Hybrid Interventions for Pulmonary Vein Stenosis: Leveraging Intraoperative Endovascular Adjuncts in Challenging Clinical Scenarios.

Background: Pediatric pulmonary vein stenosis (PVS) is often progressive and treatment-refractory, requiring multiple interventions. Hybrid pulmonary vein interventions (HPVIs), involving intraoperative balloon angioplasty or stent placement, leverage surgical access and customization to optimize patency while facilitating future transcatheter procedures. We review our experience with HPVI and explore potential applications of this collaborative approach. Methods: Retrospective chart review of all HPVI cases between 2009 to 2023. Results: Ten patients with primary (n = 5) or post-repair (n = 5) PVS underwent HPVI at median age of 12.7 months (range 6.6 months-9.5 years). Concurrent surgical PVS repair was performed in 7/10 cases. Hybrid pulmonary vein intervention was performed on 17 veins, 13 (76%) with prior surgical or transcatheter intervention(s). One patient underwent intraoperative balloon angioplasty of an existing stent. In total, 18 stents (9 bare metal [5-10 mm diameter], 9 drug eluting [3.5-5 mm diameter]) were placed in 16 veins. At first angiography (median 48 days [range 7 days-2.8 years] postoperatively), 8 of 16 (50%) HPVI-stented veins developed in-stent stenosis. Two patients died from progressive PVS early in the study, one prior to planned reintervention. Median time to first pulmonary vein reintervention was 86 days (10 days-2.8 years; 8/10 patients, 13/17 veins). At median survivor follow-up of 2.2 years (2.3 months-13.1 years), 1 of 11 surviving HPVI veins were completely occluded. Conclusions: Hybrid pulmonary vein intervention represents a viable adjunct to existing PVS therapies, with promising flexibility to address limitations of surgical and transcatheter modalities. Reintervention is anticipated, necessitating evaluation of long-term benefits and durability as utilization increases.

Myocardial bridges in a pediatric population: Outcomes following a standardized approach.

OBJECTIVE: To describe clinical, functional, surgical, and outcomes data in pediatric patients with a myocardial bridge (MB) evaluated and managed following a standardized approach. METHODS: Prospective observational study included patients evaluated in the Coronary Artery Anomalies Program. Anatomy was determined by computed tomography angiography, myocardial perfusion by stress perfusion imaging, and coronary hemodynamic assessment by cardiac catheterization. RESULTS: In total, 39 of 42 patients with a complete evaluation for MB were included (December 2012 to June 2022) at a median age of 14.1 years (interquartile range, 12.2-16.4). Sudden cardiac arrest occurred in 3 of 39 (8%), exertional symptoms in 14 (36%), and no/nonspecific symptoms in 7 (18%) patients. Exercise stress test was abnormal in 3 of 34 (9%), stress perfusion imaging in 8 of 34 (24%), and resting instantaneous wave-free ratio ≤0.89 or diastolic dobutamine fractional flow reserve ≤0.80 in 11 of 21 (52%) patients. As a result, 15 of 39 (38%) patients were determined to have hemodynamically significant MB, 1 of 15 patients started beta-blocker, and 14 of 15 were referred for surgery. Myotomy (n = 11) and coronary bypass (n = 1) were performed successfully, resulting in improved symptoms and stress testing results. One patient required pericardiocentesis postoperatively, and all were discharged without other complications. At median follow-up time of 2.9 (1.8-5.8) years, all (except 2 pending surgery) were doing well without exercise restriction. CONCLUSIONS: Pediatric patients with MB can present with myocardial ischemia and sudden cardiac arrest. Provocative stress test and intracoronary hemodynamic tests helped risk-stratify symptomatic patients with MB and concern for ischemia. Surgical repair was safe and effective in mitigating exertional symptoms and stress test results, allowing patients to return to exercise without restriction.

Implementing a Pediatric Pulmonary Embolism Response Team Model: An Institutional Experience.

Pulmonary embolism is increasing in prevalence among pediatric patients; although still rare, it can create a significant risk for morbidity and death within the pediatric patient population. Pulmonary embolism presents in various ways depending on the patient, the size of the embolism, and the comorbidities. Treatment decisions are often driven by the severity of the presentation and hemodynamic effects; severe presentations require more invasive and aggressive treatment. We describe the development and implementation of a pediatric pulmonary embolism response team designed to facilitate rapid, multidisciplinary, data-driven treatment decisions and management.

Retrieval of Large Balloon Fragments During Transcatheter Pulmonary Valve Implantation Using a Novel Retrieval System.

The removal of balloon fragments from the pulmonary artery without damaging the pulmonary and tricuspid valves can be difficult. Four cases during transcatheter pulmonary valve replacement are described in which a novel retrieval system was used to facilitate safe removal. (Level of Difficulty: Advanced.).

Single tertiary center experience using Gore Cardioform Atrial Septal Defect Occluder for secundum atrial septal defect closure with a focus on deficient rims.

BACKGROUND: The data on the use of Gore Cardioform Septal Occluder (GCA; W. L. Gore and Associates, Inc.) for atrial septal defect (ASD) with deficient rims is limited. METHODS: All patients evaluated by transesophageal echocardiogram (TEE) for ASD occlusion were included. TEE planes at 35°, 0°, and 90° were assessed for anterior-superior (AS) and posterior (P), anterior-inferior (AI) and posterior-superior (PS), as well as superior (S) and inferior (I) rims. ASD size >20 mm, and rims less than 5 mm were defined as large and deficient, respectively. We included patients who had a procedural failure along with the patients in whom the procedure was not attempted after echocardiogram in the unsuccessful group. RESULTS: In 148 patients, the median weight, age, and ASD size were 36 kg (range, 8-60 kg), 11.8 years (range, 1-60 years), and 14.2 ± 8.28 mm, respectively. One or more deficient rims were noted in 112 of 148 (75.7%): 99 (67%) AS, 36 (24%) P, 17 (11%) AI, 30 (20%) PS, 26 (18%) S, and 33 (22%) I. ASD closure was performed in 115 (78%) patients. The procedure was successful in 111 (96.5%) patients with procedural failure in 4 (3.4%) patients. Multiple deficient rims were associated with reduced procedural success (OR 0.36, 95% CI, 0.25-0.56). On multivariate analysis deficient P, PS, and I rims were associated with an unsuccessful group (P = .001, .046, and .005, respectively). Complications included 1 device embolization, 1 vascular injury, and 5 arrhythmias. CONCLUSIONS: Transcatheter closure of ASDs with deficient rims is feasible using GCA. Large ASDs with deficient P, PS, and I rims were associated with unsuccessful closure. Risk stratification and comprehensive evaluation of ASD rims is vital for the use of GCA.

Pre-Fontan Assessment Utilizing Combined Cardiac Catheterization and Cardiac MRI: Comparison to the Pre-Fontan Catheterization.

Single ventricle patients eligible for Fontan completion undergo pre-Fontan catheterization for hemodynamic and anatomic assessment prior to surgery. Cardiac magnetic resonance imaging may be used to evaluate pre-Fontan anatomy, physiology, and collateral burden. We describe our center's outcomes in patients undergoing pre-Fontan catheterization combined with cardiac magnetic resonance imaging. A retrospective review of patients undergoing pre-Fontan catheterization from 10/2018 to 04/2022 at Texas Children's Hospital was performed. Patients were divided into 2 groups: combined cardiac magnetic resonance imaging and catheterization (combined group) and those who underwent catheterization only (catheterization only group). There were 37 patients in the combined group and 40 in the catheterization only group. Both groups were similar in age and weight. Patients undergoing combined procedures received less contrast, and experienced less in-lab time, fluoroscopy time and catheterization procedure time. Median radiation exposure was lower in the combined procedure group but was not statistically significant. Intubation and total anesthesia times were higher in the combined procedure group. Patients undergoing a combined procedure were less likely to have collateral occlusion performed than in the catheterization only group. Bypass time, intensive care unit length of stay, and chest tube duration were similar in both groups at the time of Fontan completion. Combined pre-Fontan assessment decreases catheterization procedure and fluoroscopy time associated with cardiac catheterization at the expense of longer anesthetic times, and results in similar Fontan outcomes compared to when cardiac catheterization alone is utilized.

Extra-cardiac and complex Fontan baffle fenestration using radio frequency current via surgical electrocautery.

BACKGROUND: Fontan baffle punctures and creation of Fontan fenestration for cardiac catheterisation procedures remain challenging especially due to the heavy calcification of prosthetic material and complex anatomy. OBJECTIVES: We sought to evaluate our experience using radiofrequency current via surgical electrocautery needle for Fontan baffle puncture to facilitate diagnostic, electrophysiology, and interventional procedures. METHODS: A retrospective chart review of all Fontan patients (pts) who underwent Fontan baffle puncture using radiofrequency energy via surgical electrocautery from three centres were performed from January 2011 to July 2021. RESULTS: A total of 19 pts underwent 22 successful Fontan baffle puncture. The median age and weight were 17 (3-36 years) and 55 (14-88) kg, respectively. The procedural indications for Fontan fenestration creation included: diagnostic study (n = 1), atrial septostomy and stenting (n = 1), electrophysiology study and ablation procedures (n = 8), Fontan baffle stenting for Fontan failure including protein-losing enteropathy (n = 7), and occlusion of veno-venous collaterals (n = 2) for cyanosis. The type of Fontan baffles included: extra-cardiac conduits (n = 12), lateral tunnel (n = 5), classic atrio-pulmonary connection (n = 1), and intra-cardiac baffle (n = 1). A Fontan baffle puncture was initially attempted using traditional method in 6 pts and Baylis radiofrequency trans-septal system in 2 pts unsuccessfully. In all pts, Fontan baffle puncture using radiofrequency energy via electrocautery needle was successful. The radiofrequency energy utilised was (10-50 W) and required 1-5 attempts for 2-5 seconds. There were no vascular or neurological complications. CONCLUSIONS: Radiofrequency current delivery using surgical electrocautery facilitates Fontan baffle puncture in patients with complex and calcified Fontan baffles for diagnostic, interventional, and electrophysiology procedures.

Evolution of surgical repair of intraseptal anomalous left coronary artery with myocardial ischaemia.

BACKGROUND: Anomalous aortic origin of the left coronary artery with intraseptal course is a rare coronary anomaly associated with an increased risk of myocardial ischaemia. The role and techniques for surgical intervention are evolving, with numerous novel surgical techniques for this challenging anatomy reported in the last 5 years. We report our single-centre experience with surgical repair of intraseptal anomalous left coronary artery in the paediatric population, including clinical presentation, evaluation, and short- to mid-term outcomes. METHODS: All patients with coronary anomalies presenting to our institution undergo standardised clinical evaluation. Five patients aged 4 to 17 years underwent surgical intervention for intraseptal anomalous aortic origin of the left coronary artery between 2012 and 2022. Surgical techniques included coronary artery bypass grafting (n = 1), direct reimplantation with limited supra-arterial myotomy via right ventriculotomy (n = 1), and transconal supra-arterial myotomy with right ventricular outflow tract patch reconstruction (n = 3). RESULTS: All patients had evidence of haemodynamically significant coronary compression, and three had evidence of inducible myocardial ischaemia pre-operatively. There were no deaths or major complications. Median follow-up was 6.1 months (range 3.1-33.4 months). Patients who underwent supra-arterial myotomy (with or without reimplantation) had improved coronary flow and perfusion based on stress imaging and catheterisation data. CONCLUSIONS: Surgical approaches to intraseptal anomalous left coronary artery with evidence of myocardial ischaemia continue to evolve, with new techniques demonstrating promising improvement in coronary perfusion. Further studies are warranted to determine long-term outcomes and refine indications for repair.

Pulmonary Vein Atresia: Feasibility of Initial Recanalization Attempts with Subsequent Follow-up.

Pulmonary vein atresia (PVA) may lead to pulmonary hypertension, cardiac failure, and death. Transcatheter or surgical treatments have rarely been offered to this population because of perceived poor outcomes. We describe single center outcomes of transcatheter management of PVA. Retrospective chart review of PVA patients who underwent cardiac catheterization at a single tertiary center. Sixty patients underwent catheterization for evaluation of PVA from 1995 to 2019. The age at the initial catheterization was 1.6 (0.7, 5.97) years. Two thirds of PVA patients had associated congenital heart disease (n=40). PVA recanalization was attempted in 34 patients, successful in 23/34 (68%) of the initial attempts. 3/23 (13%) underwent balloon angioplasty alone, and 20/23 (87%) received drug-eluting stents, with no procedural mortalities. 22/23 patients had transcatheter reinterventions during an interval of 2.1 (0.3, 5.1) years. Right ventricular systolic to aortic systolic pressure ratio (in biventricular patients) at the index catheterization was 0.45 (0.34, 0.68) in survivors versus 0.69 (0.54, 0.83) in those who died; P = 0.012 (n=45). The baseline right ventricular or pulmonary artery systolic to aortic systolic pressure ratio of ≥0.54 at the initial catheterization was predictive of mortality. We hereby demonstrate that transcatheter recanalization of PVA with placement of drug-eluting stents can be performed safely with acceptable success rate. With appropriate use of re-interventions for restenosis as indicated, PVA can be successfully palliated with good long-term patency and distal growth of the affected veins. Pulmonary hypertension is a risk factor for mortality in patients with PVA and biventricular circulation. Percutaneous recanalization of PVA is safe and feasible, and with placement of drug-eluting stents, carries a high acute success rate and results in growth of the distal pulmonary veins. However, close vigilance and reinterventions are required due to restenosis.

Pulmonary embolism in pediatric and adolescent patients with COVID-19 infection during the SARS-CoV-2 delta wave.

Coronavirus disease 2019 (COVID-19) infection in children has been associated with thrombosis, though few cases of COVID-associated pulmonary embolism (PE) have been described. We performed a retrospective review of the nine cases of COVID-19-associated PE during the B.1617.2 variant surge at Texas Children's Hospital. The patient cohort largely contained unvaccinated obese adolescents. All patients were critically ill with two requiring catheter-directed thrombolysis in addition to anticoagulation. Eight of the nine patients had COVID pneumonia along with PE. This report stresses the importance of maintaining a high index of suspicion for PE in pediatric COVID-19 infection and vaccinating obese adolescent patients.

Anatomical Classification and Posttreatment Remodeling Characteristics to Guide Management and Follow-Up of Neonates and Infants With Coronary Artery Fistula: A Multicenter Study From the Coronary Artery Fistula Registry.

BACKGROUND: Coronary artery fistulas (CAFs) presenting in infancy are rare, and data regarding postclosure sequelae and follow-up are limited. METHODS: A retrospective review of all the neonates and infants (<1 year) was conducted from the CAF registry for CAF treatment. The CAF type (proximal or distal), size, treatment method, and follow-up angiography were reviewed to assess outcomes and coronary remodeling. RESULTS: Forty-eight patients were included from 20 centers. Of these, 30 were proximal and 18 had distal CAF; 39 were large, 7 medium, and 2 had small CAF. The median age and weight was 0.16 years (0.01-1) and 4.2 kg (1.7-10.6). Heart failure was noted in 28 of 48 (58%) patients. Transcatheter closure was performed in 24, surgical closure in 18, and 6 were observed medically. Procedural success was 92% and 94 % for transcatheter closure and surgical closure, respectively. Follow-up data were obtained in 34 of 48 (70%) at a median of 2.9 (0.1-18) years. Angiography to assess remodeling was available in 20 of 48 (41%). I. Optimal remodeling (n=10, 7 proximal and 3 distal CAF). II. Suboptimal remodeling (n=7) included (A) symptomatic coronary thrombosis (n=2, distal CAF), (B) asymptomatic coronary thrombosis (n=3, 1 proximal and 2 distal CAF), and (C) partial thrombosis with residual cul-de-sac (n=1, proximal CAF) and vessel irregularity with stenosis (n=1, distal CAF). Finally, (III) persistent coronary artery dilation (n=4). Antiplatelets and anticoagulation were used in 31 and 7 patients post-closure, respectively. Overall, 7 of 10 (70%) with proximal CAF had optimal remodeling, but 5 of 11 (45%) with distal CAF had suboptimal remodeling. Only 1 of 7 patients with suboptimal remodeling were on anticoagulation. CONCLUSIONS: Neonates/infants with hemodynamically significant CAF can be treated by transcatheter or surgical closure with excellent procedural success. Patients with distal CAF are at higher risk for suboptimal remodeling. Postclosure anticoagulation and follow-up coronary anatomic evaluation are warranted.

Mullins-Sheath Facilitated Delivery of Gore Cardioform ASD Occluder Devices for Closure of Large or Challenging Secundum Atrial Septal Defects.

OBJECTIVES: To describe a deployment technique of the Gore Cardioform atrial septal defect (ASD) occluder (W.L. Gore and Associates) for large secundum ASDs and ASDs with challenging anatomy. BACKGROUND: The Gore Cardioform ASD occluder has recently been approved for closure of secundum ASDs; however, there are limitations to its delivery system. METHODS: A retrospective study was conducted on the use of a Mullins sheath (Cook Medical) to facilitate Gore Cardioform ASD occluder delivery for secundum ASD closure in the cardiac catheterization laboratory from June, 2017 to December, 2019 at Texas Children's Hospital/Baylor College of Medicine. RESULTS: Out of 98 patients who underwent an attempt at ASD closure using the Gore Cardioform ASD occluder, a Mullins sheath was used in 52 patients (median age, 8 years [interquartile range, 4-13 years] and weight 27.2 kg [interquartile range, 17.9-51.2 kg]), with a successful implant in 46/52 patients (88%). The Mullins sheath was primarily used to deliver large devices (>32 mm) in 38/46 successful implants (83%). There were 2 major adverse events (atrial fibrillation requiring cardioversion). At a median follow-up of 43 days (interquartile range, 1-374 days), no patient had more than a mild residual shunt. The ASD size, maximum sheath size, and device size were larger in patients in whom the Mullins sheath was used as compared with those patients in whom a Mullins sheath was not used. CONCLUSIONS: The Mullins sheath-facilitated delivery of the Gore Cardioform ASD occluder device may be a useful adjunct technique for closure of large secundum ASDs and secundum ASDs with challenging anatomy.

SAPIEN S3 valve deployment in the pulmonary position using the gore DrySeal sheath to protect the tricuspid valve.

BACKGROUND: Tricuspid valve injury can occur during implantation of a SAPIEN valve in the pulmonary position. We describe our experience using a long Gore DrySeal (GDS) sheath to protect the tricuspid valve during advancement of the Commander delivery system. METHODS: Retrospective single center review of all patients who underwent placement of a SAPIEN valve in the right ventricular outflow tract between January 2016 and April 2020. Patients were divided into two groups: delivery of the valve using standard technique (Group I), and with the use of a GDS (Group II), for comparison. RESULTS: There were 48 patients in total: 25 in Group I and 23 in Group II. In Group II, the first 10 patients had a 29 mm S3 placed through a 26 French (Fr), 65 cm GDS. We then performed additional crimping of the S3 onto the balloon after the balloon catheter was withdrawn to position the valve on the balloon outside the body. Subsequently, seven had a 29 mm S3 placed through a 24 Fr GDS, and four had a 26 mm S3 placed through a 22 Fr GDS including one weighing 16 kg. Two had a 23 mm S3 placed through a 22Fr GDS as the 20Fr GDS was not available in our lab. Severe tricuspid valve injury occurred in 2/25 (8%) of Group I patients and 0/23 of Group II patients. CONCLUSION: Use of a long GDS may protect the tricuspid valve from injury during implantation of the S3 valve in the pulmonary position, and is technically feasible in smaller patients.

Resolution of Hypoxia and Ascites With Percutaneous Intervention of Mustard Baffle Obstruction and Leak.

A 45-year-old man with history of Mustard repair for transposition of the great arteries, cirrhosis, and chronic hypoxemic respiratory failure presented for subacute worsening of his chronic symptoms, which were found to be secondary to a previously unrecognized baffle stenosis and leak. Percutaneous intervention resolved his ascites and hypoxia. (Level of Difficulty: Intermediate.).