BACKGROUND:Infants with hypoplastic left heart syndrome with severely restrictive or intact atrial septum (R/IAS) have low survival. In-utero creation of an atrial septal communication has been reported, with high complication rates. METHODS:We performed a retrospective cohort study of fetuses with hypoplastic left heart syndrome, double outlet right ventricle with mitral stenosis/atresia, or mitral valve dysplasia and R/IAS from 2012 to 2024 who underwent evaluation for fetal atrial septal intervention (FASI). Excluding those with comorbidities limiting candidacy, discharge survival and overall survival were compared between (1) those undergoing and not undergoing FASI, and (2) those undergoing technically successful FASI (TS-FASI) and all others. RESULTS:Of 60 fetuses with R/IAS, 18 were considered extremely high risk and excluded from analysis. Among the remaining 42, 25 underwent FASI and 17 did not, with no significant baseline echocardiographic differences. FASI was technically successful in 18 cases, with 13 survivors (6 post-Glenn, 6 post-Fontan, 1 post-Ross). In the 7 unsuccessful FASIs, there were 5 peri-procedural deaths and 1 fetal demise that occurred post-FASI. Technical success rates improved from 58% (first 12) to 85% (last 13). Of 20 FASI patients with fetal magnetic resonance imaging, 15 (75%) had nutmeg lung; of the 9 with nutmeg lung and post-TS-FASI fetal magnetic resonance imaging, 8 improved. Survival to discharge and overall survival were 52% in the FASI group versus 29% in the non-FASI group (P=0.147 and log-rank P=0.376, respectively). When comparing TS-FASI to all others, survival was higher (72% versus 21% for discharge and overall survival, P=0.001 and log-rank P<0.001, respectively). All TS-FASI survivors with catheterization data (11/13) had pulmonary vascular resistance <3 Woods units before Glenn/Fontan. CONCLUSIONS:In our cohort, fetuses with R/IAS post-TS-FASI had significantly higher discharge and overall survival rates compared with all other fetuses with R/IAS. Nutmeg lung improved in the majority of TS-FASI cases with post-FASI imaging.

Pulmonary artery banding (PAB) is used to restrict pulmonary blood flow in select patients with large left-to-right intracardiac shunts or unrestrictive pulmonary blood flow prior to eventual surgical repair or palliation. More recently, surgical placement of an internal or intraluminal PAB (IPAB) has been used to restrict pulmonary circulation. Here we present two patients who underwent balloon angioplasty of the IPAB to treat cyanosis and improve pulmonary blood flow.

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.

BackgroundPrimary mediastinal B cell lymphoma (PMBCL) is a rare type of non-Hodgkin's lymphoma, and prompt diagnosis and initiation of chemotherapy are necessary to limit compression of cardiovascular structures.Case reportA 13-year-old patient was diagnosed with PMBCL, resulting in acute hypoxemic respiratory failure and cardiogenic shock with clinical pericardial tamponade. Chemotherapy was initiated after veno-arterial extracorporeal membrane oxygenation and subsequent endovascular stenting of the superior vena cava (SVC) to optimize cardiac output due to malignant SVC syndrome.ConclusionWe discuss the challenges of diagnosis and emergency management of pediatric patients with PMBCL and review the existing relevant literature for SVC syndrome secondary to PMBCL.

Background: Hepatic and splenic venous access are specialised techniques used to perform diagnostic and interventional procedures in the cardiac catheterisation laboratory. Bleeding events are the most commonly reported complication following hepatic or splenic venous access. The VASCADE Vascular Closure System (Cardiva Medical Inc. Santa Barbara, CA) is an approved device for closure of femoral vascular access tracts in patients ≥18 years of age. We report our experience using VASCADE to close the hepatic or splenic venous access site in the cardiac catheterisation laboratory. Methods: This is a single centre retrospective review of all patients who had percutaneous hepatic or splenic venous access obtained in the cardiac catheterisation laboratory from March 1, 2022 through October 30, 2023 and underwent tract closure with VASCADE. Results: Ten patients (six male) underwent 16 procedures (median age and weight 3.5 years and 12.5 kg) with 15 hepatic and two splenic veins accessed. Successful closure of the access tracts with VASCADE was performed in all patients. There were no major adverse events related to closure of the access sites with VASCADE. Conclusion: VASCADE can be used following transhepatic and trans-splenic venous access in the cardiac catheterisation laboratory to safely close the access tract and potentially reduce the risk of post-procedural bleeding complications. Further evaluation in a larger cohort of patients is needed to ensure VASCADE is safe for use and provides adequate haemostasis following hepatic or splenic venous access, particularly in children.

Packed red blood cells (PRBC) are frequently ordered for cardiac catheterization procedures, which increases resource utilization and patient charges. We created a quality improvement project focused on optimizing ordering of PRBC for pediatric cardiac procedures. Our primary aim was to increase adherence to ordering guidelines to greater than 97%, with a global aim to decrease patient cost by reducing associated charges. The existing blood product ordering guidelines were revised and procedure reports were updated to include administration of blood products. Appropriate measures were tracked to monitor the rate of pre-procedure PRBC orders, adherence to the new protocol guidelines, verify appropriate documentation and monitor the rate of blood transfusion within 24 hours post-procedure as well as incidence of emergency release blood during a procedure. During our study period, there was a statistically significant process change from a median of 86–100% adherence (p = 0.01) since implementation of the updated guidelines. With improved adherence, we decreased the use of hospital resources and charges to the patient in low-risk cardiac catheterization procedures, without an increase in adverse events.

In patients with transposition of the great arteries, the continuation of prostaglandin E1 is more frequent in patients with intact ventricular septum in comparison to patients with ventricular septal defect. Ballon atrial septostomy did not eliminate the need for prostaglandin E1 infusion until the time of surgery in both subgroups of patients.

Partial anomalous venous connection with sinus venosus atrial septal defect is repaired with different approaches including the Warden procedure. Complications include stenosis of the superior caval vein and pulmonary venous baffle; however, cyanosis is rarely seen post-operatively. We report a patient presenting with cyanosis 5 years after a Warden, which was treated with a transcatheter approach.

Introduction: We describe a unique case of a pediatric patient diagnosed with a diffuse large B cell lymphoma causing acute superior vena cava (SVC) syndrome and cardiogenic shock. She required veno-arterial extracorporeal membrane oxygenation (VA-ECMO) and endovascular SVC stent placement to optimize cardiac output prior to completing her first two rounds of chemotherapy. Description: A 13-year-old girl was admitted to the pediatric intensive care unit (PICU) with acute hypoxemic respiratory failure following three weeks of cough and progressive shortness of breath, initially presumed to be secondary to community acquired pneumonia with a right sided pleural effusion. After escalating respiratory support to endotracheal intubation, she developed acute hypotension and tachycardia with rising lactic acidosis. Further diagnostic workup revealed a large anterior mediastinal mass compressing the SVC, right upper pulmonary vein, right pulmonary artery, and distal trachea. She underwent femoral cannulation to initiate VA-ECMO, but the degree of obstruction of venous return resulted in persistent poor cardiac output with biventricular systolic dysfunction and elevated central venous pressure. To bypass the obstruction, she underwent endovascular stenting of her superior vena cava, resulting in a dramatic improvement in cardiac output while awaiting histopathology results and initiating chemotherapy for a presumed lymphoma. Discussion: This case is the first report of a pediatric patient with an oncologic process who underwent endovascular stent placement while simultaneously requiring VA-ECMO. Pediatric oncology patients had previously been considered relative contraindications to ECMO, but recent reports have described the utility of ECMO as a bridge to initiation of chemotherapy, especially in a setting of a mediastinal mass with vascular invasion or compression. Additionally, SVC syndrome in adults typically is treated with endovascular stenting as first line therapy, often as a palliative procedure in malignant causes of SVC obstruction. Pediatric patients are undergoing endovascular therapy for SVC syndrome more commonly, and this case demonstrates the feasibility of completing an endovascular intervention to augment venous return in a pediatric patient with a mediastinal mass.

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.

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.

Vascular access-related complications are an important consideration in patients undergoing cardiac catheterisation. Patients with CHD are increasingly undergoing percutaneous treatment for suitable procedures as an alternative, less invasive option to surgical intervention. As such, recognition and treatment of these complications are becoming increasingly important. We present a case of a patient with repaired Tetralogy of Fallot who developed a femoral arteriovenous fistula and femoral artery pseudoaneurysm arising from an accessory arterial branch following percutaneous Harmony valve implantation, both of which were treated endovascularly with placement of a stent.

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.

Due to the increasing frequency of pediatric penetrating trauma, pediatric surgeons need to be prepared to evaluate and manage complex penetrating injuries. In this report, we discuss the endovascular management of a traumatic aortic pseudoaneurym and subsequent bullet retrieval following penetrating chest trauma in a child. The key to successful management included multidisciplinary decision making and use of an expandable covered stent generally used for management of aortic coarctation.

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.

The author declares no conflict of interest.

When resources in a society are dispersed unevenly, generally through allocation standards, distinct patterns emerge along lines of socially defined categories of people. Power, religion, kinship, prestige, race, ethnicity, gender, age, sexual orientation, and class all play a role in determining who has access to social goods in society. In most cases, social inequality refers to a lack of equality of outcome, but it can also refer to a lack of equality of access to opportunity. Unfortunately, health care is not immune to these social disparities and/or inequalities. These health care disparities in interventional cardiology were recently brought to the forefront by the Society for Cardiovascular Angiography and Interventions (SCAI) as a major focus of 2020-2021. In a recent publication, unique factors leading to disparities were reported to exist among the subsections of interventional cardiology. The congenital heart disease council of SCAI created a task force to further investigate the unique challenges and disparities impacting the practice of congenital heart disease and pediatric cardiology.