Transcatheter pulmonary valve replacement after arterial switch operation.

BACKGROUND: Patients with d-transposition of the great arteries (d-TGA) who have undergone an arterial switch operation (ASO) can develop right ventricular outflow tract (RVOT) dysfunction with pulmonary regurgitation (PR) or stenosis. In these patients, treatment may include transcatheter pulmonary valve replacement (TPVR). Coronary compression is a contraindication occurring in 5% of typical TPVR cases. After ASO, there are various anatomical considerations that can confound TPVR, including potential coronary artery compression. Our goal is to understand feasibility of TPVR in patients following ASO. METHODS: This was a retrospective multicenter cohort study of patients with RVOT dysfunction after ASO who underwent cardiac catheterization with intention to perform TPVR from 2008 to 2020. RESULTS: Across nine centers, 33 patients met inclusion criteria. TPVR was successful in 22 patients (66%), 19 receiving a Melody valve and 3 a SAPIEN valve. RVOT stenosis in isolation or with PR dictated need for TPVR in nearly all patients. One serious adverse event occurred with valve embolization. After TPVR, the RVOT peak gradient decreased from 43 to 9 mm Hg (p < 0.001); PR was trivial/none in all but one patient, in whom it was mild. Coronary compression prohibiting TPVR occurred in eight patients (24%) and two patients (6%) had severe aortic regurgitation from aortic root deformation precluding TPVR. Seven patients underwent RVOT reintervention with a median of 5.3 years post-TPVR. CONCLUSIONS: TPVR in patients with d-TGA after ASO is feasible, but in this cohort, coronary compression or aortic root distortion precluded TPVR in one-third of patients. The rate of RVOT reintervention after TPVR was higher in this cohort of ASO patients that in prior studies.

Augmented Reality Visualization of 3D Rotational Angiography in Congenital Heart Disease: A Comparative Study to Standard Computer Visualization.

Augmented reality (AR) visualization of 3D rotational angiography (3DRA) provides 3D representations of cardiac structures with full visualization of the procedural environment. The purpose of this study was to evaluate the feasibility of converting 3DRAs of congenital heart disease patients to AR models, highlight the workflow for 3DRA optimization for AR visualization, and assess physicians' perceptions of their use. This single-center study prospectively evaluated 30 retrospectively-acquired 3DRAs that were converted to AR, compared to Computer Models (CM). Median patient age 6.5 years (0.24-38.8) and weight 20.6 kg (3.4-107.0). AR and CM quality were graded highly. RV pacing was associated with higher quality of both model types (p = 0.02). Visualization and identification of structures were graded as "very easy" in 81.1% (n = 73) and 67.8% (n = 61) of AR and CM, respectively. Fifty-nine (66%) grades 'Agreed' or 'Strongly Agreed' that AR models provided superior appreciation of 3D relationships; AR was found to be least beneficial in visualization of aortic arch obstruction. AR models were thought to be helpful in identifying pathology and assisting in interventional planning in 85 assessments (94.4%). There was significant potential seen in the opportunity for patient/family counseling and trainee/staff education with AR models. It is feasible to convert 3D models of 3DRAs into AR models, which are of similar image quality as compared to CM. AR models provided additional benefits to visualization of 3D relationships in most anatomies. Future directions include integration of interventional simulation, peri-procedural counseling of patients and families, and education of trainees and staff with AR models.

Optimizing 3D Rotational Angiography for Congenital Cardiac Catheterization.

The aim of the study was to determine the variables associated with high-quality (HQ) versus low-quality (LQ) three-dimensional rotational angiography (3DRA) and create guides for optimization of approach to 3DRA in congenital cardiac catheterization (CCC). CCC has adopted 3DRA as a mainstay, but there has not been systematic analysis of approach to and factors associated with HQ 3DRA. This was a single-center, retrospective study of 3DRAs using Canon Infinix-I platform. Reconstructions were graded by 3 interventionalists. Quality was dichotomized into HQ and LQ. Univariable analyses and multivariable logistic regression models were performed. From 8/2016 to 12/2018, 208 3DRAs were performed in 195 CCCs; median age 7 years (2, 16), weight 23 kg (12, 57). The majority of 3DRAs were performed in patients with biventricular physiology (N = 137, 66%) and in pulsatile sites (N = 144, 69%). HQ 3DRA (N = 182, 88%) was associated with greater total injection volume [2.20 mL/kg (1.44, 3.29) vs. 1.62 mL/kg (1.10, 1.98), p = 0.005] and more dilute contrast solution [60% (50, 100) vs. 100% (60, 100), p = 0.007], but not with contrast volume administered (p = 0.2) on univariable analysis. On multivariable logistic regression, HQ 3DRA was significantly associated with patient weight [OR 0.97 (95% CI (0.94, 0.99), p = 0.018], total injection volume [OR 1.04 (95% CI 1.01, 1.07) p = 0.011], and percent contrast solution [OR 0.97 (95% CI 0.95, 1.00), p = 0.022]. These data resulted in creation of scatter plots and a novel 3DRA Nomogram for estimating the probability of HQ 3DRA. This is the first study to create evidence-based contrast dose guides and nomogram for 3DRA in CCC. HQ 3DRA was associated with lower weight, higher total injection volumes, and more dilute contrast solution.

Patch Materials for Pulmonary Artery Arterioplasty and Right Ventricular Outflow Tract Augmentation: A Review.

Patch augmentation of the right ventricular outflow tract (RVOT) and pulmonary artery (PA) arterioplasty are relatively common procedures in the surgical treatment of patients with congenital heart disease. To date, several patch materials have been applied with no agreed upon clinical standard. Each patch type has unique performance characteristics, cost, and availability. There are limited data describing the various advantages and disadvantages of different patch materials. We performed a review of studies describing the clinical performance of various RVOT and PA patch materials and found a limited but growing body of literature. Short-term clinical performance has been reported for a multitude of patch types, but comparisons are limited by inconsistent study design and scarce histologic data. Standard clinical criteria for assessment of patch efficacy and criteria for intervention need to be applied across patch types. The field is progressing with improvements in outcomes due to newer patch technologies focused on reducing antigenicity and promoting neotissue formation which may have the ability to grow, remodel, and repair.

Feasibility of intraprocedural augmented reality visualisation of 3D rotational angiography in congenital cardiac catheterisation.

Three-dimensional rotational angiography has become a mainstay of congenital cardiac catheterisation. Augmented reality is an exciting and emerging technology that allows for interactive visualisation of 3D holographic images in the user's environment. This case series reports on the feasibility of intraprocedural augmented reality visualisation of 3D rotational angiography in five patients with CHD.

First-in-paediatric uses of a mechanical aspiration system for percutaneous removal of right atrial masses.

We present the first-in-paediatric uses of a mechanical aspiration system for percutaneous removal of right atrial masses in three patients, including central line-related thrombus and metastatic tumour. Percutaneous mechanical removal of right atrial masses can be performed safely and effectively.

mRNA Coronavirus Disease 2019 Vaccine-Associated Myopericarditis in Adolescents: A Survey Study.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.

Novel use of a mechanical suction thrombectomy system for interposition graft occlusion in complex congenital heart disease.

Complete thrombosis of a pulmonary artery interposition graft in an adolescent with complex repaired CHD was treated successfully with a combination of a novel mechanical thrombectomy system, stent implantation, and thrombolysis. This thrombectomy system used a flexible catheter with a built-in mechanism to attenuate blood loss, while providing effective recanalisation of a foreign graft.

Ruptured sinus of Valsalva aneurysm: An uncommon presentation of shock to the pediatric emergency department.

This case report describes a rare etiology of cardiogenic shock, particularly in the pediatric population. A healthy 17 year old male presents from an outside hospital in undifferentiated shock requiring vasopressor support. Ruptured sinus of Valsalva aneurysm was diagnosed by echocardiogram and the patient went emergently to the operating room for surgical repair. We discuss the anatomy, incidence, and risk factors for sinus of Valsalva aneurysms, along with the range of clinical presentations and Emergency Department management of symptomatic rupture of sinus of Valsalva aneurysms.

Implantable pulmonary artery pressure monitoring device in patients with palliated congenital heart disease: Technical considerations and procedural outcomes.

OBJECTIVE: We describe the technical considerations of transcatheter implantation of the CardioMEMS™ HF System (Abbott, Abbott Park, IL) in adult patients with complex palliated congenital heart disease (CHD) and advanced heart failure (HF). BACKGROUND: Ambulatory pulmonary artery (PA) pressure monitoring with implantable hemodynamic monitors (IHMs) has been shown to reduce HF-related hospital admissions in non-CHD populations. HF is a common late cardiovascular complication in adult CHD necessitating better understanding of IHM application in this population. METHODS: We analyzed adults with complex CHD and advanced HF who were referred for CardioMEMS™ device implantation (2015-2018). Feasibility of device implantation, defined by successful device implantation and calibration, and procedural outcomes were evaluated. RESULTS: CardioMEMS™ was successfully implanted in all 14 adults (35.5 ± 9.2 years old, 72 ± 12 kg) with complex CHD (single ventricle/Fontan, n = 8 [57%]; d-transposition of the great arteries/atrial switch, n = 6 [43%]). The device was delivered via femoral venous access in 13 (93%) patients and implanted in the left PA in 12 (86%). A long sheath was used in 8 (57%) patients, including 5/6 with an atrial switch operation. There was one device migration that did not require retrieval. CONCLUSIONS: Transcatheter implantation of an IHM is feasible in select complex adult CHD patients with advanced HF. Further studies evaluating integration of ambulatory hemodynamics and the impact on clinical care are needed. This technology has the potential to improve medical management of advanced HF in patients with Fontan and atrial switch physiologies and provide new insights into their ambulatory hemodynamics.

Personalized Interventions: A Reality in the Next 20 Years or Pie in the Sky.

There is no better representation of the need for personalization of care than the breadth and complexity of congenital heart disease. Advanced imaging modalities are now standard of care in the field, and the advancements being made to three-dimensional visualization technologies are growing as a means of pre-procedural preparation. Incorporating emerging modeling approaches, such as computational fluid dynamics, will push the limits of our ability to predict outcomes, and this information may be both obtained and utilized during a single procedure in the future. Artificial intelligence and customized devices may soon surface as realistic tools for the care of patients with congenital heart disease, as they are showing growing evidence of feasibility within other fields. This review illustrates the great strides that have been made and the persistent challenges that exist within the field of congenital interventional cardiology, a field which must continue to innovate and push the limits to achieve personalization of the interventions it provides.

Use of the Gore Viabahn VBX balloon-expandable endoprosthesis in the congenital heart disease population.

The Gore Viabahn VBX balloon-expandable endoprosthesis (W. L. Gore & Associates, Flagstaff, AZ) is a flexible covered stent that is FDA-approved for the treatment of iliac artery stenosis, including lesions at the aortic bifurcation. In this case series, we report the first use of the VBX covered stent in congenital heart disease, highlighting several of its unique advantages.

Tie a yellow ribbon around a papillary muscle.

An 8-year-old boy with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals status post complete repair including a fenestrated ventricular septal defect patch presented to the catheterization laboratory for fenestration closure. During the procedure, the catheterization wire was found to have an unusual intracardiac loop and was unable to be straightened within the heart. Three-dimensional transesophageal echocardiography revealed the wire was looped around a right ventricular papillary muscle. The wire was readjusted, and the fenestration was successfully closed. Three-dimensional imaging was essential in unambiguously defining the catheter course and assisting in fenestration closure.

Transcatheter stenting of superior vena cava obstruction after pediatric heart transplantation: A single-center experience assessing risk factors and outcomes.

Transcatheter stent implantation for SVC obstruction following OHT has been well described, particularly in pediatric patients. This study describes a large single-center pediatric heart transplant experience that investigates the risk factors for SVC stenosis requiring stent implantation and its associated outcomes. All pediatric OHTs between January 1, 2000, and December 12, 2016, were examined for risk factors. Twelve of 349 (3.4%) OHTs required SVC stent implantation. Patients who required stents were younger (2.5 years vs 10.0 years, P = 0.0097), weighed less (8.6 kg vs 26.0 kg, P = 0.0024), and were more likely to have had CHD (83% vs 32%, P = 0.001) or previous SCPA (58% vs 18%, P = 0.002). Bicaval anastomosis was not associated with subsequent SVC stent implantation. Symptoms included SVC syndrome and chylous effusions. All 12 patients had evidence of significant SVC obstruction by both echocardiographic Doppler interrogation and transcatheter angiography. There were no acute procedural complications; however, reinterventions were common (four of 12 patients) and occurred at a mean of 5.7 ± 3.6 months poststent implantation. In conclusion, transcatheter SVC stent implantation is safe and effective after OHT. There were significant associations between SVC stent implantation and younger age, smaller weight, CHD, and history of SCPA, but not with bicaval anastomosis or donor-recipient weight ratio.

Transcatheter pulmonary valve implantation: valve technology and procedural outcome.

PURPOSE OF REVIEW: Procedural technique and short-term outcomes of transcatheter pulmonary valve implantation (TPVI) have been widely described. The purpose of this article is to provide an update on current valve technology, and to focus on recent data surrounding TPVI in the dilated right ventricular outflow tract (RVOT), hybrid interventions, significant outcomes, and procedural costs. RECENT FINDINGS: Transcatheter valve technology has expanded with current trials evaluating self-expandable valves that can be implanted in dilated RVOTs. Until those valves are widely available, hybrid techniques have been shown to offer a potential alternative in these patients, as well as in patients of small size. Although medium-term results of TPVI have shown 5-year freedom from reintervention or replacement of 76%, new data have underlined some concerns relating to bacterial endocarditis after the procedure. Procedural costs remain a concern, but vary greatly between institutions and healthcare systems. SUMMARY: TPVI has emerged as one of the most innovative procedures in the treatment of patients with dysfunctional RVOT and pulmonary valves. Further device development is likely to expand the procedure to patients of smaller size and with complex, dilated RVOTs.

Wolff-Parkinson-White syndrome: a single exercise stress test might be misleading.

Risk stratification of patients with Wolff-Parkinson-White syndrome for sudden death is a complex process, particularly in understanding the utility of the repeat exercise stress test. We report a case of an 18-year-old patient who was found to have a high-risk pathway by both invasive and exercise stress testing after an initial exercise stress test showing beat-to-beat loss of pre-excitation.

Hybrid Interventions in Congenital Heart Disease.

Hybrid interventions in congenital heart disease (CHD) embody the inherent collaboration between congenital interventional cardiology and cardiothoracic surgery. Hybrid approaches to complex and common lesions provide the opportunity to circumvent the limitations of patient size, vascular access, severity of illness, and anatomy that would otherwise be prohibitive to surgical and percutaneous techniques alone. This review describes several important hybrid approaches to interventions in CHD.

Invasive Implanted Hemodynamic Monitoring in Patients With Complex Congenital Heart Disease: State-of-the-Art Review.

As the number of patients with congenital heart disease (CHD) continues to increase, the burden of heart failure (HF) in this population requires innovative strategies to individualize management. Given the success of implanted invasive hemodynamic monitoring (IHM) with the CardioMEMSTM HF system in adults with acquired HF, this is often suggested for use in patients with CHD, though published data are limited to case reports and case series. Therefore, this review summarizes the available published reports on the use of IHM in patients with complex CHD, describes novel applications, and highlights future directions for study. In patients with CHD, IHM has been used across the lifespan, from age 3 years to adulthood, with minimal device-related complications reported. IHM uses include (1) prevention of HF hospitalizations; (2) reassessment of hemodynamics after titration of medical therapy without repeated cardiac catheterization; (3) serial monitoring of at-risk patients for pulmonary hypertension to optimize timing of heart transplant referral; (4) and hemodynamic assessment with exercise (5) or after ventricular assist device placement. IHM has the potential to reduce the number of cardiac catheterizations in anatomically complex patients and, in patients with Fontan circulation, IHM pressures may have prognostic implications. In conclusion, though further studies are needed, as patients with CHD age and HF is more prevalent, this tool may assist CHD physicians in caring for this complex patient population.

Novel uses for implanted haemodynamic monitoring in adults with subaortic right ventricles.

BACKGROUND: Pulmonary hypertension (PH) is a common complication in patients with complete dextro-transposition of the great arteries (TGA) after atrial switch (D-TGA/AS) and congenitally corrected TGA (ccTGA). In this population with subaortic right ventricles (sRVs), echocardiography is a poor screening tool for PH; implantable invasive haemodynamic monitoring (IHM) could be used for this purpose, but data are limited. The aim of this study is to report on novel uses of IHM in patients with sRV. METHODS: This retrospective study describes the uses of IHM, impact of IHM on heart failure hospitalisation (HFH) and device-related complications in adults with sRV from a single centre (2015-2022). RESULTS: IHM was placed in 18 patients with sRV (median age 43 (range 30-54) years, 8 female, 16 with D-TGA/AS, 2 with ccTGA); 16 had moderate or severe sRV systolic dysfunction, 13 had PH on catheterisation. IHM was used for (1) Medical therapy titration, (2) Medical management after ventricular assist device in patients with transplant-limiting PH and (3) Serial monitoring of pulmonary artery pressures without repeat catheterisations to help identify the optimal time for heart transplant referral. In follow-up (median 23 months), HFHs/year were similar to the year prior to IHM (median 0 (IQR 0-1.0) before vs 0 (0-0.8) after, p=0.984). Device migration occurred in one, without long-term sequelae. CONCLUSIONS: Uses of IHM in patients with sRV are described which may minimise the need for serial catheterisations in a population where PH is prevalent. HFHs were low overall but not impacted by IHM. One device-related complication occurred without long-term consequence.

Percutaneous Balloon-Expandable Stent Implantation to Treat Transverse Aortic Arch Obstruction: Medium- to Long-Term Outcomes of a Retrospective Multicenter Study.

BACKGROUND: Transverse aortic arch obstruction is a challenging lesion for which stent implantation provides a potentially important alternate therapy. The objectives were to evaluate the technical, procedural, and medium-to-long-term clinical outcomes of percutaneous stent implantation of transverse aortic arch obstruction. METHODS: This is a retrospective, multicenter study of transverse aortic arch stent implantation. Univariable and multivariable analyses were performed. RESULTS: Index catheterization included 187 stent implants in 146 patients. The median age is 14.3 years (interquartile range, 9.3-19), weight is 53 kg (30-69), and follow-up is 53 months (12-120). The most common stent design was open cell (n=90, 48%). Stents overlapped 142 arch vessels (37 carotid arteries) in 118 (81%) cases. Technical and procedural success rates were 100% and 88%, respectively. Lower weight (P=0.018), body surface area (P=0.013), and minimum-to-descending aortic diameter ratio (P<0.001) were associated with higher baseline aortic gradient. The residual gradient was inversely associated with implant and final dilation diameters (P<0.001). The combined incidence of aortic injury and stent-related complications was 14%. There were no reports of abnormal brain scans or stroke. Blood pressure cuff gradient, echocardiographic arch velocity, and hypertension rates improved within 1-year follow-up with increased antihypertensive medication use. Reintervention was reported in 60 (41%) patients at a median of 84 (22-148) months to first reintervention. On multivariable logistic regression, residual aortic gradient >10 mm Hg was associated with increased odds of reintervention at all time points when controlling for each final dilation diameter, weight, and minimum-to-descending aortic diameter ratio. CONCLUSIONS: Transverse aortic arch stent implantation has high rates of technical, procedural, and medium-to-long-term clinical success. Aortic gradient >10 mm Hg is associated with increased odds of reintervention at 1-year and most recent follow-ups. Open cell stent design was frequently used for its advantages in conformability, perfusion of arch vessels, low fracture rate, and the ability to perform effective angioplasty of side cells.