Update on Transcatheter Interventions in Adults with Congenital Heart Disease.

The field of adult congenital interventions is unique in the world of cardiac catheterization, combining the structural concepts commonly employed in pediatric heart disease and applying them to adult patients, who are more amenable to device intervention as they no longer experience somatic growth. Rapid advances in the field have been made to match the growing population of adult patients with congenital heart disease, which currently surpasses the number of pediatric patients born with congenital heart disease. Many congenital defects, which once required surgical intervention or reoperation, can now be addressed via the transcatheter approach, minimizing the morbidity and mortality often encountered within adult congenital surgeries. In this paper, we aim to provide a focused review of the more common procedures that are utilized for the treatment of adult congenital heart disease patients in the catheterization laboratory, as examples of current practices in the United States, as well as emerging concepts and devices awaiting approval in the future.

Transcatheter Structural Heart Interventions in the Acute Setting: An Emerging Indication.

Structural heart disease is increasingly prevalent in the general population, especially in patients of increased age. Recent advances in transcatheter structural heart interventions have gained a significant following and are now considered a mainstay option for managing stable valvular disease. However, the concept of transcatheter interventions has also been tested in acute settings by several investigators, especially in cases where valvular disease comes as a result of acute ischemia or in the context of acute decompensated heart failure. Tested interventions include both the mitral and aortic valve, mostly evaluating mitral transcatheter edge-to-edge repair and transcatheter aortic valve implantation, respectively. This review is going to focus on the use of acute structural heart interventions in the emergent setting, and it will delineate the available data and provide a meaningful discussion on the optimal patient phenotype and future directions of the field.

Transcatheter-Based Interventions for Tetralogy of Fallot Across All Age Groups.

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. Palliative procedures, either surgical or transcatheter, aim to improve oxygen saturation, affording definitive procedures at a later stage. Transcatheter interventions have been used before and after surgical palliative or definitive repair in children and adults. This review aims to provide an overview of the different catheter-based interventions for TOF across all age groups, with an emphasis on palliative interventions, such as patent arterial duct stenting, right ventricular outflow tract stenting, or balloon pulmonary valvuloplasty in infants and children and transcatheter pulmonary valve replacement in adults with repaired TOF, including the available options for a large, dilated native right ventricular outflow tract.

Transcatheter treatment of the tricuspid valve: current status and perspectives.

Transcatheter tricuspid valve interventions (TTVI) are emerging as alternatives to surgery in high-risk patients with isolated or concomitant tricuspid regurgitation. The development of new minimally invasive solutions potentially more adapted to this largely undertreated population of patients, has fuelled the interest for the tricuspid valve. Growing evidence and new concepts have contributed to revise obsolete and misleading perceptions around the right side of the heart. New definitions, classifications, and a better understanding of the disease pathophysiology and phenotypes, as well as their associated patient journeys have profoundly and durably changed the landscape of tricuspid disease. A number of registries and a recent randomized controlled pivotal trial provide preliminary guidance for decision-making. TTVI seem to be very safe and effective in selected patients, although clinical benefits beyond improved quality of life remain to be demonstrated. Even if more efforts are needed, increased disease awareness is gaining momentum in the community and supports the establishment of dedicated expert valve centres. This review is summarizing the achievements in the field and provides perspectives for a less invasive management of a no-more-forgotten disease.

The Lifesaving Impact of Transcatheter Interventions in the Early Post-Fontan Palliation Period.

Despite advancements in postoperative outcomes after Fontan surgery, there remains a risk of suboptimal outcomes and significant morbidity in the early postoperative period. Anatomical obstructions in the Fontan pathway can lead to prolonged pleural effusion or ascites, cyanosis, and low cardiac output syndrome (LCOS). Transcatheter interventions offer an alternative to early re-surgery for treating these complications. Over a 13-year period, early catheter angiography, performed within 30 days post-index procedure, was administered to 41 patients, identifying anatomical issues that necessitated re-intervention in 39 cases. This led to transcatheter interventions in 37 (10.4%) of the 344 Fontan surgery patients. The median age was 4.8 years (IQR: 4-9.4), and the median weight was 16.5 kg (IQR: 15-25.2), with females comprising 51.4% (19/37) of this group. The primary indications for the procedures were persistent pleural effusion or ascites in 27 patients (66%), LCOS in 8 patients (20%), and cyanosis in 6 patients (14%). Among the 37 undergoing transcatheter intervention, 30 were treated solely with this method and discharged, three died in ICU follow-up, and four required early re-surgery. No procedural mortality was observed. Our findings demonstrate that transcatheter interventions, including stent implantation, balloon angioplasty, and fenestration dilation, are safe and effective in the early post-Fontan period. Therefore, they should be considered an integral part of the management strategy for this patient group.

Atrial secondary tricuspid regurgitation: pathophysiology, definition, diagnosis, and treatment.

Atrial secondary tricuspid regurgitation (A-STR) is a distinct phenotype of secondary tricuspid regurgitation with predominant dilation of the right atrium and normal right and left ventricular function. Atrial secondary tricuspid regurgitation occurs most commonly in elderly women with atrial fibrillation and in heart failure with preserved ejection fraction in sinus rhythm. In A-STR, the main mechanism of leaflet malcoaptation is related to the presence of a significant dilation of the tricuspid annulus secondary to right atrial enlargement. In addition, there is an insufficient adaptive growth of tricuspid valve leaflets that become unable to cover the enlarged annular area. As opposed to the ventricular phenotype, in A-STR, the tricuspid valve leaflet tethering is typically trivial. The A-STR phenotype accounts for 10%-15% of clinically relevant tricuspid regurgitation and has better outcomes compared with the more prevalent ventricular phenotype. Recent data suggest that patients with A-STR may benefit from more aggressive rhythm control and timely valve interventions. However, little is mentioned in current guidelines on how to identify, evaluate, and manage these patients due to the lack of consistent evidence and variable definitions of this entity in recent investigations. This interdisciplinary expert opinion document focusing on A-STR is intended to help physicians understand this complex and rapidly evolving topic by reviewing its distinct pathophysiology, diagnosis, and multi-modality imaging characteristics. It first defines A-STR by proposing specific quantitative criteria for defining the atrial phenotype and for discriminating it from the ventricular phenotype, in order to facilitate standardization and consistency in research.

Systematic approach to obtain axillary arterial access for pediatric heart catheterizations.

Background: Axillary arterial access (AAA) in pediatric heart catheterizations is undervalued. Methods: We retrospectively reviewed children with congenital heart diseases (CHDs) who received trans-axillary arterial catheterizations between January 2019 and February 2023. We aimed ultrasound-guided punctures in the proximal two-thirds of axillary arteries with diameters ≥2 mm to insert 7 cm/4 Fr short introducers. We administrated intra-arterial verapamil (1.25 mg) and heparin (100 UI/kg). We infiltrated per-operatively 2% lignocaine (10 mg) for arterial spasms, long sheaths use (≥5 Fr), and ≥60 min procedures in <3 kg patients. Results: We identified 30 patients (66.7% males) with a median age of 1.1 months (IQR, 0.3-5.4), and a median weight of 3.1 kg (IQR, 2.7-3.7). 5/30 patients had six redo interventions after a median of 3.9 months (IQR, 1.7-5.1). Overall, 27/36 procedures were interventional, including 6 aortic valvuloplasties, 6 balloon angioplasties, and 15 stenting procedures. The median arterial axillary angiographic diameter was 2.6 mm (IQR, 2.4-3). Access was right-sided in 23/36 (63.9%) procedures and obtained using 21G/2.5 cm bevel needles in 25/36 (69.4%) procedures. No hemodynamical change occurred after introducing spasmolytic drugs. The median fluoroscopy time was 26.1 min (IQR, 19.2-34.8). There were two self-resolving arterial dissections, one sub-occlusive arterial thrombosis (resolved with 6 weeks of enoxaparin), and one occlusive arterial thrombosis (resolved with alteplase thrombolysis and 6 weeks of enoxaparin). Median follow-up was 11.7 months (IQR, 8-17.5). Four patients with complex univentricular hearts died from non-procedural causes at a median of 40 days (IQR, 31-161) postoperative. Conclusion: Systematic approach for AAA is the key to success and unlocks the many potentials of trans-axillary pediatric cardiology interventions.

Right Heart Reverse Remodeling and Prosthetic Valve Function After Transcatheter vs Surgical Pulmonary Valve Replacement.

BACKGROUND: There are limited data about postprocedural right heart reverse remodeling and long-term prosthesis durability after transcatheter pulmonary valve replacement (TPVR) and how these compare to surgical pulmonary valve replacement (SPVR). OBJECTIVES: This study sought to compare right heart reverse remodeling, pulmonary valve gradients, and prosthetic valve dysfunction after TPVR vs SPVR. METHODS: Patients with TPVR were matched 1:2 to patients with SPVR based on age, sex, body surface area, congenital heart lesion, and procedure year. Right heart indexes (right atrial [RA] reservoir strain, RA volume index, RA pressure, right ventricular [RV] global longitudinal strain, RV end-diastolic area, and RV systolic pressure) were assessed at baseline (preintervention), 1 year postintervention, and 3 years postintervention. Pulmonary valve gradients were assessed at 1, 3, 5, 7, and 9 years postintervention. RESULTS: There were 64 and 128 patients in the TPVR and SPVR groups, respectively. Among patients with TPVR, 46 (72%) and 18 (28%) received Melody (Medtronic) vs SAPIEN (Edwards Lifesciences) valves, respectively. The TPVR group had greater postprocedural improvement in RA reservoir strain and RV global longitudinal strain at 1 and 3 years. The TPVR group had a higher risk of prosthetic valve dysfunction mostly because of a higher incidence of prosthetic valve endocarditis compared to SPVR but a similar risk of pulmonary valve reintervention because some of the patients with endocarditis received medical therapy only. Both groups had similar pulmonary valve mean gradients at 9 years postintervention. CONCLUSIONS: These data suggest a more favorable right heart outcome after TPVR. However, the risk of prosthetic valve endocarditis and prosthetic valve dysfunction remains a major concern.

Word of caution: silent late device embolisation after perimembranous ventricular septal defect closure in a 6-Kg infant.

We report on a 6-month-old infant (6 Kg/ 64 cm) with perimembranous ventricular septal defect (absent sub-aortic rim, 10 mm left ventricular entry, and 4 and 6 mm right ventricular exists) and successful retrograde closure using an 8x6 mm KONAR-MF™ VSD occluder (Lifetech, China). Immediate and 48 hours post-procedure ultrasounds showed an accurately positioned device and two jets of mild-to-moderate residual shunts. At the 2-week follow-up, the device did not change position and the shunt was stable and intra-prosthetic. The scheduled 3-month follow-up was skipped for familial reasons. The patient came back without alarming symptoms for the regular 6-month follow-up, and the device was found embolised to the left pulmonary artery. The device was retrieved surgically, and the defect was patch-closed with excellent outcomes. There was a pseudoaneurysm involving the tricuspid valve chordae and the device was endothelialized partially on one edge suggesting that embolization occurred somewhere between 3 months and 6 months post-operative. Defects with compromised anatomies should be closed surgically to avoid suboptimal results, especially in small infants.

SwiftNINJA steerable microcatheter: a new kid on the block for selective catheterization of vascular and valvular congenital lesions.

Background: SwiftNINJA (Merit Medical Systems, USA) is a novel steerable microcatheter intended for coronary and peripheral vascular interventions. We evaluate and report the first use of SwiftNINJA in pediatric catheterization of congenital heart defects (CHDs). Methods: We performed a retrospective clinical data review of children with CHDs in whom SwiftNINJA was used during cardiac catheterization between April 2022 and June 2023. Utility, application, and standard safety were described comprehensively. Results: We identified 19 patients (78.9% males) with a median age of 5.3 months (IQR, 2-13.9), and a median weight of 5.3 kg (IQR, 4-7.7). 36.8% of the catheterizations were transarterial and 78.9% were interventional. SwiftNINJA was applied upfront in 3/19 patients to cannulate precisely the right pulmonary artery and eliminate the risk of dislocating a freshly implanted left pulmonary flow restrictor. In 16/19 patients, SwiftNINJA was applied after a median of 5 (IQR, 5-7) failed catheterization attempts using various combinations of catheters, microcatheters, and wires to cannulate challenging vasculature in seven, engage the lumen of stented vessels in five, cross complex aortic valve stenosis in three, and cross an apical ventricular septal defect in one patient with Damus-Kaye-Stansel repair. After the SwiftNINJA application, catheterization was done from the first attempt in 12/16 patients and from the second attempt in 4/16 patients. The median applied tip angulation was 90 degrees (IQR, 85-95). All procedures were completed successfully. No device malfunction or adverse events occurred. Conclusions: SwiftNINJA is a valuable addition for selective catheterization of challenging vasculatures or valvular anatomies in children with CHDs.

Novel Approaches to the Failing Congenital Heart.

PURPOSE OF REVIEW: Heart failure is the leading cause of morbidity and mortality in adults with congenital heart disease and is characterized by a variety of underlying mechanisms. Here, we aim to elaborate on the medical and technological advancements in the management of heart failure in adult patients with congenital heart disease and highlight the use of imaging modalities to guide therapy. RECENT FINDINGS: There have been several advances over the past decade with angiotensin receptor neprilysin and sodium-glucose cotransporter-2 inhibitors, atrioventricular valve clips, transcatheter pulmonary valves, catheter ablation, and cardiac resynchronization therapy, as well as the introduction of lymphatic interventions. Expanded use of echocardiography, cardiac magnetic resonance imaging, and cardiac computed tomography has guided many of these therapies. Significant innovations in the management of heart failure in adults with congenital heart disease have evolved with advancements in imaging modalities playing a critical role in guiding treatment therapies.

Short-Term Effects of Different Transcatheter Edge-to-Edge Devices on Mitral Valve Geometry.

Background Short-term effects on mitral valve (MV) anatomy after transcatheter edge-to-edge repair using the PASCAL system remain unknown. Precise quantification might allow for an advanced analysis of predictors for mean transmitral gradients. Methods and Results Consecutive patients undergoing transcatheter edge-to-edge repair for secondary mitral regurgitation using PASCAL or MitraClip systems were included. Quantification of short-term MV changes throughout the cardiac cycle was performed using peri-interventional 3-dimensional MV images. Predictors for mean transmitral gradients were identified in univariable and multivariable regression analysis. Long-term results were described during 1-year follow-up. A total of 100 patients undergoing transcatheter edge-to-edge repair using PASCAL (n=50) or MitraClip systems (n=50) were included. Significant reductions of anterior-posterior diameter, annular circumference, and area throughout the cardiac cycle were found in both cohorts (P<0.05 for all). Anatomic MV orifice area remained larger in the PASCAL cohort in mid (2.8±1.0 versus 2.4±0.9 cm2; P=0.049) and late diastole (2.7±1.1 versus 2.2±0.8 cm2; P=0.036) compared with the MitraClip cohort. Besides a device-specific profile of independent predictor of mean transmitral gradients, reduction of middiastolic anatomic MV orifice area was identified as an independent predictor in both the PASCAL (ß=-0.410; P=0.001) and MitraClip cohorts (ß=-0.318; P=0.028). At follow-up, reduction of mitral regurgitation grade to mild or less was more durable in the PASCAL cohort (90% versus 72%; P=0.035). Conclusions PASCAL and MitraClip showed comparable short-term effects on MV geometry. However, PASCAL might better preserve MV function and demonstrated more durable mitral regurgitation reduction during follow-up. Identification of independent predictors for mean transmitral gradients might potentially help to guide device selection in the future.

Novel Therapeutic Strategies in Heart Failure in Adult Congenital Heart Disease: of Medicines and Devices.

PURPOSE OF REVIEW: This paper reviews the latest literature on the growing field of heart failure in the adult congenital heart disease population. RECENT FINDINGS: After highlighting the increasing prevalence and a few of the unique potential causes, including the concept of early senescence, this review begins with novel medical management strategies such as the angiotensin II receptor blocker and neprilysin inhibitors and sodium glucose cotransporter-2 inhibitors. Then, it addresses the latest applications of percutaneous techniques like implantable hemodynamic monitoring, transcatheter pulmonary and aortic valve replacement, and mitral clips. Cardiac resynchronization therapy and novel lymphatic system imaging and intervention are then described. Finally, the use of mechanical support devices, temporary and durable, is discussed as well as heart and combined heart and liver transplantation. There have been recent exciting advances in the strategies used to manage adult congenital heart disease patients with heart failure. As this population continues to grow, it is likely we will see further rapid evolution in this field.

Transcatheter Interventions for Neonates with Congenital Heart Disease: A Review.

Newborns with congenital heart disease often require interventions linked to high morbidity and mortality rates. In the last few decades, many transcatheter interventions have become the first-line treatments for some critical conditions in the neonatal period. A catheter-based approach provides several advantages in terms of procedural time, length of hospitalization, repeatability and neurodevelopmental issues (usually related to cardiopulmonary bypass). The main transcatheter procedures will be reviewed, as they are now valid alternatives to conventional surgical management.

Transcatheter Management of Pulmonary Sequestrations in Children-A Single-Center Experience.

BACKGROUND: A pulmonary sequestration (PS) is an area of bronchopulmonary tissue with aberrant arterial supply. Transcatheter occlusion of PSs is an appealing treatment option, but data on outcomes remain scarce. We aim to describe our experience with transcatheter management of PS in infants and children. METHODS: Retrospective review of clinical data of all patients with suspected PS sent for diagnostic and/or interventional cardiac catheterization at our institution between January 1999 and May 2021. Procedural considerations, techniques, standard safety, and outcomes were assessed. RESULTS: We identified 71 patients (52.1% males), with median age and weight of 4.9 months (IQR, 2.1-26.6) and 4.2 kg (IQR, 3.9-12.1), respectively. Sixty-one (86%) patients had associated congenital heart defects (CHDs). Forty-two (59%) patients had pulmonary arterial hypertension (PAH) at the time of diagnosis. Fifty-three (74.7%) patients underwent embolization of the PS feeding vessel using microcoils and/or vascular plugs, and eight (15.1%) of these were neonates who presented with severe PAH and cardiac failure. Two patients had large feeding vessels and were treated surgically. Sixteen (22.5%) patients with small feeding vessels received conservative management. At median follow-up of 36.4 months (IQR, 2.1-89.9), seven patients had died, 24 patients had CHD corrective surgeries, 26 patients had redo catheterizations, and five patients had persistent PAH. No PS surgical resection was needed, and no infection of the remaining lung tissue occurred. CONCLUSIONS: Transcatheter assessment and treatment of PSs is a safe and effective procedure. Neonates with large PSs are severely symptomatic and improve remarkably after PS closure. PS embolization and surgical repair of associated CHDs generally leads to the normalization of pulmonary pressures.

FAST technique: fast atrial sheath traction technique for device closure of atrial septal defects.

Background: Transcatheter closure of atrial septal defects (ASDs) is well-established. However, this procedure can be challenging, requiring multiple attempts and advanced implantation maneuvers. Materials and methods: From July 2019 to July 2022, patients to whom the fast atrial sheath traction (FAST) technique was applied for ASD device closure were prospectively followed up. The device was rapidly unsheathed in the middle of the left atrium (LA) to let it clamp the ASD from both sides simultaneously. This novel technique was directly applied in patients with absent aortic rims and/or ASD size-to-body weight ratio higher than 0.9 or after failed attempts of standard implantation. Results: Seventeen patients (64.7% males) were involved with a median age of 9.8 years [interquartile range (IQR), 7.6-15.1] and a median weight of 34 kg (IQR, 22-44). The median ASD size on ultrasound was 19 mm (IQR, 16-22). Five (29.4%) patients had absent aortic rims, and three (17.6%) patients had an ASD size-to-body weight ratio higher than 0.9. The median device size was 22 mm (IQR, 17-24). The median difference between device size and ASD two-dimensional static diameter was 3 mm (IQR, 1-3). All interventions were straightforward without any complications using three different occluder devices. One device was removed before release and upsized to the next size. The median fluoroscopy time was 4.1 min (IQR, 3.6-4.6). All patients were discharged the next postoperative day. On a median follow-up of 13 months (IQR, 8-13), no complications were detected. All patients achieved full clinical recovery with complete shunt closure. Conclusion: We present a new implantation technique to efficiently close simple and complex ASDs. The FAST technique can be of benefit in overcoming left disc malalignment to the septum in defects with absent aortic rims and in avoiding complex implantation maneuvers and the risks of injuring the pulmonary veins.

Structural Valve Deterioration in Transcatheter Aortic Bioprostheses: Diagnosis, Pathogenesis, and Treatment.

Transcatheter aortic valve replacement has emerged as the preferred treatment modality in most patients with severe aortic stenosis. With its global adoption and broader application in younger and healthier patients, the issue of transcatheter bioprosthetic valve degeneration and its impact on valve durability continues to earn clinical relevance. Differences in the pathophysiologic processes that separate native from transcatheter heart valve deterioration remain poorly understood. When compared to surgical aortic bioprostheses, the mechanisms of valve degeneration are similar in transcatheter heart valves, with meaningful differences most noticeably found between the individual constructs of their design. Recognizing the clinical and hemodynamic presentation of structural valve degeneration remains paramount. The recently revised consensus guidelines that incorporate the integration of advanced multimodality imaging with invasive hemodynamics represent a major step forward in our ability to accurately diagnose bioprosthetic valve degeneration, and to identify differences in durability patterns, and to establish treatment recommendations for the lifetime management of patients with aortic stenosis. Parallel efforts to unmask the biomolecular differences in atherosclerotic plaque burden, valve calcification, and thrombotic diathesis, including host immunocompetence, between the different available bioprostheses, will further advance the role of emerging valve tissue technologies to improve durability. As with surgical heart valves, the optimal treatment options for redo-transcatheter aortic valve replacement and surgical explant remain poorly understood. Ongoing translational research in bench testing coupled with prospectively designed core lab-adjudicated clinical trials are much needed. This report provides a contemporary overview of transcatheter structural valve degeneration, including evolving concepts in its pathogenesis, diagnosis, and treatment.

The clinical role of invasive hemodynamics in the evaluation and treatment of structural heart disease.

INTRODUCTION: Recognition and evaluation of structural heart disease has become more common in clinical practice and will continue to grow as the population ages. With the growing availability of surgical and transcatheter interventional options, appropriate evaluation and patient selection for therapy is required. While echocardiography can frequently provide the required anatomic and hemodynamic information required to guide therapeutic decisions, there remains subsets of patients in which noninvasive testing yields inconclusive results prompting the need for invasive hemodynamics. AREAS COVERED: This article reviews the indications and strengths of invasive hemodynamics in a variety of structural heart diseases. We describe the utilization and benefits of continuous hemodynamics during transcatheter interventions and review the prognostic information that can be gleaned from changes in hemodynamics after intervention. EXPERT OPINION: The advancement of transcatheter therapies for structural heart disease has sparked a renewed interest in the utilization of invasive hemodynamics. Continued growth and accessibility of comprehensive hemodynamics for clinical practice will rely on clinicians to continually review, refine, and develop procedural techniques beyond the current training standards in order to further advance the field.

Catheter-based Interventions to Reduce or Modify Surgical Risk in High-Risk Adult Congenital Heart Disease Patients.

The field of adult congenital heart disease has changed greatly over the past sixty years. As patients are now surviving longer into adulthood due to various improvements in surgical technique and medical technology, the demographic of patients with congenital heart disease (CHD) has changed, such that there are now more adults with CHD than there are children with CHD. This older and more medically complex population needs more interventions to treat residual defects or sequelae of their initial surgeries, and many of these patients are now deemed high risk for surgery. When the surgical risk becomes too great, either due to patient complexity, surgical complexity, or both, then transcatheter procedures may have a role in either mitigating or avoiding the risk altogether.