Hypoxia following warden procedure: evaluation and percutaneous treatment.

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.

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.

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.

Fetoscopic Balloon Dilation and Stent Placement of Congenital High Airway Obstruction Syndrome Leading to Successful Cesarean Delivery.

INTRODUCTION: Without fetal or perinatal intervention, congenital high airway obstruction syndrome (CHAOS) is a fatal anomaly. The ex utero intrapartum treatment (EXIT) procedure has been used to secure the fetal airway and minimize neonatal hypoxia but is associated with increased maternal morbidity. CASE PRESENTATION: A 16-year-old woman (gravida 1, para 0) was referred to our hospital at 31 weeks gestation with fetal anomalies, including echogenic lungs, tracheobronchial dilation, and flattened diaphragms. At 32 weeks, fetoscopic evaluation identified laryngeal stenosis, which was subsequently treated with balloon dilation and stent placement. The patient developed symptomatic and regular preterm contractions at postoperative day 7 with persistent sonographic signs of CHAOS, which prompted a repeat fetoscopy with confirmation of a patent fetal airway followed by Cesarean delivery under neuraxial anesthesia. Attempts to intubate through the tracheal stent were limited and resulted in removal of the stent. A neonatal airway was successfully established with rigid bronchoscopy. Direct laryngoscopy and bronchoscopy confirmed laryngeal stenosis with a small tracheoesophageal fistula immediately inferior to the laryngeal stenosis and significant tracheomalacia. A tracheostomy was then immediately performed for anticipated long-term airway and pulmonary management. The procedures were well tolerated by both mom and baby. The baby demonstrated spontaneous healing of the tracheoesophageal fistula by day of life 7 with discharge home with ventilator support at 3 months of life. CONCLUSION: Use of repeated fetoscopy in order to relieve fetal upper airway obstruction offers the potential to minimize neonatal hypoxia, while concurrently decreasing maternal morbidity by avoiding an EXIT procedure. Use of the tracheal stent in CHAOS requires further investigation. The long-term reconstruction and respiratory support of children with CHAOS remain challenging.

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.

Heart Transplantation for Pediatric and Congenital Cardiac Disease: A Comparison of Two Eras over 23 Years and 188 Transplants at a Single Institution.

BACKGROUND: To assess changes in patterns of practice and outcomes over time, we reviewed all patients who underwent heart transplantation (HTx) at our institution and compared two consecutive eras with significantly different immunosuppressive protocols (cohort 1 [80 HTx, June 1995-June 2006]; cohort 2 [108 HTx, July 2006-September 2018]). METHODS: Retrospective study of 180 patients undergoing 188 HTx (June 1995-September 2018; 176 first time HTx, 10 second HTx, and 2 third HTx). In 2006, we commenced pre-HTx desensitization for highly sensitized patients and started using tacrolimus as our primary postoperative immunosuppressive agent. The primary outcome was mortality. Survival was modeled by the Kaplan-Meier method. Univariable and multivariable Cox proportional hazard models were created to identify prognostic factors for survival. RESULTS: Our 188 HTx included 18 neonates, 85 infants, 83 children, and 2 adults (>18 years). Median age was 260.0 days (range: 5 days-23.8 years). Median weight was 7.5 kg (range: 2.2-113 kg). Patients in cohort 1 were less likely to have been immunosensitized preoperatively (12.5% vs 28.7%, P = .017). Nevertheless, Kaplan-Meier analysis suggested superior survival in cohort 2 (P = .0045). Patients in cohort 2 were more likely to be alive one year, five years, and ten years after HTx. Multivariable analysis identified the earlier era (hazard ratio [HR] [95% confidence interval] for recent era = 0.32 [0.14-0.73]), transplantation after prior Norwood operation (HR = 4.44 [1.46-13.46]), and number of prior cardiac operations (HR = 1.33 [1.03-1.71]) as risk factors for mortality. CONCLUSIONS: Our analysis of 23 years of pediatric and congenital HTx reveals superior survival in the most recent 12-year era, despite the higher proportion of patients with elevated panel reactive antibody in the most recent era. This improvement was temporally associated with changes in our immunosuppressive strategy.

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.

Use of Near-Infrared Spectroscopy to Monitor Lower Extremity Perfusion in Pediatric Patients Undergoing Cardiac Catheterization.

Acute femoral artery occlusion is common in pediatric patients following cardiac catheterization. A variety of means are utilized to assess lower extremity (LE) perfusion and arterial patency following cardiac catheterization including palpation of pulses, pulse oximetry, subjective assessment of lower extremity color and temperature, and ultrasound. We sought to evaluate the utility of Near-Infrared Spectroscopy (NIRS) to monitor LE perfusion in pediatric patients undergoing cardiac catheterization. INVOS pediatric sensors were placed on bilateral LE in all pediatric patients ≤ 40 kg undergoing cardiac catheterization. Data were recorded continuously from the start of the procedure until 4-6 h after completion of the procedure. NIRS readings were compared between the accessed versus non-accessed LE at baseline before start of case, time of vascular access, arterial sheath exchange when applicable, sheath withdrawal, and Safeguard application, deflation, and removal. 133 patients underwent 152 catheterizations with mean age 2.4 ± 2.3 years and mean weight 12.4 ± 13.2 kg. NIRS oximetry readings were significantly decreased in the LE with arterial access compared to non-accessed LE from time of sheath insertion until removal of the pressure assist device post procedure. A greater difference was noted in smaller patients. NIRS oximetry readings did not correlate with subjective assessment of lower extremity perfusion after arterial sheaths were removed. One patient had pulse loss 4 h post procedure with a decrease in oximetry readings noted at this point on review. Weight-based heparin protocol was initiated, and a gradual improvement in oximetry readings was noted over the next 5 h. Vascular ultrasound 12 h later showed no evidence of arterial thrombus. NIRS may be helpful in identifying patients who are risk for developing arterial thrombus post cardiac catheterization and for monitoring response to therapy; however, further study in these patients is warranted.

The use of a three-dimensional print model of an aortic arch to plan a complex percutaneous intervention in a patient with coarctation of the aorta.

Recently, three-dimensional printing of heart models is being used to plan percutaneous and surgical interventions in patients with CHD. We describe a case where we used a three-dimensional print model to plan a complex percutaneous intervention in a patient with coarctation of the aorta.

Closure of a secundum atrial septal defect in two infants with chronic lung disease using the Gore HELEX Septal Occluder.

Children with a secundum atrial septal defect are usually asymptomatic and are referred for elective closure after 3-4 years of age; however, in premature infants with chronic lung disease, bronchopulmonary dysplasia, or pulmonary hypertension, increased pulmonary blood flow secondary to a left-to-right atrial shunt, may exacerbate their condition. Closure of the atrial septal defect in these patients can result in significant clinical improvement. We report the cases of two premature infants with chronic lung disease, who underwent atrial septal defect closure with the Gore HELEX Septal Occluder and discuss the technical aspects of using the device in these patients and their clinical outcomes.

Transcatheter management of neonatal aortic stenosis.

Neonatal aortic valvar stenosis can be challenging to treat because of the varied morphology of the valve, the association with hypoplasia of other left heart structures, and the presence of left ventricular systolic dysfunction or endomyocardial fibroelastosis. Balloon valvuloplasty and surgical valvotomy have been well described in the literature for the treatment of neonatal aortic stenosis. Transcatheter therapy for neonatal aortic stenosis is the preferred method at many centres; however, some centres prefer a surgical approach. Balloon valvuloplasty for neonatal aortic stenosis is reviewed in this manuscript, including the history of the procedure, technical aspects, and acute and long-term outcomes.

The role of cardiac catheterisation in patients with tetralogy of Fallot.

Cardiac catheterisation plays an important role in the management of patients with tetralogy of Fallot. There are a number of palliative transcatheter interventions that can be performed in the neonatal period to allow for improved oxygen saturations and interval growth of the pulmonary arteries until corrective surgery is performed. Most patients develop branch pulmonary artery stenosis, right ventricular outflow tract obstruction, pulmonary insufficiency, or significant residual left-to-right shunts during long-term follow-up after corrective surgery. Transcatheter interventions can be performed to treat many of these issues, often eliminating or delaying the need for subsequent surgery. The indications for cardiac catheterisation and the specifics for various interventional procedures for patients with tetralogy of Fallot are reviewed in this manuscript.

Cardiac catheterisation of patients with common arterial trunk and transposition of the great arteries.

Cardiac catheterisation continues to play an important role in the long-term management of patients with common arterial trunk and transposition of the great arteries. Although non-invasive imaging has largely eliminated the need for diagnostic catheterisation in newborns with these congenital cardiac lesions, cardiac catheterisation is an important tool for the diagnosis of a variety of problems encountered after surgical intervention, and allows interventions to be performed when feasible. We review the indications for cardiac catheterisation and describe the specifics for various interventional procedures for these patients in this manuscript.

Blalock-taussig shunt thrombosis prophylaxis in a patient with jacobsen syndrome and thrombocytopenia.

Jacobsen syndrome (JS) is a rare chromosomal anomaly caused by deletions in the distal long arm of chromosome 11. Features of the syndrome include growth and developmental delays, a distinctive facial appearance, and a variety of physical problems including heart defects and bleeding disorders. Congenital heart defects occur in approximately 50% of children with JS. Hypoplastic left heart syndrome (HLHS) has been occasionally reported in association with JS. In such cases, the hematological abnormalities may influence the outcome from single-ventricle palliation through staged surgical reconstruction. Thrombotic obstruction or occlusion of the modified Blalock-Taussig (BT) shunt is a well-documented cause of interstage mortality following the Norwood operation. Although there is no consensus regarding the therapeutic value of antiplatelet therapy during the interstage period following the first stage of palliation, maintenance of shunt patency is critically important. For patients with JS undergoing single-ventricle palliation, decisions regarding antiplatelet therapy during the interstage period may be further complicated by the presence of thrombocytopenia and platelet dysfunction related to JS. We report the case of a patient with HLHS, JS, and thrombocytopenia who underwent the Norwood procedure, and we describe our strategy for prophylaxis against thrombosis of the BT shunt.

Simultaneous stent implantation to treat bifurcation stenoses in the pulmonary arteries: Initial results and long-term follow up.

BACKGROUND: Balloon angioplasty of bifurcating pulmonary artery (PA) stenoses is often inadequate, and stent treatment often requires simultaneous implantation of two stents. This study evaluates initial results and long-term follow up of transcatheter stent placement in bifurcating PAs. METHODS: This is a retrospective review of patients (pts) who had bifurcating PA stents placed in main and lobar branches from 1993 to 2007. RESULTS: Forty-nine pts had bifurcating PA stents placed at a median age of 10.9 years (range 1-43 years). The mean minimum vessel diameter increased from 5.7 +/- 2.5 mm to 11.0 +/- 3.6 mm (P < 0.001), the mean gradient across the stenoses decreased from 37.0 +/- 26.9 to 9.2 +/- 13 mm Hg (P < 0.001), whereas the mean RV:FA ratio decreased from 0.76 +/- 0.29 to 0.53 +/- 0.24 (P < 0.001). There was one death due to severe pulmonary hemorrhage. F/U data were available in 38 pts (mean duration 6.3 +/- 4.1 years, range 1.2-13.1 years). Thirty pts underwent repeat catheterizations (mean 2.3 +/- 2.2 years poststent), with 26 requiring further interventions: Fifteen had balloon angioplasty alone and 11 had additional stents placed. There were no complications at f/u catheterization. Six pts underwent further palliative surgeries, although none for repair of branch PA stenoses. CONCLUSIONS: Simultaneous transcatheter placement of bifurcating PA stents provides immediate gradient relief of bifurcating stenoses in the proximal or lobar branch PAs and reduces RV systolic pressure. Further interventions can be safely performed in future procedures, and the presence of stents does not complicate future surgeries.

Efficient, long-term hepatic gene transfer using clinically relevant HDAd doses by balloon occlusion catheter delivery in nonhuman primates.

Helper-dependent adenoviral vectors (HDAd) are devoid of all viral coding sequences and are thus an improvement over early generation Ad because they can provide long-term transgene expression in vivo without chronic toxicity. However, high vector doses are required to achieve efficient hepatic transduction by systemic intravenous injection, and this unfortunately results in dose-dependent acute toxicity. To overcome this important obstacle, we have developed a minimally invasive method to preferentially deliver HDAd into the liver of nonhuman primates. Briefly, a balloon occlusion catheter was percutaneously positioned in the inferior vena cava to occlude hepatic venous outflow. HDAd was injected directly into the occluded liver via a percutaneously placed hepatic artery catheter. Compared to systemic vector injection, this approach resulted in substantially higher hepatic transduction efficiency using clinically relevant low vector doses and was accompanied by mild-to-moderate acute but transient toxicities. Transgene expression was sustained for up to 964 days. These results suggest that our minimally invasive method of delivery can significantly improve the vector's therapeutic index and may be a first step toward clinical application of HDAd for liver-directed gene therapy.