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  • Ductus arteriosus‐associated infective endarteritis: Lessons from the past, future perspective
  • Abstract Background: Since routine clinical use of antibiotics as well as surgical and catheter‐ based closure of a patent arterial duct (PDA), PDA‐associated infective endarteritis (PDA‐IE) is rare but can still occur when the ductus is still open or as it closes. Thus, clinicians should maintain a high index of concern for patients with unexplained fever.
    Methods: We report on a PDA‐IE in a young infant shortly after potentially delayed obliteration of a PDA. We discuss this case report by reviewing the literature in regard to the pathogenesis (infection primary or secondary to PDA thrombus formation), clinical (new heart murmur) and…
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  • Surgical treatment of anomalous left main coronary artery with an intraconal course
  • Abstract Objective: Anomalous left main coronary artery (LMCA) with an intraconal course is a relatively rare form of anomalous aortic origin of a coronary artery (AAOCA) from the wrong sinus of Valsalva. There is currently a paucity of information regarding this entity. The purpose of this article is to review our surgical experience with repair of anomalous LMCA with an intraconal course.
    Methods: This was a retrospective review of 12 patients with an anomalous LMCA and an intraconal course who underwent surgical repair. The median age at surgery was 15 years (range 2‐47). The seven oldest patients all had preoperative symptoms…
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  • Alternative approach to pediatric cardiac quality assessment for low‐volume centers
  • Abstract Background: In pediatric cardiac care, many centers participate in multiple, national, domain‐specific registries, as a major component of their quality assessment and im‐ provement efforts. Small cardiac programs, whose clinical activities and scale may not be well‐suited to this approach, need alternative methods to assess and track quality.
    Methods: We conceived of and piloted a rapid‐approach cardiac quality assessment, intended to encompass multiple aspects of the service line, in a low‐volume program. The assessment incorporated previously identified measures, drawn from multiple sources, and ultimately relied on retrospective chart review.
    Results: A collaborative, multidisciplinary team formed and came to consensus on…
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  • Cardiovascular risk factors in adults with coarctation of the aorta
  • Abstract Background: The aging patient with adult congenital heart disease (ACHD) faces the risk of developing atherosclerotic disease. Patients with coarctation of the aorta (CoA) are especially vulnerable because of an inherent high risk of developing hy‐ pertension. However, data on the prevalence of other cardiovascular risk factors are scarce. Therefore, this study aimed to describe the prevalence of traditional cardio‐ vascular risk factors (diabetes, hypertension, hyperlipidemia, smoking, obesity, and sedentary lifestyle) in adult patients with CoA.
    Methods: Patients with CoA who were registered at the ACHD clinic in Gothenburg were asked to participate in a comprehensive cardiovascular risk assessment. This…
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  • Lost in the system? Transfer to adult congenital heart disease care—Challenges and solutions
  • Abstract Objective: Transfer of congenital heart disease care from the pediatric to adult set‐ ting has been identified as a priority and is associated with better outcomes. Our objective is to determine what percentage of patients with congenital heart disease transferred to adult congenital cardiac care.
    Design: A retrospective cohort study.
    Setting: Referrals to a tertiary referral center for adult congenital heart disease pa‐ tients from its pediatric referral base.
    Patients: This resulted in 1514 patients age 16‐30, seen at least once in three pediat‐ ric Georgia health care systems during 2008‐2010.
    Interventions: We analyzed for protective factors associated with age‐appropriate…
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  • Too little too late? Communication with patients with congenital heart disease about challenges of adult life
  • Abstract Objective: To investigate the experiences and communication preferences of adult patients with congenital heart disease (CHD) in the domains of employment, insur‐ ance, and family planning.
    Design: Patients ≥ 18 years of age completed a questionnaire about experiences and communication preferences regarding employment, health insurance, and family planning.
    Results: Of 152 patients (median age = 33 years, 50% female, 35% with CHD of great complexity), one in four reported work‐related problems due to CHD and a quar‐ ter also recalled a previous gap in health insurance. Of females, 29% experienced an unplanned pregnancy. The median importance of discussion ratings (on…
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  • Do we have the ACHD physician resources we need to care for the burgeoning ACHD population?
  • Abstract Background: Delivery of care to the adult congenital heart disease (ACHD) popula‐ tion has been limited by a shortage in the ACHD physician resources. There is limited data regarding the adequacy of the ACHD physician resources in the United States and our population estimates are extrapolated from Canadian data. Therefore, we proposed to evaluate the adequacy of ACHD physician: patient ratios in the United States at both national and regional levels.
    Methods: Data from the Adult Congenital Heart Association (ACHA) website along with metropolitan area and statewide population data from 2016 US Census Bureau estimates were analyzed. Physicians listed on…
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  • Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics
  • Abstract 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 reso‐ nance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modi‐ fied Ishak congestive hepatic fibrosis score.
    Results: Median age 17.8 years, median time since…
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  • Initiating a Fontan multidisciplinary clinic: Decreasing care variability, improving surveillance, and subsequent treatment of Fontan survivors
  • Abstract Background: Children with single ventricle (SV) heart disease who undergo Fontan operation are at risk for developing multiorgan dysfunction. Although survival has improved, significant comorbidities involving multiple organ systems may develop, requiring evaluation and management by many subspecialists. Using data from an internal survey, we documented high care variability for our Fontan population. We then developed a multidisciplinary clinic, designed and implemented a clinical care pathway to decrease variability of patient assessment.
    Methods: After creating a multidisciplinary team and a clinical care pathway, we initiated a multidisciplinary clinic (MDC) where patients could see multiple subspecialists during a single encounter. We…
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  • Right ventricular and pulmonary vascular function indices for risk stratification of patients with pulmonary regurgitation
  • Abstract Background: We hypothesized that echocardiographic indices of right ventricular to pulmonary artery (RV‐PA) coupling were comparable to cardiac magnetic resonance imaging (CMRI)‐derived RV volumetric indices in predicting disease severity in chronic pulmonary regurgitation (PR).
    Methods: Patients with ≥ moderate PR (2003‐2015) with and without prior CMRI scans were enrolled into the study cohort and validation cohort, respectively. Endpoint was to determine the association between noninvasive RV‐PA coupling in‐ dices (tricuspid annular plane systolic excursion/right ventricular systolic pressure [TAPSE/RVSP] and fractional area change [FAC]/RVSP ratio) and markers of disease severity, and compared this association to that of CMRI‐derived RV volumetric indi‐…
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