Transcatheter Hepatic Conduit-Azygous Vein Connection Reduces Pulmonary Arteriovenous Malformations in a Cyanotic Fontan Patient.

The authors report a closed-chest, transcatheter large-vessel connection (hepatic conduit to azygous vein) to reverse pulmonary arteriovenous malformations in a 10-year-old patient after Fontan for heterotaxy/interrupted inferior vena cava, with an increase in oxygen saturation from 78% to 96%. Computational fluid dynamics estimated a 14-fold increase in hepatic blood flow to the left pulmonary artery (from 1.3% to 14%). (Level of Difficulty: Advanced.).

Pulmonary Atresia With Intact Ventricular Septum: From Radiofrequency Perforation to Transcatheter Pulmonary Valve.

OBJECTIVES: We intend to describe an entirely transcatheter management pathway for patients with pulmonary atresia with intact ventricular septum (PA/IVS). BACKGROUND: PA/IVS is a rare cyanotic congenital heart lesion traditionally palliated with multiple procedures and surgeries. Entirely non-surgical transcatheter management from infancy to adulthood may be possible; however, the pathway from neonatal radiofrequency (RF) pulmonary valve perforation to later transcatheter pulmonary valve replacement (TPV) is not well described. METHODS: This retrospective study was performed at a pediatric cardiac center between 2007-2018. All patients with PA/IVS who were managed exclusively with catheterization-based interventions as neonates (RF perforation, pulmonary valvuloplasty, or ductal stenting) were analyzed. Demographic, procedural and clinical data were collected. RESULTS: Fifteen patients had exclusively catheterization-based RV decompression as neonates, 7 of whom did not require subsequent surgery. Six patients required a right ventricular outflow tract (RVOT) augmentation later in life; all were born before 2013. All 6 later had a TPV placed. Two of the patients underwent a surgical Glenn shunt alone. Of the 7 patients that never had surgery, 3 have since undergone a TPV, and 4 are awaiting candidacy for TPV placement. No patients with PA/IVS have had an RVOT augmentation at our institution since 2012. CONCLUSIONS: Patients with PA/IVS who undergo catheterization-based neonatal interventions can make it to TPV without requiring surgery. At our institution, there has been a shift in management over the last 8 years favoring entirely non-surgical transcatheter management from infancy to adulthood.

Selective Valve Removal for Melody Valve Endocarditis: Practice Variations in a Multicenter Experience.

Guidelines for management of Melody transcatheter pulmonary valve (TPV) infective endocarditis (IE) are lacking. We aimed to identify factors associated with surgical valve removal versus antimicrobial therapy in Melody TPV IE. Multicenter retrospective analysis of all patients receiving Melody TPV from 10/2010 to 3/2019 was performed to identify cases of IE. Surgical explants versus non-surgical cases were compared. Of the 663 Melody TPV implants, there were 66 cases of IE in 59 patients (59/663, 8.8%). 39/66 (59%) were treated with IV antimicrobials and 27/66(41%) underwent valve explantation. 26/59 patients (44%) were treated medically without explantation or recurrence with average follow-up time of 3.5 years (range:1-9). 32% of Streptococcus cases, 53% of MSSA, and all MRSA cases were explanted. 2 of the 4 deaths had MSSA. CART analysis demonstrated two important parameters associated with explantation: a peak echo gradient ≥ 47 mmHg at IE diagnosis(OR 10.6, p < 0.001) and a peak echo gradient increase of > 24 mmHg compared to baseline (OR 6.7, p = 0.01). Rates of explantation varied by institution (27 to 64%). In our multicenter experience, 44% of patients with Melody IE were successfully medically treated without valve explantation or recurrence. The degree of valve stenosis at time of IE diagnosis was strongly associated with explantation. Rates of explantation varied significantly among the institutions.

Purpose-Built Transcatheter Cavopulmonary Anastomosis Device Requirements: Multi-Modality Imaging Evaluation.

BACKGROUND/PURPOSE: Patients with a functional single ventricle undergo multiple, palliative open-heart surgeries. This includes a superior cavopulmonary anastomosis or bidirectional Glenn shunt. A less-invasive transcatheter approach may reduce morbidity. METHODS/MATERIALS: We analyzed pre-Glenn X-ray contrast angiography (XA), cardiac computed tomography (CT), and cardiac magnetic resonance (CMR) studies. RESULTS: Over an eleven-year period (1/2007 - 6/2017), 139 Glenn surgeries were performed at our institution. The typical age range at surgery was 59 - 371 days (median = 163; IQR = 138 - 203). Eight-nine XA, ten CT, and ten CMR studies obtained from these patients were analyzed. Cephalad SVC measurements (millimeters) were 7.3 ± 1.7 (XA), 7.7 ± 1.6 (CT) and 6.9 ± 1.8 (CMR). RPA measurements were 7.3 ± 1.9 (XA), 7.4 ± 1.6 (CT) and 6.6 ± 1.9 (CMR). Potential device lengths were 10.9 ± 6 - 17.4 ± 6.4 (XA), 10.1 ± 2.1 - 17.7 ± 2.4 (CT) and 17.3 ± 4. - 23.7 ± 5.5 (CMR). SVC-RPA angle (degrees) was 132.9 ± 13.2 (CT) and 140 ± 10.2 (MRI). Image quality of all CT (100%), almost all XA (SVC 100%, RPA 99%), and most MRI (SVC 80%, RPA 90%) were deemed sufficient. Parametric modeling virtual fit device with 10 mm diameter and 20 - 25 mm length was ideal. CONCLUSIONS: Ideal transcatheter cavopulmonary shunt device for the typical patient would be 10 mm in diameter and 20-25 mm in length.

Intravascular Ultrasound (IVUS) Provides the Filling for the Angiogram's Crust: Benefits of IVUS in Pediatric Interventional Cardiology.

BACKGROUND: Intravascular ultrasound (IVUS) is a catheter-based imaging modality that generates cross-sectional views of vessel walls and lumens. This technique is used in adult interventional and vascular surgeries to guide the management of coronary artery and peripheral arterial disease. IVUS has been described as superior to angiography in providing data about lesions of interest, including degree of vessel stenosis and stent apposition following intervention. IVUS use to guide transcatheter management of congenital heart disease is limited. OBJECTIVE: We reviewed our experience using IVUS as an adjunctive tool to diagnose lesions and assess intervention in pediatric patients during cardiac catheterization. METHODS AND RESULTS: A retrospective chart review of all pediatric patients who underwent IVUS during cardiac catheterization to evaluate the cross-sectional lumen of non-coronary vessel(s) at Rady Children's Hospital from January 2018 to December 2019 was performed. Median patient age was 637 days (range, 44-4328 days), with mean weight of 12.1 ± 9 kg. Twenty-six vessels were interrogated with IVUS (pulmonary venous stenosis [n = 8], coarctation [n = 5], branch pulmonary artery stenosis [n = 6], systemic shunts and conduits [n = 3], and other peripheral vasculature [n = 4]). IVUS added value in all cases (100%). We found that IVUS guided the intervention in 88% of procedures and defined the endpoint in 62% of transcatheter interventions. There were no IVUS-related complications. CONCLUSIONS: IVUS enhanced our diagnostic interpretation and identified occult lesions not visualized by angiography. IVUS was valuable in guiding and defining the endpoints of these interventions.

Expanded Use of the One-Step Technique for Simultaneous Landing Zone Stenting and Placement of the Melody Transcatheter Pulmonary Valve.

OBJECTIVES: We report a multicenter experience with simultaneous right ventricular outflow tract (RVOT) stenting and transcatheter pulmonary valve implantation using the Melody valve (Medtronic). BACKGROUND: Prestenting the RVOT before Melody valve implantation is now the standard of care. Prestenting is usually performed as a separate step. The "one-step" technique for simultaneous landing zone stenting and Melody delivery was previously reported using only Max LD stents (Medtronic). We report a multicenter experience of simultaneous stenting and Melody implantation using multiple stent types in combination. METHODS: This retrospective cohort study includes 33 patients from 3 centers who underwent simultaneous stenting and Melody valve implantation between 2017 and 2020. Key variables were compared with 31 patients from the same centers who underwent standard (non-simultaneous) prestenting followed by Melody implantation during the same time frame. RESULTS: The 2 groups were similar in terms of age, weight, sex, and total procedure time. The 2 groups had similar clinical results and safety profiles, with no difference between the postimplantation right ventricle (RV) to pulmonary artery systolic pressure gradient, RV to aortic pressure ratio, and complication rate. The simultaneous group had lower radiation exposure as measured by dose area product. Up to 3 stents were safely placed simultaneously with a Melody valve. CONCLUSIONS: Simultaneous RVOT stenting and Melody valve implantation can safely be used to place up to 3 stents outside a Melody valve. This approach can simplify the catheterization procedure and potentially reduce radiation dose.

Patent Ductus Arteriosus Stenting for All Ductal-Dependent Cyanotic Infants: Waning Use of Blalock-Taussig Shunts.

[Figure: see text].

Infants with pulmonary atresia intact ventricular septum who require balloon atrial septostomy have significantly higher 18-month mortality.

INTRODUCTION: Many newborns with pulmonary atresia/intact ventricular septum require intervention to establish pulmonary flow and sufficient cardiac output. The resulting haemodynamic changes are not well characterised and may have unintended consequences. METHODS: This is a 30-year (1988-2018) retrospective study of patients with pulmonary atresia intact ventricular septum. RESULTS: Eighty-nine patients were included, and median follow-up was 8 years. Fifty-five per cent had coronary sinusoids and 27% had right ventricular-dependent coronary circulation. Most patients were managed with surgical aortopulmonary or modified Blalock-Taussig shunt (73%), and 12 patients underwent balloon atrial septostomy before surgical intervention. The remaining patients (27%) underwent only transcatheter interventions; 7 required an atrial septostomy and 17 required ductal stentings. All-cause mortality was 10%, most deaths (89%) occurred before 18 months of age. Of these early deaths, 87% required a balloon atrial septostomy and 85% had right ventricular-dependent coronary sinusoids. Eighteen-month mortality was significantly higher for patients who required a balloon atrial septostomy compared to those who did not (36% versus 1.4% p < 0.0001). DISCUSSION: Patients with pulmonary atresia/intact ventricular septum who require balloon atrial septostomy in the newborn period have significantly higher 18-month mortality. Quantifying the mortality difference may help guide prognostication and expectation setting. Infants who had septostomy and a surgical shunt in the newborn period fared better than those who only underwent septostomy (even when accompanied by ductal stenting). For infants with right ventricular-dependent circulation, atrial septostomy should only be performed on an urgent or emergent basis and these patients should be considered for early surgical intervention and neonatal transplant.

Comparison of the investigational device exemption and post-approval trials of the Melody transcatheter pulmonary valve.

OBJECTIVE: We compared 5-year outcomes of transcatheter pulmonary valve (TPV) replacement with the Melody TPV in the post-approval study (PAS) and the investigational device exemption (IDE) trial. BACKGROUND: As a condition of approval of the Melody TPV after the IDE trial, the Food and Drug Administration required that a PAS be conducted to evaluate outcomes of TPV replacement in a "real-world" environment. The 5-year outcomes of the PAS have not been published, and the IDE and PAS trials have not been compared. METHODS: The cohorts comprised all patients catheterized and implanted at 5 IDE sites and 10 PAS sites. Differences in trial protocols were detailed. Time-related outcomes and valve-related adverse events were compared between the two trials with Kaplan-Meier curves and log-rank testing. RESULTS: 167 patients (median age, 19 years) were catheterized and 150 underwent TPV replacement in the IDE trial; 121 were catheterized (median age, 17 years) and 100 implanted in the PAS. Freedom from hemodynamic dysfunction (p = .61) or any reintervention (p = .74) over time did not differ between trials. Freedom from stent fracture (p = .003) and transcatheter reintervention (p = .010) were longer in PAS, whereas freedom from explant (p = .020) and TPV endocarditis (p = .007) were shorter. Clinically important adverse events (AEs) were reported in 14% of PAS and 7.2% of IDE patients (p = .056); the incidence of any particular event was low in both. CONCLUSIONS: Hemodynamic and time-related outcomes in the PAS and IDE trials were generally similar, confirming the effectiveness of the Melody TPV with real-world providers. There were few significant complications and limited power to identify important differences in AEs. The lack of major differences in outcomes between the two studies questions the usefulness of mandated costly post-approval studies as part of the regulatory process for Class III medical devices.

Transjugular liver biopsy for Fontan associated liver disease surveillance: Technique, outcomes and hemodynamic correlation.

BACKGROUND: Fontan associated liver disease (FALD) is attributed to the limitations of the Fontan circulation, resulting in congestive hepatopathy. The technique and outcomes of transjugular liver biopsies (TJLB) in Fontan patients warrant definition as part of a rigorous FALD surveillance program. METHOD: Four year review of patients with Fontan physiology who underwent a TJLB during surveillance catheterizations. Biopsy site, technique, histology, angiography, hemodynamics, and complications were reviewed to assess correlation of biopsy scores with simultaneously obtained catheterization hemodynamics. RESULTS: 125 patients with a TJLB from 10/1/14 to 5/1/18. Median age 17 years (2-50.5). Technical success 100% (125/125), all samples diagnostic. 17% (21) accessed via the left internal jugular vein, secondary to right IJ occlusion or Heterotaxy syndrome. No patients had superior compartment obstruction preventing transjugular approach. 3.2% complication rate (4/125). Complications were early in the experience, including capsular perforation (2), renal hematoma (1) and hemobilia (1), all without long-term effect and all avoidable. After standardized entry/exit angiography was adopted, no further complications noted. There is a significant correlation between the newly described modified Ishak congestive hepatic fibrosis (ICHF) score with mean Fontan pressure, time from Fontan and cardiac index. CONCLUSIONS: TJLB is an alternate method for obtaining critical FALD surveillance data, with lower complication rates that traditional techniques. Vascular anomalies in Fontan physiology appear common and warrant pre-biopsy assessment. There is a significant correlation between biopsy score, time from Fontan, mean Fontan pressure and cardiac index.

An Elusive Prize: Transcutaneous Near InfraRed Spectroscopy (NIRS) Monitoring of the Liver.

Introduction: We postulate a relationship between a transcutaneous hepatic NIRS measurement and a directly obtained hepatic vein saturation. If true, hepatic NIRS monitoring (in conjunction with the current dual-site cerebral-renal NIRS paradigm) might increase the sensitivity for detecting shock since regional oxygen delivery changes in the splanchnic circulation before the kidney or brain. We explored a reliable technique for hepatic NIRS monitoring as a prelude to rigorously testing this hypothesis. This proof-of-concept study aimed to validate hepatic NIRS monitoring by comparing hepatic NIRS measurements to direct hepatic vein samples obtained during cardiac catheterization. Method: IRB-approved prospective pilot study of hepatic NIRS monitoring involving 10 patients without liver disease who were already undergoing elective cardiac catheterization. We placed a NIRS monitor on the skin overlying liver during catheterization. Direct measurement of hepatic vein oxygen saturation during the case compared with simultaneous hepatic NIRS measurement. Results: There was no correlation between the Hepatic NIRS values and the directly measured hepatic vein saturation (R = -0.035; P = 0.9238). However, the Hepatic NIRS values correlated with the cardiac output (R = 0.808; P = 0.0047), the systolic arterial blood pressure (R = 0.739; P = 0.0146), and the diastolic arterial blood pressure (R = 0.7548; P = 0.0116). Conclusions: Using the technique described, hepatic NIRS does not correlate well with the hepatic vein saturation. Further optimization of the technique might provide a better measurement. Hepatic NIRS does correlate with cardiac output and thus may still provide a valuable additional piece of hemodynamic information when combined with other non-invasive monitoring.

Serial Dilation of Low-Profile Stents Delivered in the Aorta and Pulmonary Arteries in Pediatric Patients Leads to Spontaneous Fractures but Not to Adverse Events.

OBJECTIVES: Low-profile stents placed in pediatric patients with congenital heart disease must be expanded by balloon angioplasty to accommodate patient growth. During the process of serial dilation, some stents may spontaneously fracture. The incidence and safety profile of spontaneous fracture is unclear. We report the performance characteristics and safety profile of a cohort of low-profile, premounted stents placed in the pulmonary arteries and aorta and then serially dilated over time to accommodate patient growth, including incidence of fracture and any adverse events. METHODS: A retrospective chart review was conducted of 25 pediatric patients who underwent 27 stent placements with low-profile, premounted stents from January 2005 to September 2018. RESULTS: Nine stents (33%) sustained a spontaneous fracture. There was no statistically significant association between stent fracture and our variables of interest, ie, patient gender, patient weight at time of original stenting, stent location (aorta vs pulmonary artery), stent type, original diameter of stent, and weight at the time of stent implantation. There was no association between time to spontaneous fracture and the aforementioned variables of interest. The majority of the spontaneous fractures occurred within the first 4 years after stent implantation, and there was no difference in survival between the 3 stent types investigated in our cohort. CONCLUSION: One-third of stents undergoing serial dilation for patient growth fractured spontaneously. Patients with fractured stents were free from significant adverse events in this cohort.

Alternative Access in Congenital Heart Disease.

Limited arterial vascular access precluded necessary transcatheter intervention in a 22-year-old woman with repaired interrupted aortic arch type B. Alternative transcaval vascular access enabled percutaneous therapy. This practice evolution is likely to benefit the growing numbers of adults with congenital heart disease. (Level of Difficulty: Advanced.).

Early experience with the Micro Plug Set for preterm patent ductus arteriosus closure.

OBJECTIVES: We intend to describe early experience using a new, commercially available Micro Plug Set for preterm neonate and infant transcatheter patent ductus arteriosus (PDA) occlusion. BACKGROUND: Transcatheter PDA occlusion in premature neonates and small infants is safe and effective. The procedure is early in its evolution. METHODS: Procedural and short-term outcomes of preterm neonates and infants undergoing transcatheter PDA occlusion with a new, commercially available device were reviewed. RESULTS: Eight preterm neonates and infants born at median 27 weeks gestation (23-36 weeks) underwent transcatheter PDA device closure with the Micro Plug Set. The device is short (2.5 mm) with a range of diameters (3, 4, 5, 6 mm) and delivered through a microcatheter. Procedures were performed at median 41 days of age (12-88 days) and at 1690 g (760-3,310 g). Transvenous PDA device occlusion was performed with fluoroscopic and echocardiography guidance. All procedures were successful with complete PDA occlusion. There were no procedural or short-term adverse events. CONCLUSIONS: Preterm neonate and infant transcatheter PDA device closure with a new, commercially available short and microcatheter delivered device (Micro Plug Set) was safe and effective in a small, early series of patients.

Bronchus compression relieved by patent ductus arteriosus stenting.

BACKGROUND: Patent ductus arteriosus (PDA) stenting is evolving as an alternative to surgical aorto-pulmonary shunts for infants with ductal-dependent pulmonary blood flow. Given anatomical proximity, the PDA can compress the ipsilateral bronchus. We report a case series of four patients with bronchial compression by a tortuous PDA who underwent PDA stenting. METHODS: Our four patients received PDA stents for ductal-dependent pulmonary blood flow despite preprocedure imaging evidence of bronchial compression. We reviewed the cross-sectional chest imaging to assess the degree of bronchial compression and the variables that affect it, namely PDA size, PDA tortuosity, and the anatomical relationship between the compressed bronchus and the PDA. RESULTS: Three out of the four patients had postprocedure imaging, and all showed relief of the previously seen bronchial compression. Post-PDA stenting patients had a smaller and straight PDA with significant lateralization away from the compressed bronchus. None of the four patients developed symptoms of bronchial compression poststenting. CONCLUSIONS: Our study suggests that pre-existing bronchial compression does not preclude PDA stenting. Stent placement in an engorged and tortuous PDA led to significant improvement in pre-existing bronchial compression. Improvement may be attributed to PDA shrinkage, straightening, and lateralization. Further studies are needed to confirm our findings.

Exercise MRI highlights heterogeneity in cardiovascular mechanics among patients with Fontan circulation: proposed protocol for routine evaluation.

Single ventricle physiology and palliation via the Fontan operation lead to a series of cardiovascular changes. In addition, organs such as the kidneys and liver have been shown to experience insults and subsequent injury. This has led to routine surveillance of patients. We present findings from a small cohort of patients that was deeply phenotyped to illustrate the need for comprehensive evaluation. A cohort of four Fontan patients with fairly high cardiovascular function was recruited 5-10 years post-Fontan. Patients underwent a rigorous clinical work-up after which a research MRI scan was performed during which (I) data were obtained during exercise to evaluate changes in stroke volume during supine exercise and (II) magnetic resonance angiograms with phase-contrast images were obtained for computational modeling of flows through the Fontan circulation at rest. Clinical measures were consistent with a fairly homogeneous high function cohort (peak oxygen consumption >20 mL/kg/min, robust response to exercise, peak ventilatory efficiency below levels associated with heart failure, MR-derived ejection fraction >50%). Liver evaluation did not reveal clear signs of cirrhosis or extensive fibrosis. However, we observed considerable variability (27-162%) in the increase in stroke index with exercise [100%±64% increase, 53.9±17.4 mL/beat m2 (rest), 101.1±20.7 mL/beat m2, (exercise)]. Computational flow modeling at rest in two patients also showed marked differences in flow distribution and shear stress. We report marked differences in both changes in stroke index during an exercise MRI protocol as well as computational flow patterns at rest suggesting different compensation strategies may be associated with high functioning Fontan patients. The observed heterogeneity illustrates the need for deep phenotyping to capture patient-specific adaptive mechanisms.

Association between patient age at implant and outcomes after transcatheter pulmonary valve replacement in the multicenter Melody valve trials.

OBJECTIVES: To investigate whether age and valve size at implant contribute to outcomes after Melody transcatheter pulmonary valve replacement (TPVR). BACKGROUND: Patient age and valve size at implant contribute to longevity of surgical pulmonary valves. METHODS: All patients discharged with a Melody valve in the pulmonary position, as part of three prospective Melody valve multicenter studies, comprised the study cohort. Acute and time-related outcomes were analyzed according to age: children (≤12 years), adolescents (13-18 years), young adults (19-29 years), and older adults (≥30 years). RESULTS: Successful Melody valve implantation occurred in 49 children, 107 adolescents, 96 young adults, and 57 older adults. Pediatric patients (≤18 years) were more likely to have TPVR for conduit stenosis than adults (62% vs. 44%); children had the smallest conduits. After TPVR, pediatric and adult patients had similar decreases in right ventricular (RV) size by MRI, but adults had improved percentage predicted peak VO2 (58% preimplant to 64% postimplant, p = .02) and FEV1 (69% pre to 71% post, p = .005). Younger age was associated with shorter freedom from RVOT dysfunction, reintervention, and explant. Children had the shortest freedom from endocarditis (p = .041), but all other groups had 5-year freedom from endocarditis of ≥90%. CONCLUSIONS: Younger age was associated with shorter time to RVOT dysfunction, reintervention, and explant after Melody TPVR. Patients ≥13 years of age were at low risk for endocarditis and explant to 5 years. A better understanding of time-related outcomes by age will aid in the comparison of therapeutic options for TPVR candidates. CLINICAL TRIAL REGISTRATION: https://clinicaltrials.gov/ct2/show/NCT00740870 (NCT00740870), https://clinicaltrials.gov/ct2/show/NCT01186692 (NCT01186692), and https://clinicaltrials.gov/ct2/show/NCT00688571 (NCT00688571).

Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics.

INTRODUCTION: Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics. METHODS: Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic resonance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modified Ishak congestive hepatic fibrosis score. RESULTS: Median age 17.8 years, median time since Fontan 15.2 years. Primary diagnosis and Fontan type were variables, but predominantly LV morphology (30/49), lateral tunnel Fontan (29/49), originally fenestrated (37/49), and 11/49 had a pacemaker. Histologic fibrosis correlated with MRE (R = 0.62, P ≤ .001). Histologic fibrosis and MRE correlated with Fontan pressure (R = 0.38, P = .008 & R = 0.59, P ≤ .001). Morphology of the single ventricle did not correlate with liver fibrosis. The presence of a fenestration resulted in a higher cardiac index (P = .026) but did not resulted in lower liver fibrosis (P = .64). CONCLUSION: Noninvasive tests, such as MRE, may be suitable for longitudinal follow-up in patients with single ventricle physiology. Our data suggest that there is reasonable correlation of MRE liver stiffness with biopsy scoring systems and Fontan pressures. We demonstrated the feasibility of TJLB in the setting of Fontan palliation and demonstrated its correlation with noninvasive measures particularly MRE. We recommend selective use of TJLB when MRE score is >5 KPa or when there are other clinical signs of cirrhosis.

Results of the combined U.S. multicenter postapproval study of the Nit-Occlud PDA device for percutaneous closure of patent ductus arteriosus.

OBJECTIVES: To report the results of the Nit-Occlud PDA prospective postapproval study (PAS) along with a comparison to the results of the pivotal and continued access trials. BACKGROUND: The Nit-Occlud PDA (PFM Medical, Cologne, Germany), a nitinol coil patent ductus arteriosus (PDA) occluder, was approved by the Food and Drug Administration in 2013. METHODS: The PAS enrolled a total of 184 subjects greater than 6 months of age, weighing at least 5 kg, with PDAs less than 4 mm by angiography at 11 centers. Patients were followed prospectively at 2 months, 12 months, and 24 months postprocedure. These outcomes were compared to the 357 subjects enrolled in the pivotal and continued access protocols. Efficacy and safety data were reported. RESULTS: Among 184 subjects enrolled for the PAS between 2014 and 2017, 180 (97.8%) had successful device implantation. After 12 months, 98.7% (150/152) had trivial or no residual shunt by echocardiography and two subjects had only small residual shunts. There were three device embolizations that were all retrieved by snare without clinical consequence. Together with the pivotal and continued access study, 97.4% (449/461) had complete echocardiographic closure at 12 months in 541 enrolled subjects. The composite success was 94.4%. There were no mortalities and no serious device-related adverse events. CONCLUSIONS: The Nit-Occlud PDA is a safe and effective device for closure of a small to moderate sized PDA. There were no serious device-related adverse events in a large cohort of three clinical trials.