Open Access

REVIEW

Robert W. Loar, Cory V. Noel, Hari Tunuguntla, John L. Colquitt, Ricardo H. Pignatelli
Congenital Heart Disease, Vol.13, No.1, pp. 5-15, 2018, DOI:10.1111/chd.12564
Abstract Chemotherapy-induced cardiotoxicity in adults and children is a topic with a growing interest in the cardiology literature. The ability to detect cardiac dysfunction in a timely manner is essential in order to begin adequate treatment and prevent further deterioration. This article aims to provide a review on the myocardial injury process, chemotherapeutic agents that lead to cardiotoxicity, the definition of cardiotoxicity, and the methods of timely detection and treatment. More >

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1231

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REVIEW

Uri Pollak^1,2,3, David Mishaly^3,4, Gili Kenet^3,5, Amir Vardi^1,3
Congenital Heart Disease, Vol.13, No.1, pp. 16-25, 2018, DOI:10.1111/chd.12557
Abstract Heparin-induced thrombocytopenia (HIT) is a life-threatening complication of heparin therapy. The risk for HIT correlates with the cumulative dosage of heparin exposure. In Fontan patients, recurrent systemic anticoagulation, traditionally with heparin, is used to alleviate the thrombotic complications that may occur postoperatively when the venous pressure rises and the systemic venous flow into the pulmonary arteries becomes sluggish, putting them at increased risk. As a pressure gradient-dependent circulation, elevation in systemic venous pressure, most often by venous thrombosis, contributes to circuit failure. Therefore, when HIT complicates patients after the Fontan procedure, it is associated with More >

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1471

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1093

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ARTICLE

Philip Wackel^1,2, Bryan Cannon^1,2, Joseph Dearani³, Kristen Sessions^1,2, Kimberly Holst³, Jonathan Johnson^1,2, Frank Cetta^1,2
Congenital Heart Disease, Vol.13, No.1, pp. 26-30, 2018, DOI:10.1111/chd.12566
Abstract Background: The increased incidence of preoperative and postoperative arrhythmia in Ebstein anomaly (EA) prompted some clinicians to perform an electrophysiology study (EPS) in all patients prior to surgery for EA. The cone repair (CR) is the current surgical option of choice for most young patients with EA but the effect of the CR on arrhythmia is not well established.
Objectives: To assess the burden of arrhythmia in young patients after CR and to assess the utility of selective preoperative EPS.
Materials and Methods: A retrospective review of all patients <21 years of age with EA who had a… More >

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1170

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Daniel E. Ehrmann¹, Matthew Mulvahill², Shaunda Harendt^1,3, Jessica Church¹, Amy Stimmler¹, Piyagarnt Vichayavilas^1,4, Sanja Batz^1,5, Jennifer Rodgers^1,5, Michael DiMaria¹, James Jaggers¹, Cindy Barrett¹, Jon Kaufman¹
Congenital Heart Disease, Vol.13, No.1, pp. 31-37, 2018, DOI:10.1111/chd.12550
Abstract Background: Feeding practices after neonatal and congenital heart surgery are complicated and variable, which may be associated with prolonged hospitalization length of stay (LOS). Systematic assessment of feeding skills after cardiac surgery may earlier identify those likely to have protracted feeding difficulties, which may promote standardization of care.
Methods: Neonates and infants ≤3 months old admitted for their first cardiac surgery were retrospectively identified during a 1-year period at a single center. A systematic feeding readiness assessment (FRA) was utilized to score infant feeding skills. FRA scores were assigned immediately prior to surgery and 1, 2, and… More >

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1081

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William N. Evans^1,2, Ruben J. Acherman^1,2, Michael L. Ciccolo^1,3, Sergio A. Carrillo^1,2, Alvaro Galindo^1,2, Abraham Rothman^1,2, Gary A. Mayman^1,2, Elizabeth A. Adams^1,2, Leigh C. Reardon^1,4, Brody J. Winn⁵, Noel S. Yumiaco⁵, Lesley Shimuizu¹, Yoko Inanaga¹, Rowena J. Deleon¹, Humberto Restrepo^1,2
Congenital Heart Disease, Vol.13, No.1, pp. 38-45, 2018, DOI:10.1111/chd.12558
Abstract Objective: We hypothesized that clinic-based, hepatic-ultrasound, elastography measurements, either alone or in combination with other noninvasive variables, might correlate with liver-biopsy fibrosis scores in patients post-Fontan.
Methods: Between March 2012 and February 2017, we identified patients post-Fontan that underwent elective cardiac catheterization and simultaneous transvenous hepatic biopsy. From this group, we selected patients that met inclusion criteria for liver-ultrasound, shear-wave elastography. Utilizing the results of elastography, laboratory testing, and time post-Fontan, we constructed a composite Fontan hepatic index as a sum of elastography measurements in kilopascals, model for end-stage liver disease excluding INR scores, and the square… More >

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1218

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Susan F. Saleeb¹, Sarah R. McLaughlin², Dionne A. Graham², Kevin G. Friedman¹, David R. Fulton¹
Congenital Heart Disease, Vol.13, No.1, pp. 46-51, 2018, DOI:10.1111/chd.12539
Abstract Objective: Using a Standardized Clinical Assessment and Management Plan (SCAMP) for pediatric patients presenting to clinic with chest pain, we evaluated the cost impact associated with implementation of the care algorithm. Prior to introduction of the SCAMP, we analyzed charges for 406 patients with chest pain, seen in 2009, and predicted 21% reduction of overall charges had the SCAMP methodology been used. The SCAMP recommended an echocardiogram for history, examination, or ECG findings suggestive of a cardiac etiology for chest pain.
Design: Resource utilization was reviewed for 1517 patients (7-21 years) enrolled in the SCAMP from July… More >

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1340

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1039

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ARTICLE

Carissa M. Baker-Smith¹, Karina Carlson², Jose Ettedgui³, Takeshi Tsuda⁴, K. Anitha Jayakumar⁵, Matthew Park⁶, Nikola Tede⁷, Karen Uzark⁸, Craig Fleishman⁹, David Connuck¹⁰, Maggie Likes¹¹, Daniel J. Penny¹²
Congenital Heart Disease, Vol.13, No.1, pp. 52-58, 2018, DOI:10.1111/chd.12540
Abstract Objective: To develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO).
Design: Under the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RAND-UCLA methodology. QMs were then made available for review by the entire ACC ACPC during an “open comment period.” Final… More >

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1387

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1027

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ARTICLE

Fred H. Rodriguez III^1,2,3,4, Georges Ephrem^1,2, Jennifer F. Gerardin^1,2, Cheryl Raskind-Hood⁵, Carol Hogue⁵, Wendy Book^1,2
Congenital Heart Disease, Vol.13, No.1, pp. 59-64, 2018, DOI:10.1111/chd.12563
Abstract Objective: Although the ICD-9-CM code 745.5 is widely used to indicate the presence of a secundum atrial septal defect (ASD), it is also used for patent foramen ovale (PFO) which is a normal variant and for “rule-out” congenital heart disease (CHD). The ICD-10-CM code Q21.1 perpetuates this issue. The objective of this study was to assess whether code 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 miscodes for CHD, and if true CHD positives decrease with age.
Design: Echocardiograms of patients with an ICD-9-CM code of 745.5 in isolation or in combination… More >

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1270

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Jill M. Steiner¹, James N. Kirkpatrick¹, Susan R. Heckbert², Asma Habib¹, James Sibley³, William Lober³, J. Randall Curtis³
Congenital Heart Disease, Vol.13, No.1, pp. 65-71, 2018, DOI:10.1111/chd.12524
Abstract Introduction: There is relatively sparse literature on the use of administrative datasets for research in patients with adult congenital heart disease (ACHD). The goal of this analysis is to examine the accuracy of administrative data for identifying patients with ACHD who died.
Methods: A list of the International Classification of Diseases codes representing ACHD of moderate- or great-complexity was created. A search for these codes in the electronic health record of adults who received care in 2010–2016 was performed, and used state death records to identify patients who died during this period. Manual record review was completed… More >

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1343

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1006

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ARTICLE

Astrida Kaugars¹, Clarissa Shields², Cheryl Brosig³
Congenital Heart Disease, Vol.13, No.1, pp. 72-78, 2018, DOI:10.1111/chd.12547
Abstract Objective: The study examined parent stress and health-related quality of life (HRQOL) among families of children with congenital heart disease (CHD) referred for psychological services.
Methods: Parents of 54 children (85% boys) aged 3 to 13 (M_age = 7.48, SD = 2.38) completed measures to assess parenting stress (Parenting Stress Index – Short Form; Pediatric Inventory for Parents) and the PedsQL Family Impact Module. Medical information was retrieved from medical record review.
Results: Half of parents of children with single ventricle anatomy had clinically significant levels of parenting stress. Parents of children with single ventricle anatomy reported more More >

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1351

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1084

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ARTICLE

Yumi Shiina¹, Tomoaki Murakami², Noriko Matsumoto³, Daisuke Okamura⁴, Yuta Takahashi⁴, Yosuke Nishihata¹, Nobuyuki Komiyama¹, Koichiro Niwa¹
Congenital Heart Disease, Vol.13, No.1, pp. 79-84, 2018, DOI:10.1111/chd.12555
Abstract Objectives: To assess body composition and relationships among body composition, appetiterelated hormones, adipocytokines, and heart failure (HF) in adult patients with congenital heart disease (CHD).
Patients: This prospective study enrolled 46 consecutive adult patients with CHD and 12 agematched healthy controls. The patients and control subjects were divided into four groups: 13 patients with Fontan circulation (group A), 16 patients with cyanosis (group B), 17 patients who previously underwent biventricular repair (group C), and 12 age-matched healthy controls.
Design: Body composition was measured using InBody730, and levels of appetite-related hormones (ghrelin and leptin) and adipocytokines (leptin, interleukin-6, and… More >

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1550

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1103

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ARTICLE

Sumeet S. Vaikunth^1,2, Roberta G. Williams³, Merujan Y. Uzunyan⁴, Han Tun⁵, Cheryl Barton³, Philip M. Chang⁵
Congenital Heart Disease, Vol.13, No.1, pp. 85-91, 2018, DOI:10.1111/chd.12549
Abstract Objective: Transition from pediatric to adult care is a critical time for patients with congenital heart disease. Lapses in care can lead to poor outcomes, including increased mortality. Formal transition clinics have been implemented to improve success of transferring care from pediatric to adult providers; however, data regarding outcomes remain limited. We sought to evaluate outcomes of transfer within a dedicated transition clinic for young adult patients with congenital heart disease.
Design, Setting, and Patients: We performed a retrospective analysis of all 73 patients seen in a dedicated young adult congenital heart disease transition clinic from January… More >

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1026

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ARTICLE

Rohit S. Loomba^1,2, Andrew N. Pelech^1,2
Congenital Heart Disease, Vol.13, No.1, pp. 92-97, 2018, DOI:10.1111/chd.12510
Abstract Background: Pulmonary atresia with intact ventricular septum is a unique congenital malformation of the heart in which patients may undergo single-, two- or 1½- ventricle repair. Size of the tricuspid valve annulus, morphology of the right ventricle and presence of ventriculocoronary connections can all impact the selection of appropriate palliative strategy. We developed the aortic perfusion score, a novel scoring system based on anterograde coronary perfusion with the aim of being able to identify patients at risk for death or transplant.
Methods: A retrospective study was conducted. Patients were included if an initial catheterization was done prior… More >

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1559

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1198

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Ashley R. Kroeger¹, Jacqueline Morrison², Andrew H. Smith¹
Congenital Heart Disease, Vol.13, No.1, pp. 98-104, 2018, DOI:10.1111/chd.12525
Abstract Objective: Unplanned readmission to the pediatric cardiac intensive care unit (CICU) is associated with significant morbidity and mortality. The Pediatric Early Warning Score (PEWS) predicts ward patients at risk for decompensation but has not been previously reported to identify at-risk patients with cardiac disease prior to ward transfer. This study aimed to determine whether PEWS prior to transfer may serve as a predictor of unplanned readmission to the CICU.
Design: All patients discharged from a tertiary children’s hospital CICU from September 2012 through August 2015 were included for analysis. PEWS assessment was performed following transfer to the… More >

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1363

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1079

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ARTICLE

Alberto Cresti¹, Raffaele Giordano², Martin Koestenberger³, Isabella Spadoni⁴, Marco Scalese⁵, Ugo Limbruno¹, Susanna Falorini⁶, Stefania Stefanelli¹, Andrea Picchi¹, Francesco De Sensi¹, Angela Malandrino⁷, Massimiliano Cantinotti^4,5
Congenital Heart Disease, Vol.13, No.1, pp. 105-112, 2018, DOI:10.1111/chd.12528
Abstract Background: Despite ventricular septal defects (VSDs) are the most common congenital heart diseases (CHDs) in the neonatal period, their incidence and natural history are still debated and their follow-up and management strategies remain controversial. Our aim was to evaluate the incidence and natural history of isolated VSDs.
Methods: From January 1996 to December 2015 all neonates with a CHD suspicion were referred to the Cardiological Department of Grosseto Misericordia Hospital. Only newborns with confirmed isolated VSD were enrolled in this study and followed for 6 years.
Results: Our 343 newborns with an isolated VSD (incidence of 10.45/1000/births) account… More >

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1186

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ARTICLE

Felicit e Kamdem^1,2, Danielle Kedy Koum^2,3, Ba Hamadou^1,4, Melanie Yemdji¹, Henry Luma^1,4, Marie Solange Doualla^1,4, Diomède Noukeu⁵, Esther Barla⁵, Christophe Akazong⁵, Anastase Dzudie^1,4, Henry Ngote¹, Yves Monkam¹, Sidiki Mouliom¹, Samuel Kingue^4,6
Congenital Heart Disease, Vol.13, No.1, pp. 113-117, 2018, DOI:10.1111/chd.12529
Abstract Introduction: Cardiovascular diseases in pediatric pathologies have emerged in the recent years in sub-Saharan Africa (SSA), with congenital heart diseases (CHDs) being the most frequent. Unfortunately, their diagnosis is usually delayed, thereby increasing childhood morbidity and mortality.
Objectives: Describe the clinical, echocardiographic, and therapeutic aspects of CHDs of children at Douala General Hospital.
Methods: We carried out a cross-sectional descriptive study over a 10-year period, from January 2006 to December 2015. Files and reports of cardiac ultrasounds of patients aged ≤ 15 years were reviewed.
Results: We reviewed the medical records of 1616 children, of which 370 (22.9%) had… More >

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1348

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1006

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ARTICLE

Randa M. Matter¹, Iman A. Ragab¹, Alaa M. Roushdy², Ahmed G. Ahmed¹, Hanan H. Aly¹, Eman A. Ismail³
Congenital Heart Disease, Vol.13, No.1, pp. 118-123, 2018, DOI:10.1111/chd.12530
Abstract Objectives: Congenital heart defects are common noninfectious causes of mortality in children. Bleeding and thrombosis are both limiting factors in the management of such patients. We assessed the frequency of thrombocytopenia in pediatric patients with congenital cyanotic heart disease (CCHD) and evaluated determinants of platelet count including immature platelet fraction (IPF) and their role in the pathogenesis of thrombocytopenia.
Methods: Forty-six children and adolescents with CCHD during pre-catheter visits were studied; median age was 20.5 months. Complete blood count including IPF as a marker of platelet production and reticulated hemoglobin content (RET-He) as a marker of red… More >

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1344

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963

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Jason F. Goldberg¹, Craig L. Jensen², Rajesh Krishnamurthy³, Nidhy P. Varghese⁴, Henri Justino¹
Congenital Heart Disease, Vol.13, No.1, pp. 124-130, 2018, DOI:10.1111/chd.12537
Abstract Objective: We describe the long-term follow-up of a child with recurrent hemoptysis due to severe pulmonary vein stenosis decompressing via collaterals to esophageal varices.
Design: Case report
Setting: Tertiary children’s hospital
Patient: Single child through ages 2- to 11-year old
Interventions: The child underwent cutting balloon angioplasty, bare metal stenting, and implantation of a PTFE-covered stent, all of which failed rapidly. Only after placement of a paclitaxel drug eluting stent did he have prolonged relief from hemoptysis and long-term patency of the treated vein. The stents were serially dilated to keep up with somatic growth of the child, eventually culminating More >

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1013

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Byung W. Yoo¹, Jung O. Kim², Lucy Y. Eun², Jae Y. Choi², Dong S. Kim³
Congenital Heart Disease, Vol.13, No.1, pp. 131-139, 2018, DOI:10.1111/chd.12541
Abstract Objective: The purpose of this study was to evaluate the changes in right ventricle (RV) and left ventricle (LV) function after transcatheter atrial septal defect (ASD) closure and to assess the influence of the age and the amount of shunt.
Design: Retrospective study
Patients: Fifty-three adult patients who underwent transcatheter closure were enrolled, then divided into subgroups according to the age (< 40 years vs ≥ 40 years), and the amount of shunt flow (QpQs < 2.5 vs QpQs ≥ 2.5).
Outcome Measures: Two-dimensional tissue Doppler imaging was performed in a four-chamber view at the basal ventricular septum (VS)… More >

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1166

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Wendy F. Li¹, Heidi Pollard², Mohsen Karimi³, Jeremy D. Asnes¹, William E. Hellenbrand¹, Veronika Shabanova⁴, Constance G. Weismann^1,5
Congenital Heart Disease, Vol.13, No.1, pp. 140-146, 2018, DOI:10.1111/chd.12544
Abstract Objective: Trans-catheter (TC) pulmonary valve replacement (PVR) has become common practice for patients with right ventricular outflow tract obstruction (RVOTO) and/or pulmonic insufficiency (PI). Our aim was to compare PVR and right ventricular (RV) function of patients who received TC vs surgical PVR.
Design: Retrospective review of echocardiograms obtained at three time points: before, immediately after PVR, and most recent.
Patients: Sixty-two patients (median age 19 years, median follow-up 25 months) following TC (N = 32) or surgical (N = 30) PVR at Yale-New Haven Hospital were included.
Outcome Measures: Pulmonary valve and right ventricular function before, immediately after,… More >

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Ashley E. Neal¹, Elizabeth Lehto¹, Karen Hughes Miller², Craig Ziegler², Erin Davis²
Congenital Heart Disease, Vol.13, No.1, pp. 147-153, 2018, DOI:10.1111/chd.12559
Abstract Objective: Although pediatrician-reported relevance of Canadian cardiology-specific objectives has been studied, similar data are not available for the 2016 American Board of Pediatrics (ABP) cardiology-specific objectives. This study asked Kentucky trainees, pediatricians, and pediatric cardiologists to identify “most important” content within these objectives.
Design, Methods, Outcome Measures: This cross-sectional study used an original, online survey instrument based on the 2016 ABP cardiology-specific objectives. We collected quantitative data (numerical indications of importance) and qualitative data (open-ended replies regarding missing content and difficulty in teaching and learning). Respondents indicated the top two choices of most important items within eight… More >

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957

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Joshua A. Daily^1,2, Elijah Bolin^1,2, Brian K. Eble^1,2
Congenital Heart Disease, Vol.13, No.1, pp. 154-156, 2018, DOI:10.1111/chd.12561
Abstract Pediatric cardiologists teach complicated concepts to a diverse group of learners that include medical students, nurses, residents, fellows, patients, and parents. Unfortunately, much of what is taught is not retained. In order to increase the likelihood of long-term retention, a cardiologist should teach with both meaning and sense. The authors provide a review of these concepts and give specific examples of how to teach in ways that both make sense and are meaningful to a cardiologist’s leaners. More >

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