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We intend to describe an entirely transcatheter management pathway for patients with pulmonary atresia with intact ventricular septum (PA/IVS).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.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.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.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.

Through the implementation of the free website/smartphone application Sketchfab, we have found a simple means to introduce 3D visual tools into the chemistry classroom. Sketchfab stores 3D models and animations with free cross-platform accessibility. Blender is a free 3D modeling tool that has been used to prepare models and animations for dissemination via Sketchfab. By combining these two tools, we have created and are currently maintaining an ever-growing free library of interactive 3D models on various chemistry topics. Through the Sketchfab smartphone application, these models are viewable in augmented reality on most devices. Topics include molecular motion, isomerism, molecular orbital theory, chirality, and polymerization.

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.

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 m(2) (rest), 101.1±20.7 mL/beat m(2), (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.

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.We reviewed our experience using IVUS as an adjunctive tool to diagnose lesions and assess intervention in pediatric patients during cardiac catheterization.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.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.

We report a multicenter experience with simultaneous right ventricular outflow tract (RVOT) stenting and transcatheter pulmonary valve implantation using the Melody valve (Medtronic).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.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.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.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.

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 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.

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).

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.

Pulmonary arteriovenous malformations (AVMs) can be a complication of certain postoperative Fontan patients whose hepatic venous blood return is not distributed evenly to both lungs. A ten-year-old female, who had previously undergone staged single ventricle palliation for complex congenital heart disease, underwent a Fontan revision due to significant left-sided pulmonary AVMs and increasing arterial oxygen desaturation. The combination of four-dimensional flow cardiac magnetic resonance imaging and three-dimensional printing enabled presurgical planning for a Fontan takedown and diversion of hepatic venous flow to the azygous vein that resulted in significant clinical improvement.

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.

Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent. She required restenting of the PDA and pulmonary artery banding prior to arterial switch procedure. At 16 months of age, the patient successfully underwent arterial switch procedure without complication. This report demonstrates the feasibility of an arterial switch operation along with long-term follow-up of this rare condition.

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.

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.

Background: Ductal-dependent cyanotic newborns require a secure source of pulmonary blood flow. There has been a recent migration to selective ductal (patent ductus arteriosus [PDA]) stenting for some of these children. Universal (nonselective) ductal stenting for all infants with ductal-dependent pulmonary blood flow is controversial. We examine outcomes from a single center with this practice change. Methods: We compare outcomes of all ductal-dependent pulmonary blood flow infants (2013–2020 [January–June]) in the following treatment eras: Era 1 (selective PDA stenting; 2013–2017) or Era 2 (universal PDA stenting; 2018–2020 [January–June]). Results: Eighty-eight patients (Blalock-Taussig shunt, n=41; PDA stent, n=47) met inclusion criteria. In Era 1, most received Blalock-Taussig shunt (62% [41/66]). In Era 2, all received PDA stents (100% [22/22]). There were more females in Era 2, but otherwise no demographic differences between eras. There were no differences in mortality, treatment failures, complications, or reinterventions between eras. Postprocedure length of stay was shorter in Era 2 (8 versus 22 days, P =0.02). There were less surgical revisions for PDA stent patients (2% versus 20%, P =0.02). Postprocedure recovery surrogate end points favored Era 2 and PDA stenting. Additional analysis revealed PDA stent (compared with Blalock-Taussig shunt) patients had shorter post-procedure (10 versus 29 days, P ≤0.001) length of stay and more symmetrical branch pulmonary arteries (0.9 versus 0.7, P =0.001) at subsequent surgery. Conclusions: PDA stenting for almost all ductal dependent cyanotic newborns can be safe and effective and may have lower morbidity than selective PDA stenting.

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.

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.

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.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.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.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.

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.), Graphical abstract, Limited arterial vascular access precluded necessary transcatheter intervention in a 22-year-old woman with repaired interrupted aortic arch type B…

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.

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.

Visual representations play an important role in chemistry. They provide opportunities to engage students actively in individual and collaborative sense-making of complex concepts. In this chapter, we review research on learning with visual representations in individual and collaborative activities. We then describe how we implemented the lessons learned from this research to design activities that combine individual and collaborative sense-making activities with visual representations. We provide concrete examples of these activities for an advanced general chemistry course. To evaluate the effectiveness of these activities, we conducted a quasi-experiment as part of this course. Our results suggest that individual sense-making in self-study activities prior to collaborative sense-making activities in class yield higher learning outcomes than traditional lecture-centric instruction.

Background Dacron tube grafts have been used in the surgical management of cardiovascular disease since the 1970s. Complications at the site of the anastomosis have been well described. Non-anastomotic failure is far less common. We present a series of four patients who presented with complications of non-anastomotic failure of woven Dacron tube grafts. Methods A retrospective chart review of four patients who presented to our institution between March 2014 and March 2017 with clinical complications of a Dacron tube graft was conducted. Results All four patients underwent a staged surgical repair for an interrupted aortic arch between the years of 1988 and 2001. All four patients underwent revision of their original interposition graft (Gore-Tex, W.L. Gore & Associates, Flagstaff, AZ) with implantation of a Hemashield woven Dacron tube graft (Maquet, Rastatt, Germany). From 13 to 22 years postimplant of the Dacron tube graft, all patients presented with symptoms or clinical evidence of primary graft failure. Two patients underwent urgent surgical intervention and did not survive. One patient underwent attempted surgical intervention, which was aborted in the setting of profuse bleeding, and ultimately had an endovascular rescue of the tube graft with a Zenith Alpha endograft (Cook Medical, Bloomington, IN). One patient underwent elective endovascular intervention prior to onset of symptoms. Conclusion Non-anastomotic failure of woven Dacron tube grafts can occur in the thoracic aorta in young adults and may be managed with endovascular techniques.

Author(s): Nguyen, Heajung L; Calfon Press, Marcella A; Aboulhosn, Jamil A; Lin, Jeannette P; Benharash, Peyman; Yang, Eric H

Background Right ventricular outflow tract (RVOT) stents have been used as palliation in patients with severe tetralogy of Fallot (TOF). Radiofrequency perforation of the RVOT has also been described in patients with pulmonary atresia (PA)/ventricular septal defect (VSD). However, RVOT stenting in conjunction with radiofrequency perforation as a means for establishing reliable pulmonary blood flow in patients with PA/VSD has not previously been reported. Objectives Our aim is to report our experience with using perforation of plate-like pulmonary valve atresia combined with stenting of RVOT as an alternative and equally efficacious intervention for infants with PA/VSD, as compared to a surgical pulmonary artery shunt (SPS). Methods A retrospective review of patients with PA/VSD at our institution from Jan 2006 to October 2015 was performed. Results Twenty-seven patients received palliation for PA/VSD. Five (median 2.5 kg, 2.1-4.1 kg) underwent RVOT stenting and 22 (3.5 kg, 2.1-4.6 kg) underwent surgical SPS. Of the patients who received an RVOT stent, 3 underwent guidewire perforation and balloon dilation of the plate-like pulmonary valve, and 2 underwent radiofrequency perforation of the pulmonary plate, followed by balloon dilation. Postprocedure saturations averaged 93% in the stent group and 89% in the shunt group. One of the patients who underwent RVOT stenting required additional stenting of the PDA for isolation of the LPA, but none required re-intervention. Four patients in the SPS cohort required re-intervention (18%). Two required stenting of the shunt, and 2 required surgical revision of the shunt. There was one death in the stent group, however this was secondary to withdrawal of support given an underlying genetic diagnosis (trisomy 13). There was one fatality in the SPS cohort prior to hospital discharge. Conclusions Perforation followed by RVOT stenting may be a safe and effective alternative to surgical SPS in infants with PA/VSD.

Much evidence shows that instruction that actively engages students with learning materials is more effective than traditional, lecture-centric instruction. These “active learning” models comprise an extremely heterogeneous set of instructional methods: they often include collaborative activities, flipped classrooms, or a combination of both. To date, it is unclear whether active learning is more effective if it combines collaboration support with flipped classroom methods. We conducted a quasi-experiment as part of an advanced general chemistry course with 413 undergraduate students. We tested whether active learning is more effective than traditional instruction if it includes collaboration support only or a combination of collaboration support and flipped classrooms. Further, we explored effects on students’ attitude. Our results show that only the combination of collaboration support and flipped classroom methods led to significantly higher learning outcomes than traditional instruction. Furthermore, ...

Background In the care of patients with congenital heart disease, percutaneous interventional treatments have supplanted many surgical approaches for simple lesions, such as atrial septal defect. By contrast, complex congenital heart defects continue to require open-heart surgery. In single-ventricle patients, a staged approach is employed, which requires multiple open-heart surgeries and significant attendant morbidity and mortality. A nonsurgical transcatheter alternative would be attractive. Objectives The authors sought to show the feasibility of catheter-only, closed-chest, large-vessel anastomosis (superior vena cava and pulmonary artery [PA] or bidirectional Glenn operation equivalent) in a patient. Methods In preclinical testing over a decade, the authors developed the techniques and technology needed for nonsurgical crossing from a donor (superior vena cava) to a recipient (PA) vessel and endovascular stent-based anastomosis of those blood vessels. The authors undertook this transcatheter approach for an adult with untreated congenital heart disease with severe cyanosis and significant surgical risk. They rehearsed the procedure step by step using contrast-enhanced cardiac computed tomography and a patient-specific 3-dimensional printed heart model. Results The authors describe a first-in-human, fully percutaneous superior cavopulmonary anastomosis (bidirectional Glenn operation equivalent). The patient, a 35-year-old woman, was homebound due to dyspnea and worsening cyanosis. She was diagnosed with functional single ventricle and very limited pulmonary blood flow. The heart team believed surgical palliation conferred high operative risk due to the patient’s complete condition. With the percutaneous procedure, the patient recovered uneventfully and remained improved clinically after 6 months. Conclusions This procedure may provide a viable alternative to one of the foundational open-heart surgeries currently performed to treat single-ventricle congenital heart disease.

The phenomenon of coronary steal is well known in the setting of HLHS (Hypoplastic Left Heart Syndrome) early after the Classical Norwood Operation. We report a rare case of an infant with HLHS [Severe Aortic Stenosis (AS), Mitral Stenosis (MS) and small Left Ventricle (LV)], who developed aortic regurgitation and presumed coronary steal late after the Sano Modification of the Norwood Procedure. Coronary steal developed secondary to progressive aortic and mitral regurgitation and resulted in poor right ventricular function and severe tricuspid regurgitation. We describe a novel interventional approach for obliteration of the LV cavity by using hydrogel coils. LV obliteration eliminated the presumed steal and resulted in improvement in ventricular function, tricuspid regurgitation and clinical course.

Undergraduate STEM instruction increasingly uses educational technologies to support problem-solving activities. Educational technologies offer two key features that may make them particularly effective. First, most problem-solving activities involve multiple visual representations, and many students have difficulties in understanding, constructing, and connecting these representations. Educational technologies can provide adaptive support that helps students make sense of visual representations. Second, many problems with visual representations involve collaboration. However, students often do not collaborate effectively. Educational technologies can provide collaboration scripts that adaptively react to student actions to prompt them to engage in specific effective collaborative behaviors. These observations lead to the hypothesis we tested: that an adaptive collaboration script enhances students' learning of content knowledge from visual representations. We conducted a quasi-experiment with 61 undergraduate students in an introductory chemistry course. A control condition worked on a traditional worksheet that asked students to collaboratively make sense of connections among multiple visual representations. An experimental condition worked on the same problems embedded in an educational technology that provided an adaptive collaboration script. The experimental condition showed significantly higher learning gains on a transfer test immediately after the intervention and on complex concepts on a midterm exam three weeks later. Educational technologies can enhance collaborative learning with visual representations.A quasi-experiment with 61 students tests effects of an adaptive collaboration script.The collaboration script yields higher learning outcomes than traditional instruction.The effects were more pronounced for learning of complex concepts.Adaptive collaboration scripts can help students connect visual representations.

Despite few institutions stenting the ascending aorta, it has been discouraged because of the proximity of the aortic valve, the coronary artery orifices, and the aortic arch branches. We describe a small case series of patients having acquired stenosis of the ascending that was relieved successfully by stenting. © 2017 Wiley Periodicals, Inc.

Polymers are currently underrepresented in the undergraduate chemistry curriculum, especially in general chemistry. Nevertheless, there are good reasons and ways to include them in the two-semester (three-quarter) general chemistry course sequence. We describe both a course and a textbook in which polymers and biopolymers are integrated into the general chemistry curriculum.

Transcatheter electrosurgery is used for planned caval-aortic access and to facilitate transcatheter mitral and aortic valve implantation [(1,2)][1]. It has been used for percutaneous cavopulmonary shunt [(3)][2]. This technique can also be used for unplanned clinical rescue; we describe re

Background Adverse events from Melody valve implantation may be catastrophic. To date a role for three dimensional rotational angiography of the aortic root (3DRAA) during Melody valve implantation has not been established. Objectives To describe the role of 3DRAA in the assessment of Melody valve candidacy and to demonstrate that it may improve outcomes. Methods All patients who underwent cardiac catheterisation for Melody valve implantation and 3DRAA between August 2013 and February 2015 were reviewed. Results 31 patients had 3DRAA with balloon sizing. Ten were deemed not Melody candidates (5 coronary compression, 2 aortic root distortion with cusp flattening, 2 RVOT was too large, and 1 had complex branch stenosis and a short landing zone). Of the 21 patients who were Melody candidates, 12 had conduits, 6 prosthetic valves and 3 native RVOTs. In patients with conduits, the technique of stenting the conduit prior to dilation was used after measuring the distance between the conduit and the coronary arteries on 3DRAA. In the Melody patients, we had 100% procedural success and no serious adverse events (coronary compression, tears, stent fracture or endocarditis). Conclusion As a tool for case selection, 3DRAA may facilitate higher procedural success and decreased risk of serious adverse events. Furthermore, 3D rotational angiography allows stenting of the conduit prior to dilation, which may prevent tears and possibly endocarditis.

Pulmonary vein stenosis (PVS) in children is a progressive disease with poor outcomes. Surgical and catheter-based interventions can provide immediate relief of stenosis, but re-stenosis is common, often progressive, and can be fatal. We pursued serial balloon angioplasty (BA) to treat patients with recurrent PVS at our center. We performed a retrospective chart review and data analysis of all children (age

Objective To evaluate whether preprocedural transthoracic echocardiography (TTE) can be used to predict Amplatzer septal occluder (ASO) size for device closure of atrial septal defect (ASD). Design Retrospective review of patients who underwent ASD device closure at our institution between August 2006 and August 2013 was performed. Patients with complex congenital heart disease, devices other than the ASO, multiple devices, or inadequate TTE images were excluded. Those who had transesophageal echocardiography (TEE)-guided device placement were evaluated. A blinded observer reviewed their preprocedural TTE images and applied a scaled formula to predict device size. Results A total of 186 patients underwent ASO placement during the study period, 87 had TEE guidance, of which 45 met inclusion criteria. The mean predicted device size by the scaled formula was 18.0 ± 5.11 mm, compared to the mean implanted device size of 18.8 ± 5.22 mm. The mean absolute difference between each predicted and final deployed device size was 1.44 mm with 95% CI [1.08, 1.81]. The Pearson correlation showed that the predicted device size had a positive correlation coefficient of 0.94. Conclusion Preprocedural TTE assessment of ASD size using a scaling formula in patients with adequate TTE windows can accurately predict ASO device size and aid in device selection.

Objective Pulmonary insufficiency following balloon valvuloplasty for pulmonary valve stenosis has been shown to result in right ventricular dilation, which may necessitate pulmonary valve replacement. This study investigates the consequences of employing a more conservative balloon:annulus ratio of ≤1.2 against the currently used ratios of >1.2. The aim of the study was to evaluate whether a more conservative balloon:annulus ratio would maintain procedural success with adequate relief of the pulmonary valve gradient, while minimizing pulmonic insufficiency. Design Procedural data were collected on cases considered for balloon valvuloplasty for congenital pulmonic stenosis at Rady Children's Hospital, San Diego between March 31, 2003 and April 9, 2014. The study includes 98 patients whose median age at the time of procedure was 3.6 months (range: 1 day to 271 months); median follow-up was 15.2 months (range 24 days to 106 months). The subjects were divided into two groups based on their balloon:annulus ratio: ≤1.2 (n = 67) and >1.2 (n = 31). Results The difference in pulmonary gradient reduction—the marker of procedural success—was not significant between the two groups (P = .33). Although both groups had an increase in pulmonary valve insufficiency from baseline, the ≤1.2 group had significantly less insufficiency compared to the >1.2 group (P = .008). Reintervention rates were not statistically significant between both groups (P = .89). Conclusion Performing pulmonary balloon valvuloplasty with a balloon:annulus ratio of ≤1.2 produces significantly less pulmonary insufficiency than a ratio of >1.2 without reducing procedure efficacy.

Background: Overseeing proper insulin initiation and titration remains a challenging task in diabetes care. Recent advances in mobile technology have enabled new models of collaborative care between patients and healthcare providers (HCPs). We hypothesized that the adoption of such technology could help individuals starting basal insulin achieve better glycemic control compared with standard clinical practice. Materials and Methods: This was a 12 ± 2-week randomized controlled study with 40 individuals with type 2 diabetes who were starting basal insulin due to poor glycemic control. The control group (n = 20) received standard face-to-face care and phone follow-up as needed in a tertiary center, whereas the intervention group (n = 20) received care through the cloud-based diabetes management program where regular communications about glycemic control and insulin doses were conducted via patient self-tracking tools, shared decision-making interfaces, secure text messages, and virtual visits (audio, video, and shared screen control) instead of office visits. Results: By intention-to-treat analysis, the intervention group achieved a greater hemoglobin A1c decline compared with the control group (3.2 ± 1.5% vs. 2.0% ± 2.0%; P = 0.048). The Diabetes Treatment Satisfaction Questionnaire showed a significant improvement in the intervention group compared with the control group (an increase of 10.1 ± 11.7 vs. 2.1 ± 6.5 points; P = 0.01). HCPs spent less time with patients in the intervention group compared with those in the control group (65.9 min per subject vs. 81.6 min per subject). However, the intervention group required additional training time to use the mobile device. Conclusions: Mobile health technology could be an effective tool in sharing data, enhancing communication, and improving glycemic control while enabling collaborative decision making in diabetes care.

Background— As US health care increasingly focuses on outcomes as a means for quantifying quality, there is a growing demand for risk models that can account for the variability of patients treated at different hospitals so that equitable comparisons between institutions can be made. We sought to apply aspects of prior risk-standardization methodology to begin development of a risk-standardization tool for the National Cardiovascular Data Registry (NCDR) IMPACT (Improving Pediatric and Adult Congenital Treatment) Registry. Methods and Results— Using IMPACT, we identified all patients undergoing diagnostic or interventional cardiac catheterization between January 2011 and March 2013. Multivariable hierarchical logistic regression was used to identify patient and procedural characteristics predictive of experiencing a major adverse event after cardiac catheterization. A total of 19 608 cardiac catheterizations were performed between January 2011 and March 2013. Among all cases, a major adverse event occurred in 378 of all cases (1.9%). After multivariable adjustment, 8 variables were identified as critical for risk standardization: patient age, renal insufficiency, single-ventricle physiology, procedure-type risk group, low systemic saturation, low mixed venous saturation, elevated systemic ventricular end-diastolic pressure, and elevated main pulmonary artery pressures. The model had good discrimination (C statistic, 0.70), confirmed by bootstrap validation (validation C statistic, 0.69). Conclusions— Using prior risk-standardization efforts as a foundation, we developed and internally validated a model to predict the occurrence of a major adverse event after cardiac catheterization for congenital heart disease. Future efforts should be directed toward further refinement of the model variables within this large, multicenter data set.

ObjectivesThe objective of this study was to report procedural characteristics and adverse events on the data collected in the IMproving Paediatric and Adult Congenital Treatment registry.BackgroundThe IMproving Paediatric and Adult Congenital Treatment– registry is a catheterisation registry focussed on paediatric and adult patients with congenital heart disease who are undergoing diagnostic catheterisations and catheter-based interventions. This study reports procedural characteristics and adverse events of patients who have undergone selected catheterisation procedures from January, 2011 to June, 2013.MethodsDemographic, clinical, procedural, and institutional data elements were collected at participating centres and entered via either a web-based platform or software provided by the American College of Cardiology-certified vendors, and were collected in a secure, centralised database. For the purpose of this study, procedures that were not classified as one of the ‘core’ IMproving Paediatric and Adult Congenital Treatment procedures originally chosen for additional data collection were identified and selected for further data analysis.ResultsDuring the time frame of data collection, a total of 8021 cases were classified as other procedures and/or multiple procedures. The most commonly performed case types – isolated or in combination with other procedures – were right ventricular biopsy in 3433 (42.8%), conduit/MPA interventions in 979 (12.3%), and systemic pulmonary artery collateral occlusion in 601 (7.5%). For the whole cohort, adverse events of any severity occurred in 957 (12.0%) cases, whereas major adverse events occurred in 113 (1.4%) cases; six patients (0.1%) died in the catheterisation laboratory.ConclusionsThe IMproving Paediatric and Adult Congenital Treatment registry has provided important data on the frequency and spectrum of cardiac catheterisation procedures performed in the present era. For many procedures, more data and work are needed to identify more subtle differences between case categories, especially as it relates to the incidence of major adverse events, and to further develop a risk-adjustment methodology to allow equitable comparisons among institutions.

The National Cardiovascular Data Registry (NCDR) of the American College of Cardiology consists of 10 national programs designed to measure quality of care for high-impact cardiovascular conditions and procedures. The NCDR provides feedback with benchmarking of a broad range of quality metrics to