 4.6 years, with 63.3% (nineteen patients) females. There were no residual shunts or complications encountered immediately after the procedure and at the latest follow-up. CONCLUSION: This study confirmed the transcatheter closure (TCC) of secundum ASDs using the Occlutech® ACCELL® Flex II device to be safe and effective with no complications detected in children and adolescents.

Hot topics in interventional cardiology: Proceedings from the society for cardiovascular angiography and interventions (SCAI) 2021 think tank.

The Society for Cardiovascular Angiography and Interventions (SCAI) Think Tank is a collaborative venture that brings together interventional cardiologists, administrative partners, and select members of the cardiovascular industry community annually for high-level field-wide discussions. The 2021 Think Tank was organized into four parallel sessions reflective of the field of interventional cardiology: (a) coronary intervention, (b) endovascular medicine, (c) structural heart disease, and (d) congenital heart disease. Each session was moderated by a senior content expert and co-moderated by a member of SCAI's Emerging Leader Mentorship program. This document presents the proceedings to the wider cardiovascular community in order to enhance participation in this discussion, create additional dialog from a broader base, and thereby aid SCAI, the industry community and external stakeholders in developing specific action items to move these areas forward.

Congenital Aorto-Cardiac Connections (CACC) Revisited: Introduction of a Novel Anatomic-therapeutic Classification.

Abnormal congenital aorto-cardiac communications (CACC) are a heterogeneous constellation of anomalies that provide an abnormal connection between the aorta and other cardiac chambers or structures, including the atria, ventricles, the main pulmonary artery, and the coronary sinus. The current terminology of CACC has significant errors and shortcomings including inconsistent and interchangeable use of terms of fistula and tunnel and lack of an inclusive classification with practical information on therapeutic management. The aims of this study were threefold: firstly, to perform a concise narrative review of congenital pathologic connections between the aortic root and cardiac chambers which include rupture of congenital sinus of Valsalva aneurysm, aorto-left ventricular and less commonly right ventricular tunnels, coronary cameral fistulas, and aorto-atrial communications; secondly, to investigate the differentiating features of the so-called aorta right atrial tunnel (ARAT), with and without coronary artery take-off from the tunnel, and coronary cameral fistula (CCF) by applying a differential diagnostic assistance toolbox to two groups of patients with ARAT and CCF; and lastly, to propose a practical and inclusive anatomic-therapeutic classification for CACCs. The two main cornerstones of the proposed classification are the type of the connector between the aorta and cardiac chamber (hole versus passage) and the nature of the connecting passage ( anatomic versus extra-anatomic). We classified CACCs into three types. Depending on the intramural versus extramural course of the extra-anatomic connecting passage, type 3 is further subdivided into type 3A and type 3B.

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.

Update on shunt closure in neonates and infants.

Introduction: Cardiac defects that result in shunting are the most common types of congenital heart anomalies. Although these lesions can be simple, they can cause significant hemodynamic changes and can be challenging to manage in neonates and infants. Over the recent decades, the development of new transcatheter techniques and devices has made it safe and feasible to manage such defects when indicated, even in the smallest of patients. Understanding these interventional procedures is essential in order to manage those patients.Areas covered: In this article, we review the techniques and experience for closure of atrial septal defects, ventricular septal defects, patent ductus aarteriosus,as well as coronary arteriovenous malformations and fistulas in neonates and infants. Literature review of PubMed articles was performed through January 2021, with focus on the latest data and results of the usage of interventional techniques in treating these lesions specifically in this age-group.Expert opinion: Significant shunting lesions can be particularly challenging to manage in neonates and infants. Newer lower profile devices will likely continue to be developed in the future, allowing their use for transcatheter interventions in even smaller patients and those with more complex anatomy.

Hot topics in interventional cardiology: Proceedings from the society for cardiovascular angiography and interventions 2020 think tank.

The society for cardiovascular angiography and interventions (SCAI) think tank is a collaborative venture that brings together interventional cardiologists, administrative partners, and select members of the cardiovascular industry community for high-level field-wide discussions. The 2020 think tank was organized into four parallel sessions reflective of the field of interventional cardiology: (a) coronary intervention, (b) endovascular medicine, (c) structural heart disease, and (d) congenital heart disease (CHD). Each session was moderated by a senior content expert and co-moderated by a member of SCAI's emerging leader mentorship program. This document presents the proceedings to the wider cardiovascular community in order to enhance participation in this discussion, create additional dialogue from a broader base, and thereby aid SCAI and the industry community in developing specific action items to move these areas forward.

Aortopulmonary Collaterals in Single Ventricle Physiology: Variation in Understanding Occlusion Practice Among Interventional Cardiologists.

Although aortopulmonary collaterals (APCs) frequently develop in patients with single ventricle palliation, there is a lack of understanding of pathophysiology, natural history, and outcomes with no universal guidelines for management and interventional practice. We conducted a study to assess the views held by interventional congenital cardiologists regarding the hemodynamic impact of APCs in patients with single ventricle palliation, and their embolization practice. An electronic survey using the Pediatric Interventional Cardiology Symposium (PICS) mailing list was conducted between February and March 2019 with one reminder sent 2 weeks after initial invitation for participation. Of the 142 interventional cardiologist respondents, 95 (66.9%) reside in North America and 47 (33.1%) worldwide. We elected to exclude the data from interventionalists outside North America in this analysis as it was not representative of worldwide practice. Hypoxemia was considered to be the most common trigger for development of APCs by 56 (58.9%) respondents. After completion of total cavopulmonary connection, 30 (31.6%) respondents reported the APC burden stays the same while 31 (32.6%) feel it decreases. In evaluating the burden of APC flow, only 4 (4.2%) reported measuring oxygen saturation at different pulmonary artery segments, 21 (22.1%) perform segmental aortic angiograms, and 18 (19%) perform selective bilateral subclavian artery angiograms. A majority of respondents, 71 (74.7%), occlude the feeder vessel at different locations, while 10 (10.5%) occlude only the origin of the vessel. Our study demonstrates significant variation in the understanding of the cause and prognosis of APCs in patients with single ventricle palliation. Furthermore, there is variation in the approach for diagnosis and management among interventional cardiologists. Further studies are required to improve understanding of APCs and develop universal management guidelines.

Transcatheter Interventions in Adult Congenital Heart Disease.

This article provides a detailed review of the current practices and future directions of transcatheter interventions in adults with congenital heart disease. This includes indications for intervention, risks, and potential complications, as well as a review of available devices and their performance.

Current practice in atrial septal defect occlusion in children and adults.

INTRODUCTION: Atrial septal defect (ASD) is one of the most common congenital heart diseases (CHD) in children and adults. This group of malformations includes several types of atrial communications allowing shunting of blood between the systemic and the pulmonary circulations. Early diagnosis and treatment carries favorable outcomes. Patients diagnosed during adulthood often present with complications related to longstanding volume overload such as pulmonary artery hypertension (PAH), atrial dysrhythmias, and right (RV) and left (LV) ventricular dysfunction. AREA COVERED: This article intended to review all aspects of ASD; anatomy, pathophysiology, clinical presentation, natural history, and indication for treatment. Also, we covered the transcatheter therapy in detail, including the procedural aspect, available devices, and outcomes. EXPERT OPINION: There has been a remarkable improvement in the treatment strategy of ASD over the last few decades. Transcatheter closure of ASD is currently accepted as the treatment of choice in most patients with appropriately selected secundum ASDs. This review will focus on the most recent advances in diagnosis and treatment strategy of secundum ASD.