Apical muscular ventricular septal defect closure via hybrid approach using a right ventricular stay suture.

Transcatheter closure of large apical muscular ventricular septal defects (VSDs) can be performed via transfemoral or hybrid approach. A very large apical muscular VSD was closed via a hybrid approach. A strategy for deployment of a right ventricular stay suture was utilized to minimize the risk of device embolization without the use of bypass and without externalization of a portion of the device.

Radiation dose reduction for 3D angiography images in pediatric and congenital cardiology.

OBJECTIVES: The purpose of this study was to investigate the influence of simulated reduced-dose three-dimensional angiography (3DA) on the accuracy and precision of linear measurements derived from 3DA datasets. BACKGROUND: Three-dimensional angiography is performed during X-ray guided interventional procedures to aid diagnosis and inform treatment strategies for children and adults with congenital heart disease. However, 3DA contributes substantially to patient radiation dose and may lead to an increased radiation-induced cancer risk. METHODS: Reduced-dose patient 3DA images were simulated by adding quantum noise to the 2D projection angiograms, then reconstructing the projection angiograms into the 3DA dataset. Dose reduction in the range 33-72% was simulated. Five observers performed 46 vessel diameter measurements along prespecified axes within 23 vessel segments from 11 patient 3DA datasets. Statistical tests were performed to assess the influence of radiation dose reduction on the accuracy and precision of vessel diameter measurements. RESULTS: Vessel diameter measurements were in the range 5.9- 22.7 mm. Considering all vessel segments and observers, the influence of dose level on the accuracy of diameter measurements was in the range 0.02 - 0.15 mm (p .05-.8). Interobserver variability increased modestly with vessel diameter, but was not influence by dose level (p = .52). The statistical test for observer recall bias was negative (p = .51). CONCLUSIONS: Simulated dose reduction up to 72% did not affect the accuracy or precision of the diameter measurements acquired from 3DA images. These findings may embolden 3DA radiation dose reduction for pediatric and congenital heart disease patients.

Surgically placed radiopaque markers: Proof-of-concept of a novel technique to facilitate percutaneous interventions in neonates and infants.

OBJECTIVES: Aim of this study was to evaluate feasibility and benefit of self-designed, radiopaque markers as a novel technique in neonates and infants with shunt- or duct-dependent lesions. BACKGROUND: Surgically placed radiopaque markers have the potential to facilitate postoperative percutaneous interventions. METHODS: All consecutive children with surgically placed radiopaque markers involving systemic-to-pulmonary artery connections or arterial ducts in the context of hybrid palliation and subsequent cardiac catheterization between January 2013 and March 2019 were included in this analysis. Our primary endpoint was our concept's feasibility, which we defined as a combination of surgical feasibility and the percutaneous intervention's success. Secondary endpoint was the rate of complications resulting from the surgical procedure or during catheterization. RESULTS: Radiopaque markers that reveal the proximal entry of a surgical shunt or the arterial duct proved to be a feasible and beneficial approach in 25 postoperative catheterizations. The markers' high accuracy enabled easy probing and proper stent positioning in 13 neonates with a median age and weight of 121 days (range 9-356) and 4.7 kg (1.6-9.4) at the intervention. No procedural complications or unanticipated events associated with the radiopaque marker occurred. The markers were never lost, never migrated, and caused no local obstructive lesion. Surgical removal was straightforward in all patients. CONCLUSIONS: Radiopaque markers are a promising and refined technique to substantially facilitate target vessel access and enabling the accurate positioning of stents during postoperative percutaneous procedures.

Feasibility and early outcomes of aortic coarctation treatments with BeGraft Aortic stent.

OBJECTIVES: To assess feasibility and early outcomes of using BeGraft Aortic stent in the treatment of aortic coarctation (CoA). BACKGROUND: BeGraft Aortic stent (Bentley InnoMed, Hechingen, Germany) allows large postdilation diameter up to 30 mm. With availability of lengths of 19-59 mm and lower stent profile, they can be used in native and recurrent CoA in adults and in pediatric patients. MATERIALS AND METHODS: This is a multicentre retrospective analysis of 12 implanted BeGraft Aortic stents in CoA between May 2017 and April 2019. RESULTS: Twelve patients aged 7.7-38 years (median 18.3 years) with body weight of 19.9-56 kg (median 45.5 kg). Eight patients (66%) had native juxtaductal CoA while four had recurrent CoA after previous surgical or transcatheter treatments. The stents were implanted successfully in all the patients with no serious adverse events. The length of the stents ranged from 27 to 59 mm and the implanted stent diameter varied from 12 to 18 mm. The median intraprocedural CoA pressure gradient decreased from 25 mmHg (range 16-66 mmHg) to 2 mmHg (range 0-13 mmHg). The mean follow-up duration was 10.2 months. Two patient (16.6%) had residual stent narrowing requiring staged redilation. One patient (8%) had pseudoaneurysm formation at 1 year cardiac CT follow-up. CONCLUSIONS: The BeGraft Aortic stent may be considered to be safe and effective in the short term in treatment of CoA from childhood to adulthood. Long-term follow-up is needed.

Transcatheter treatment of acquired coronary sinus ostium atresia in a child with complex congenital heart disease.

Congenital coronary sinus ostium atresia is a rare condition that is typically considered benign, as long as there is adequate drainage via another route. However, in children with single ventricle congenital heart disease, adequate drainage may not be assured after complex surgical interventions, putting them at risk for myocardial injury. We present a patient with complex single ventricle congenital heart disease who developed acquired coronary sinus ostium atresia after surgical intervention that was treated by a transcatheter approach to prevent the sequelae of coronary sinus hypertension.

Transcatheter creation of bidirectional cavopulmonary connections by needle punctures in two patients.

We report on two patients who received a transcatheter cavopulmonary connection by a needle puncture under deep conscious sedation. In both patients, the vessel-to-vessel connection was achieved by a venous access into the superior caval vein and direct needle puncture of the pulmonary artery. The two cavopulmonary anastomoses were held open by a covered stent and a bare-metal stent, respectively.

Preclinical comparative assessment of a dedicated pediatric poly-L-lactic-acid-based bioresorbable scaffold with a low-profile bare metal stent.

BACKGROUND: Polymer-based bioresorbable scaffolds (PBBS) have been assessed for coronary revascularization with mixed outcomes. Few studies have targeted pediatric-specific scaffolds. We sought to assess safety, efficacy, and short-term performance of a dedicated drug-free PBBS pediatric scaffold compared to a standard low-profile bare metal stent (BMS) in central and peripheral arteries of weaned piglets. METHODS: Forty-two devices (22 Elixir poly-L-lactic-acid-based pediatric bioresorbable scaffolds [BRS] [6 × 18 mm] and 20 control BMS Cook Formula 418 [6 × 20 mm]) were implanted in the descending aorta and pulmonary arteries (PAs) of 14 female Yucatan piglets. Quantitative measurements were collected on the day of device deployment and 30 and 90 days postimplantation to compare device patency and integrity. RESULTS: The BRS has a comparable safety profile to the BMS in the acute setting. Late lumen loss (LLL) and percent diameter stenosis (%DS) were not significantly different between BRS and BMS in the PA at 30 days. LLL and %DS were greater for BRS versus BMS in the aorta at 30 days postimplantation (LLL difference: 0.96 ± 0.26; %DS difference: 16.15 ± 4.51; p < .05). At 90 days, %DS in the aortic BRS was less, and PA BRS LLL was also less than BMS. Histomorphometric data showed greater intimal proliferation and area stenosis in the BRS at all time points and in all vessels. CONCLUSIONS: A dedicated PBBS pediatric BRS has a favorable safety profile in the acute/subacute setting and demonstrates characteristics that are consistent with adult BRSs.

Compression of the left mainstem bronchus by patent ductus arteriosus in neonates under consideration for ductal stenting.

Stent angioplasty of patent ductus arteriosus has been shown to be a viable alternative to operative shunt placement in cyanotic neonates. With broader implementation of this strategy, novel complications are bound to arise. We present a series of cases evaluated for ductal stent angioplasty in which a dilated and torturous ductus arteriosus compressed the left mainstem bronchus. After reviewing our recent experience with ductal stenting and isolated Blalock-Taussig shunts, our best estimate of the incidence of bronchial compression by the dilated ductus is 4.6% (3/64, 95% confidence interval 1.0-12.9%). Awareness of the airway and other nonvascular contents of the thorax is an important consideration prior to ductal stenting.

Pulmonary hemorrhage in children with Alagille syndrome undergoing cardiac catheterization.

OBJECTIVES: To evaluate the incidence, severity, and outcomes of pulmonary hemorrhage in children with Alagille syndrome (AGS) undergoing cardiac catheterization, and to find variables associated with hemorrhage in this population. BACKGROUND: Children with AGS have a high incidence of bleeding complications during invasive procedures. It has been our impression that catheterization-associated pulmonary hemorrhage is more common in children with AGS, but there are no published data on this topic. METHODS: This was a retrospective single institution study of children with AGS undergoing catheterization from 2010 to 2018. Pulmonary hemorrhage was defined as angiographic or fluoroscopic evidence of extravasated blood in the lung parenchyma, or blood suctioned from the endotracheal tube with documentation of pulmonary hemorrhage by the anesthesiologist or intensivist. Univariate comparisons were made between catheterizations that did and did not have pulmonary hemorrhage. RESULTS: Thirty children with AGS underwent 87 catheterizations, 32 (37%) with interventions on the branch pulmonary arteries (PA). There were 26 (30%) procedures with hemorrhage, the majority (65%) of which were self-limited or required less than 24 hr of mechanical ventilation. Moderate and severe hemorrhage occurred only in children with tetralogy of Fallot (TOF; 5 of 14, 36%). A higher right ventricle to aorta systolic pressure ratio (1.0 [0.85-1.1] vs. 0.88 [0.59-1.0], p = .029) and interventions on the branch PAs (14 of 26, 54% vs. 18 of 61, 30%, p = .032) were associated with hemorrhage. CONCLUSIONS: Pulmonary hemorrhage was common in children with AGS undergoing both intervention and diagnostic cardiac catheterization, and was associated with TOF, higher RV to aorta pressure ratio, and interventions on the branch PAs.

Decompression of the thoracic duct: A novel transcatheter approach.

In patients with total cavopulmonary connections, elevated central venous pressures (CVP) have detrimental effects on the lymphatic system causing an imbalance in fluid production and drainage of the interstitium. This combination may result in life-threatening lymphatic complications including plastic bronchitis (PB), protein losing enteropathy (PLE), chylothorax, and ascites. While embolization of the abnormal lymphatics has greatly improved outcomes from these complications, alternative treatment strategies have been proposed that would result in improved lymphatic drainage while leaving the lymphatic system intact. We report two novel transcatheter approaches for thoracic duct (TD) decompression in two patients who developed PLE after completion of the Fontan procedure as part of staged palliation for congenital heart disease. In addition, one patient had severe concurrent PB. In both patients, a connection was created between a left superior vena cava (LSVC) to the left atrium allowing for a nonsurgical method to decompress the TD. This procedure resulted in significant clinical and laboratory improvement of both patients' PLE and other symptoms of lymphatic dysfunction.

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.

Working backward: Retrograde balloon angioplasty of atretic arteries in chronic thromboembolic pulmonary hypertension.

We describe a patient with chronic thromboembolic pulmonary hypertension who presented to the pediatric cardiac catheterization laboratory for peripheral pulmonary artery recanalization and angioplasty. This case report outlines serial pulmonary arterial rehabilitation via a retrograde approach through intrapulmonary collateral arterial connections.

An unusual case of coronary artery compression that did not preclude successful transcatheter pulmonary valve placement.

During transcatheter pulmonary valve placement, coronary compression observed during simultaneous right ventricular outflow tract angioplasty and coronary angiography typically contraindicates valve implantation. We present a unique patient with tetralogy of Fallot who underwent successful transcatheter Melody valve placement despite coronary compression observed during right ventricular outflow tract balloon angioplasty.

Stent implantation to relieve native obstructed left partial anomalous pulmonary venous connections.

Obstructed partial anomalous pulmonary venous connections (APVC) are rare but may be associated with severe pulmonary hypertension (PHTN) and warrant urgent relief. There are a number of case reports of successful catheter intervention for obstructed total APVC. We present the first reported case of catheter intervention to relieve obstructed, left sided PAPVC in a neonate with Turner syndrome. © 2016 Wiley Periodicals, Inc.

Outcomes using a clinical practice pathway for the management of pulse loss following pediatric cardiac catheterization.

OBJECTIVE: Objectives To describe the results of a clinical practice pathway (CPP) for the management of postcatheterization pulse loss in a children's hospital. BACKGROUND: Standardized approaches to the diagnosis and management of postcatheterization arterial thrombus are lacking. As a result, substantial practice variation exists. METHODS: Data collected prospectively for quality improvement purposes were retrospectively reviewed. RESULTS: Since initiation of the CPP, 93/1,672 (5.4%) catheterizations resulted in pulse loss at a median patient age and weight of 73 days (1 day-5.8 years) and 4.8 kg (2-14.1 kg). Arterial thrombus was documented by ultrasound (US) in 85. Of these, 66 resolved by 12 weeks of therapy, seven patients died, and four were lost to follow-up before completing treatment. Eight patients had persistent thrombus despite a full treatment course (89% success rate in those able to complete treatment). Of patients treated with unfractionated heparin as initial therapy, 46% (17/37) achieved a therapeutic partial thromboplastin time within 12 hr with 19% (67/343) of all levels therapeutic. As a result, the CPP was modified to use enoxaparin as first line agent, of which 57% (41/72) had a therapeutic anti-Xa level after the 2nd dose and 88% by the 4th dose. No bleeding complications were observed. A priori established process metrics were achieved. CONCLUSIONS: A CPP utilizing early initiation of anticoagulation and US to aid diagnosis of postcatheterization arterial thrombus and response to therapy is feasible and effective. In those able to complete up to 12 weeks of treatment, resolution occurs in nearly 90%. © 2014 Wiley Periodicals, Inc.

Early clinical experience with a novel self-expanding percutaneous stent-valve in the native right ventricular outflow tract.

INTRODUCTION: Balloon expandable transcatheter pulmonary valve systems are not applicable to the large majority of patients with chronic severe pulmonary regurgitation (PR) following surgical right ventricular outflow tract (RVOT) rehabilitation. This report describes the clinical use and short-term follow-up of a novel transcatheter self-expanding pulmonary valve system (Venus P Valve) for rehabilitation of the RVOT in patients with chronic severe PR. METHODS: Patients with native RVOT and severe PR were selected on a case-by-case basis as part of early clinical experience with this valve. Patient demographics and pre-procedural, intra-procedural, and follow-up data were reviewed. RESULTS: Five patients (four females) with a mean weight of 54.8 ± 11.4 kg were selected for attempted valve deployment. Patients were either NYHA class II (n = 3) or class III (n = 2) at baseline. PR was grade 4 in all cases with mean right ventricular end-diastolic volumes of 155.0 ± 16.6 ml/m(2) on cardiac MRI. Mean minimum "annular" diameter on transthoracic echocardiogram was 22.8 ± 2.5 mm and mean RVOT diameter was 31.8 ± 5.1 mm. Uncomplicated successful valve delivery was achieved in all five cases with a mean fluoroscopy time of 22.8 ± 8.8 min. Valve sizes used were 26 (n = 3), 30 (n = 1), and 32 (n = 1). Mean pulmonary artery diastolic pressure increased from 3 ± 3.7 mm Hg to 9.9 ± 6.9 mm Hg (P = 0.06). On mean follow-up of 3.4 ± 2.5 months PR grade is 0 (n = 3) or 1 (n = 2) in all cases. NYHA class has improved at least one class in all cases and right ventricular volumes assessed by echocardiography have normalized in all three patients with follow-up to 3 months. CONCLUSIONS: The Venus P Valve provides excellent short-term pulmonary valvar function in the native RVOT of patients with surgically induced chronic severe PR.

Reverse Szabo technique for stenting a single major aorto-pulmonary collateral vessel in pulmonary atresia with ventricular septal defect.

Management of pulmonary atresia with ventricular septal defect (PA-VSD) in the neonatal period presents numerous challenges. Endovascular stenting of the ductus arteriosus or of a collateral vessel in ductal-dependent pulmonary circulation as an alternative to the Blalock-Taussig (BT) shunt has become increasingly popular in the last decades. The utilization of the reverse Szabo (anchor-wire) technique for single collateral vessel stenting in a case of PA-VSD is described.