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The most significant sequelae of Kawasaki disease (KD) are coronary artery aneurysms, which can lead to risk of future myocardial ischemia. Exercise stress echocardiography allows for non-invasive assessment of myocardial dysfunction. We reviewed our single center experience with exercise stress echocardiography in patients with previous history of KD with coronary aneurysms. We reviewed the records of 53 KD patients who underwent exercise stress echocardiography from 2000 to 2020. Abnormal stress echocardiograms were defined as those showing no increase in biventricular systolic function post-exercise or regional wall motion abnormalities. Computed tomography angiography and cardiac magnetic resonance imaging were reviewed for patients with abnormal stress echocardiograms. Clinical data were reviewed and correlated with stress echocardiogram results. Of the 53 patients, three (5.7%) had an abnormal exercise stress echocardiogram. All three patients were classified as AHA Risk Level 4 or 5 by coronary Z-score (internal dimension normalized for body surface area) and were confirmed to have coronary aneurysms, stenosis, or myocardial tissue perfusion defects on advanced cardiac imaging that could account for the results seen on stress echocardiogram. Exercise stress echocardiography detected signs of myocardial ischemia in a subset of high-risk patients with Kawasaki disease and coronary aneurysms and may be considered as a useful screening tool for this complex patient cohort.

Mitral valve replacement using a Melody valve is a promising solution to the challenge of surgical mitral valve replacement in infants with a hypoplastic annulus. We report the creation of a landing zone in the mitral valve annulus using a Cheatham-Platinum (CP)-covered stent that facilitates Melody valve placement, helps prevent paravalvular leak, minimizes left ventricular outflow tract obstruction, and allows for potential future dilation of the valve.

To determine if patients with congenital heart disease are undergoing laparoscopic surgery requiring abdominal insufflation and to compare the outcomes of these procedures with those who underwent an open surgical approach.This was a retrospective study using the National Inpatient Sample from 2006 to 2014. Individuals with congenital heart disease who underwent at least one of six selected surgical procedures (laparoscopic or open) were included in the study. Subgroup analysis was performed on patients with Fontan palliation.The primary outcome was to determine the frequency with which congenital heart disease patients undergo laparoscopic surgery requiring abdominal insufflation compared with open surgery. Secondary outcomes included all-cause in-hospital mortality and in-hospital length of stay. Of the 5,527 patients included, nearly half underwent laparoscopic surgery (46.3%), and 128 (2.3%) had single-ventricle circulation. All-cause mortality was significantly higher for those who underwent open surgery compared with the laparoscopic approach (3.6% v 0.9%; odds ratio [OR], 4.0 [2.6-6.3]; p0.0001). Subgroup analysis of patients with Fontal palliation older than five years showed 30 (42%) underwent laparoscopic surgery and there was no mortality difference between the laparoscopic and open approaches (OR, 1.4 [0.2-21.3], p = 0.8). Length of stay was significantly shorter for patients undergoing laparoscopic compared with open surgery (median three days [interquartile range, two-five] v six days [three-13], p0.0001).Individuals with congenital heart disease are being offered laparoscopic surgery that requires abdominal insufflation. All-cause mortality and length of stay were higher for patients who underwent open surgical operations.

The objective of this study was to evaluate the impact of the regular introduction of new technologies into interventional cardiac catheterization procedures, in this case new atrial septal defect (ASD) closure devices, while conducting a multi-center collaborative initiative to reduce radiation usage during all procedures. Data were collected prospectively by 8 C3PO institutions between January 1, 2014 and December 31, 2017 for ASD device closure procedures in the cardiac catheterization lab during a quality improvement (QI) initiative aimed at reducing patient radiation exposure. Radiation exposure was measured in dose area product per body weight (µGy*m2/kg). Use of proposed practice change strategies at the beginning and end of the QI intervention period was assessed. Radiation exposure was summarized by institution and by initial type of device used for closure. This study included 602 ASD device closures. Without changes in patient characteristics, total fluoroscopy duration, or number of digital acquisitions, median radiation exposure decreased from 37 DAP/kg to 14 DAP/kg from 2014 to 2017. While all individual centers decreased overall median DAP/kg, the use of novel devices for ASD closure correlated with a temporary period of worsening institutional radiation exposure and increased fluoroscopy time. The introduction of new ASD closure devices resulted in increased radiation exposure during a QI project designed to reduce radiation exposure. Therefore, outcome assessment must be contextualized in QI projects, hospital evaluation, and public reporting, to acknowledge the expected variation during innovation and introduction of novel therapies.

Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a rare cutaneous form of Chronic active Epstein-Barr virus (CAEBV) that presents with vesicular lesions induced by sun-exposure. We describe a patient with CAEBV and HV-LPD whose course following hematopoietic stem cell transplantation was complicated by fatal ruptured cerebral artery aneurysm.

Background: Neonatal myocardial infarction is rare and is associated with a high mortality of 40% to 50%. We report our experience with neonatal myocardial infarction, including presentation, management, outcomes, and our current patient management algorithm. Methods: We reviewed all infants admitted with a diagnosis of coronary artery thrombosis, coronary ischemia, or myocardial infarction between January 2015 and May 2021. Results: We identified 21 patients (median age, 1 [interquartile range (IQR), 0.25–9.00] day; weight, 3.2 [IQR, 2.9–3.7] kg). Presentation included respiratory distress (16), shock (3), and murmur (2). Regional wall motion abnormalities by echocardiogram were a key criterion for diagnosis and were present in all 21 with varying degrees of depressed left ventricular function (severe [8], moderate [6], mild [2], and low normal [5]). Ejection fraction ranged from 20% to 54% (median, 43% [IQR, 34%–51%]). Mitral regurgitation was present in 19 (90%), left atrial dilation in 15 (71%), and pulmonary hypertension in 18 (86%). ECG was abnormal in 19 (90%). Median troponin I was 0.18 (IQR, 0.12–0.56) ng/mL. Median BNP (B-type natriuretic peptide) was 2100 (IQR, 924–2325) pg/mL. Seventeen had documented coronary thrombosis by cardiac catheterization. Seventeen (81%) were treated with intracoronary tPA (tissue-type plasminogen activator) followed by systemic heparin, AT (antithrombin), and intravenous nitroglycerin, and 4 (19%) were treated with systemic heparin, AT, and intravenous nitroglycerin alone. Nineteen of 21 recovered. One died (also had infradiaphragmatic total anomalous pulmonary venous return). One patient required a ventricular assist device and later underwent heart transplant; this patient was diagnosed late at 5 weeks of age and did not respond to tPA. Nineteen of 21 (90%) regained normal left ventricular function (ejection fraction, 60%–74%; mean, 65% [IQR, 61%–67%]) at latest follow-up (median, 6.8 [IQR, 3.58–14.72] months). Two of 21 (10%) had residual trivial mitral regurgitation. After analysis of these results, we present our current algorithm, which developed and matured over time, to manage neonatal myocardial infarction. Conclusions: We experienced a lower mortality rate for infants with neonatal infarction than that reported in the literature. We propose a post hoc algorithm that may lead to improvement in patient outcomes following coronary artery thrombus.

Van Praagh (VP) A3 variant of truncus arteriosus (or common arterial trunk) is defined by only one pulmonary artery (usually the right) originating from the common trunk, while the other lung is supplied either by collaterals or a pulmonary artery arising from the aortic arch. This report describes a staged approach to manage a VP-A3 variant truncus arteriosus with ductal origin of the left pulmonary artery (LPA), a hypoplastic right pulmonary artery, and cyanosis. Initially, the ductal portion of the proximal LPA was stented with a Resolute Onyx drug-eluting stent. The pulmonary arteries grew and at four months of age had an acceptable McGoon ratio and Nakata index. The patient then underwent repair which included unifocalization of the branch pulmonary arteries, closure of the ventricular septal defect, and placement of a right ventricle-to-pulmonary artery homograft conduit.

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.

The diagnostic capabilities, palliative procedures, and critical care therapies for neonates with single ventricle heart disease have rapidly evolved. We aimed to review current trends for intensivists and cardiologists caring for this population. Enhanced diagnostics and 3-dimensional modeling can be instrumental in planning care pathways for these neonates. Additionally, the ability to palliate many of these patients with catheter-based therapies rather than traditional surgical approaches has allowed for reduced morbidity. There are significant improvements in the understanding of how critical care therapies affect survival and neurodevelopmental outcomes in this population. Despite advancements in the understanding of longer-term outcomes in this population, much remains to be discovered about how initial palliation, critical care strategies, surgical techniques, and extracorporeal support can be optimized for the most favorable neurodevelopmental outcomes, survival, and quality of life. This will be the major focus of future research through larger collaboratives.

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.

PURPOSE: To assess the ability of four-dimensional (4D) flow MRI to quantify flow volume of the Fontan circuit, including the frequency and hemodynamic contribution of systemic-to-pulmonary venovenous collateral vessels. MATERIALS AND METHODS: In this retrospective study, patients with Fontan circulation were included from three institutions (2017–2021). Flow measurements were performed at several locations along the circuit by two readers, and collateral shunt volumes were quantified. The frequency of venovenous collaterals and structural defects were tabulated from concurrent MR angiography, contemporaneous CT, or catheter angiography and related to Fontan clinical status. Statistical analysis included Pearson and Spearman correlation and Bland-Altman analysis. RESULTS: Seventy-five patients (mean age, 20 years; range, 5–58 years; 46 female and 29 male patients) were included. Interobserver agreement was high for aortic output, pulmonary arteries, pulmonary veins, superior vena cava (Glenn shunt), and inferior vena cava (Fontan conduit) (range, ρ = 0.913–0.975). Calculated shunt volume also showed strong agreement, on the basis of the difference between aortic and pulmonary flow (ρ = 0.935). A total of 37 of 75 (49%) of the patients exhibited shunts exceeding 1.00 L/min, 81% (30 of 37) of whom had pulmonary venous or atrial flow volume step-ups and corresponding venovenous collaterals. A total of 12% of patients (nine of 75) exhibited a high-output state (>4 L/min/m(2)), most of whom had venovenous shunts exceeding 30% of cardiac output. CONCLUSION: Fontan flow and venovenous shunting can be reliably quantified at 4D flow MRI; high-output states were found in a higher proportion of patients than expected, among whom venovenous collaterals were common and constituted a substantial proportion of cardiac output. Keywords: Pediatrics, MR Angiography, Cardiac, Technology Assessment, Hemodynamics/Flow Dynamics, Congenital Supplemental material is available for this article. © RSNA, 2021

To report a single-institution's experience of symptomatic aortopulmonary collaterals presenting as tracheostomy tube hemorrhage.Retrospective case series and Contemporary Review.Tertiary care children's hospital.Retrospective review, from 2015 to 2020, of patients18 years old who were treated for tracheostomy hemorrhage with endovascular embolization of aortopulmonary collateral (APC) vessels.4 patients were identified, 2 males and 2 females, ages 15 months-to 14 years-old, with a range of cyanotic congenital heart diseases. Direct laryngoscopy and bronchoscopy were necessary for excluding proximal airway sources. Chest computed tomography angiography did not localize pulmonary hemorrhage, but helped identify aortopulmonary collaterals. Cardiac catheterization was both diagnostic and therapeutic with use of endovascular embolization techniques by pediatric interventional cardiology and interventional radiology. Previously reported APC-related pulmonary hemorrhages occurred in significantly different settings compared to our patients, and evaluation by an otolaryngologist was key to rule out upper airway etiologies.Symptomatic aortopulmonary collaterals is an important etiology in pediatric tracheostomy tube hemorrhage in patients with cyanotic congenital heart disease.

The objective of this study was to evaluate the impact of the regular introduction of new technologies into interventional cardiac catheterization procedures, in this case new atrial septal defect (ASD) closure devices, while conducting a multi-center collaborative initiative to reduce radiation usage during all procedures. Data were collected prospectively by 8 C3PO institutions between January 1, 2014 and December 31, 2017 for ASD device closure procedures in the cardiac catheterization lab during a quality improvement (QI) initiative aimed at reducing patient radiation exposure. Radiation exposure was measured in dose area product per body weight (µGy*m

PURPOSE: To compare invasive right heart catheterization with four-dimensional (4D) flow MRI for estimating shunt fraction in patients with intracardiac and extracardiac shunts. MATERIALS AND METHODS: In this retrospective study, patients who underwent 4D flow MRI and invasive right heart catheterization with a shunt run between August 2015 and November 2018 were included. The primary objective was comparison of estimated shunt fraction (ratio of pulmonary-to-systemic flow, Q(p)/Q(s)) at 4D flow and catheterization. Secondary objectives included comparison of the right ventricular–to–left ventricular stroke volume ratio (RVSV/LVSV) to shunt fraction (for those with applicable shunts) and comparison of cardiac output between 4D flow and catheterization. Statistical analysis included Pearson correlation and Bland-Altman plots. RESULTS: A total of 33 patients met inclusion criteria (mean age, 49 years ± 16 [standard deviation]; 24 women). 4D flow measurements of Q(p)/Q(s) strongly correlated with those at catheterization (r = 0.938), and there was no bias. RVSV/LVSV correlated strongly with Q(p)/Q(s) from 4D flow (r = 0.852) and catheterization (r = 0.842). Measurements of left ventricle (Q(s)) and right ventricle (Q(P)) cardiac output from 4D flow and catheterization (Fick) correlated moderately overall (r = 0.673 [Q(p)] and r = 0.750 [Q(s)]). CONCLUSION: Shunt fraction measurement using 4D flow MRI compares well with that using invasive cardiac catheterization. Supplemental material is available for this article. © RSNA, 2021

Background/objective Stenting the ductus arteriosus (DAS) has become an alternative to surgical systemic to pulmonary artery shunts in neonates with ductal-dependent pulmonary blood flow (PBF). Femoral approach for a vertical ductus can be difficult secondary to the acute angle and tortuous course, thus alternative access sites have been explored. Carotid access complications have been reported in 5%-10%. The extensive use of an axillary arterial approach in the United States has not been reported. The aim of this study is to describe our experience with DAS using the axillary approach. Methods We reviewed all patients with DAS with an axillary approach in neonates with ductal-dependent PBF (May 2017-May 2018) in our institution. Procedural reports, angiograms, and clinical records of all consecutive patients were reviewed. Procedural technique, procedural outcomes, adverse events, and post-hospital courses are reported. Results Seven consecutive patients who received DAS utilizing axillary approach. All patients had ductal-dependent PBF through a vertical, tortuous ductus. Five had pulmonary atresia or near atresia, one had compromised PBF due to dynamic subvalvar obstruction, and one had Tetralogy of Fallot with isolated left pulmonary artery. Axillary access with 3.3 or 4 French sheath was obtained using ultrasound guidance. Bare metal coronary stents were deployed successfully in all. Intra-procedure, one developed in stent thrombus requiring re-stenting. There were no procedural mortalities or major adverse events from axillary access. There is a steep learning curve. Hemostasis was achieved with manual compression. Two patients had reintervention at 6-8 weeks. All patients underwent successful planned surgeries. Conclusions This series suggests DAS in neonates utilizing an axillary approach is a feasible and effective alternative for establishing PBF. Axillary arterial approach may be preferred as there is no risk to neurological sequelae and very low risk of limb complications. Larger series are needed to validate this approach.

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.

Background In the modern era, results of the arterial switch operation (ASO) for transposition of the great arteries are excellent. However, because of the LeCompte maneuver, there may be a propensity for development of pulmonary artery stenosis. We encountered atypical complications of pulmonary artery stenting in patients after the ASO, including aorto-pulmonary fistula and coronary compression. Methods We performed a 10-year retrospective review of catheterizations performed in patients after ASO in our institution with a focus on adverse events. Results Diagnostic and interventional catheterizations were performed in 47 patients. In 29 patients, 37 interventional procedures performed, which included pulmonary artery angioplasty and/or stenting. In this group, there were five major adverse events (14%), including three aorto-pulmonary fistulae and one coronary artery compression among patients having stent implantation or stent redilation. In addition, there were 6/37 (16%) intended stent procedures, which were aborted because there appeared to be high-risk of significant adverse events. Conclusions This review suggests that percutaneous intervention on pulmonary artery stenosis after ASO has high-risk and should be undertaken advisedly. Prior thorough evaluation of coronary arteries is mandatory as coronary reimplantation sites may be adjacent to sites of pulmonary artery stenosis. Furthermore, if pulmonary artery stent implantation or stent redilation is contemplated, the risk of stent fracture and possible AP fistula should be recognized. Primary use of reinforced covered stents should be considered.

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.

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.

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.

KEY POINTS The distribution of pulmonary perfusion is affected by gravity, vascular branching structure and active regulatory mechanisms, which may be disrupted by cardiopulmonary disease, but this is not well studied, particularly in rare conditions. We evaluated pulmonary perfusion in patients who had undergone Fontan procedure, patients with pulmonary arterial hypertension (PAH) and two groups of controls using a proton magnetic resonance imaging technique, arterial spin labelling to measure perfusion. Heterogeneity was assessed by the relative dispersion (SD/mean) and gravitational gradients. Gravitational gradients were similar between all groups, but heterogeneity was significantly increased in both patient groups compared to controls and persisted after removing contributions from large blood vessels and gravitational gradients. Patients with Fontan physiology and patients with PAH have increased pulmonary perfusion heterogeneity that is not explainable by differences in mean perfusion, gravitational gradients, or large vessel anatomy. This probably reflects vascular remodelling in PAH and possibly in Fontan physiology. ABSTRACT Many factors affect the distribution of pulmonary perfusion, which may be disrupted by cardiopulmonary disease, but this is not well studied, particularly in rare conditions. An example is following the Fontan procedure, where pulmonary perfusion is passive, and heterogeneity may be increased because of the underlying pathophysiology leading to Fontan palliation, remodelling, or increased gravitational gradients from low flow. Another is pulmonary arterial hypertension (PAH), where gravitational gradients may be reduced secondary to high pressures, but remodelling may increase perfusion heterogeneity. We evaluated regional pulmonary perfusion in Fontan patients (n = 5), healthy young controls (Fontan control, n = 5), patients with PAH (n = 6) and healthy older controls (PAH control) using proton magnetic resonance imaging. Regional perfusion was measured using arterial spin labelling. Heterogeneity was assessed by the relative dispersion (SD/mean) and gravitational gradients. Mean perfusion was similar (Fontan = 2.50 ± 1.02 ml min-1 ml-1 ; Fontan control = 3.09 ± 0.58, PAH = 3.63 ± 1.95; PAH control = 3.98 ± 0.91, P = 0.26), and the slopes of gravitational gradients were not different (Fontan = -0.23 ± 0.09 ml min-1 ml-1 cm-1 ; Fontan control = -0.29 ± 0.23, PAH = -0.27 ± 0.09, PAH control = -0.25 ± 0.18, P = 0.91) between groups. Perfusion relative dispersion was greater in both Fontan and PAH than controls (Fontan = 1.46 ± 0.18; Fontan control = 0.99 ± 0.21, P = 0.005; PAH = 1.22 ± 0.27, PAH control = 0.91 ± 0.12, P = 0.02) but similar between patient groups (P = 0.13). These findings persisted after removing contributions from large blood vessels and gravitational gradients (all P

Background This study reports the long-term outcomes using glutaraldehyde-treated cryopreserved homograft pericardium (CPH) in neonates, infants, children, and young adults undergoing congenital cardiac surgery. Methods A retrospective review was performed of all patients at a single institution (Rady Children’s Hospital, San Diego, CA) who had undergone surgical implantation with CPH between 2006 and 2016. The study identified 134 consecutive patients who underwent implantation of a total of 276 patches. The baseline demographic characteristics, primary cardiac diagnosis, surgical characteristics, operative reports, and postoperative catheterization and reoperation reports were analyzed. The use of CPH was categorized by specific anatomic insertion site. Results The median age at patch implantation was 1.47 years (range, 1 day to 31.6 years). The numbers and locations of patch use were 124 for pulmonary arterial repair, 57 for repair of the aorta, 49 for septal repair, and 43 at other sites. At a median follow-up of 5.29 years, 9 patients had died (6.7%), but none of those deaths were related to CPH. Twelve patients (8.96%) underwent reoperations, and 18 patients (13.4%) underwent catheter interventions at sites of CPH implantation. The 10-year freedom from patch-induced reoperation and catheter intervention rates were 88.5% and 86.9%, respectively. Overall patch failure–free survival was 85.8% and 79.0% at 5 and 10 years, respectively. Conclusions The use of CPH patch in the surgical correction of congenital heart disease is effective and durable, as evidenced by the low reintervention rates. These results are comparable to the early and midterm outcomes of other similarly used surgical patches..

Background: The C3PO-QI (Congenital Cardiac Catheterization Project on Outcomes – Quality Improvement), a multicenter registry launched in 2015, instituted quality improvement (QI) initiatives to reduce patient radiation exposure. Through regular collaboration, this initiative would allow for harmony among active participants, maximizing efforts and efficiency at achieving radiation best practices. This study sought to report these efforts with a detailed methodology for which institutions can target initiatives, reducing radiation exposure, and increasing patient safety. Methods: Data were collected prospectively by 8 C3PO-QI institutions between January 1, 2015 and December 31, 2017. Radiation exposure was measured in dose area product per body weight (dose area product/kg; µGy*m 2 /kg) and reported by expected radiation exposure categories (REC) and institution for 40 published unique procedure types. Targeted interventions addressing selected strategic domains for radiation reduction were implemented in the pediatric catheterization labs of the C3PO-QI institutions. Results: The study consisted of 15 257 unique cases. Median exposure (dose area product/kg) was decreased by 30% for all procedures. Dose area product/kg was reduced in all 3 REC, with the greatest improvement observed in REC I (REC I, −37%; REC II, −23%; REC III, −27%). Although the baseline radiation exposures and exact percent decrease varied across all C3PO-QI sites, each institution demonstrated improvements in radiation dose over time. These improvements occurred with the implementation of institution-specific QI interventions accelerated by participation in the C3PO-QI multicenter collaborative. Conclusions: Substantial radiation dose reductions can be achieved using targeted QI methodology and interventions. Participation in a multicenter QI collaborative may accelerate improvement across all centers due to enhanced engagement and shared learning between sites.

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.

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.

Percutaneous therapies for congenital heart disease have been evolving rapidly despite limited investment from industry. The Melody transcatheter pulmonary valve (Medtronic, Inc, Minneapolis, MN USA) replacement therapy represents an important advancement in this arena. It has been approved in the United States for use in the pulmonary position, on a Humanitarian Device Exemption status. Off-label use of the Melody transcatheter pulmonary valve has extended to the mitral, pulmonary, and aortic valves, especially in previously implanted valves with prosthetic valve degeneration. The single-ventricle patient poses additional challenges. However, there exists one report in the English literature of a patient undergoing Melody transcatheter neoaortic valve replacement after the patient developed severe neoaortic regurgitation after Fontan palliation. Here, we describe a patient with hypoplastic left heart syndrome, palliated with a Norwood modified Blalock-Taussig shunt, with a progressively regurgitant quadricusp neoaortic valve who underwent bioprosthetic valve replacement. There was early prosthetic valve degeneration after a year of bioprosthesis implantation. As he was declined for transplantation, he underwent successful perventricular Melody valve-in-valve replacement.

Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue tumor characterized by proliferation of fibroblastic cells associated with an inflammatory infiltrate. Inflammatory myofibroblastic tumors have a predilection for the pediatric population and are usually found in the lung parenchyma but rarely at the carina. They rarely metastasize but can be locally destructive. Surgical resection is the cornerstone of therapy, which results in excellent survival despite risk of local recurrence. We present the case of a nine-year-old girl with an IMT mass at the carina and obstructing the left main stem bronchus, requiring extensive resection and reconstruction.

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