Karen Texter^1,2, Jo Ann M. Davis¹, Christina Phelps^1,2, Sharon Cheatham^1,2, John Cheatham^1,2, Mark Galantowicz^1,3, Timothy F. Feltes^1,2

Congenital Heart Disease, Vol.12, No.4, pp. 403-410, 2017, DOI:10.1111/chd.12459

Abstract Introduction: With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring.
Methods: In 2013 our Heart Center began a process to build a comprehensive Single Ventricle Team designed to target these difficult issues.
Results: Comprehensive Single Ventricle Team in 2014 was begun, to standardize care for children with single ventricle heart defects from diagnosis to adulthood within our institution. The team is a multidisciplinary group of providers committed to improving… More >

Jeffrey A. Robinson, Jill K. Shivapour, Christopher S. Snyder

Congenital Heart Disease, Vol.12, No.4, pp. 411-416, 2017, DOI:10.1111/chd.12458

Abstract Objective: Pseudosyncope can be difficult to distinguish from true syncope. Often, pediatric patients with pseudosyncope undergo multiple tests and referrals before the appropriate diagnosis is reached. The purpose is to describe the utility of the head-up tilt table test to elicit the diagnosis of pseudosyncope in the pediatric population.
Design: Retrospective chart review from November 2012 to December 2015 of patients age ≤23 years referred for 30-minute, 80-degree tilt table test. Pretest probability for pseudosyncope was high if there was no response to traditional management, atypical episodes, occurrence during undesirable exercise, or prolonged episode duration. Inductive techniques… More >

Paul Tannous^1,2, Sunil J Ghelani^1,2, Audrey C Marshall^1,2, Diego Porras^1,2

Congenital Heart Disease, Vol.12, No.4, pp. 467-474, 2017, DOI:10.1111/chd.12457

Abstract Objective: Angiographically detectable Thebesian veins (ThVs) are a rare finding sometimes associated with coronary steal and myocardial ischemia in adults, but there are limited data regarding prominent ThVs in the setting of complex congenital heart disease (CHD). This study represents the largest series to date describing the presence and temporal changes of angiographically detectable ThVs in children with CHD.
Methods: This is a single center case series describing the clinical characteristics and coronary anatomy in children with CHD and angiographicall detectable ThVs. After identification of the index case, additional patients were identified in a prospective manner during… More >

M. Elisabeth Heal¹, Lanier B. Jackson², Andrew M. Atz², Ryan J. Butts³

Congenital Heart Disease, Vol.12, No.4, pp. 399-402, 2017, DOI:10.1111/chd.12451

Abstract Cardiopulmonary exercise testing (CPET) aids in clinical assessment of patients with Fontan circulation. Effects of persistent fenestration on CPET variables have not been clearly defined. Associations between fenestration and CPET variables at anaerobic threshold (AT) and peak exercise were explored in the Pediatric Heart Network Fontan Cross-Sectional Study cohort. Fenestration patency was associated with a greater decrease in oxygen saturation from rest to peak exercise (fenestration -4.9 ± 3.8 v. nonfenestration -3 ± 3.5; P < .001). Physiological dead space at peak exercise was higher in fenestrated v. nonfenestrated (25.2 ± 16.1 v. 21.4 ± More >

Daniel J Penny

Congenital Heart Disease, Vol.12, No.3, pp. 241-241, 2017, DOI:10.1111/chd.12474

Abstract This article has no abstract. More >

Katherine Smith, Bernard Gros

Congenital Heart Disease, Vol.12, No.3, pp. 251-260, 2017, DOI:10.1111/chd.12465

Abstract A well-established association exists between acute aortic dissection and pregnancy, particularly in women with Marfan syndrome. However, there is debate regarding appropriate management guidelines. In particular, there are differing opinions regarding when prophylactic aortic root repair should be recommended as well as the efficacy of beta blockers in this clinical scenario. The current study evaluated 10 years of published literature (2005-2015) in the PubMed/Medline database. Fifty articles, describing 72 cases of women who presented with aortic dissection in the antepartum or postpartum period were identified. Comparisons on demographic variables and clinical outcomes between cases of… More >

Ian K. Everitt¹, Jennifer F. Gerardin², Fred H. Rodriguez^2,3, Wendy M. Book²

Congenital Heart Disease, Vol.12, No.3, pp. 242-25, 2017, DOI:10.1111/chd.12463

Abstract The transition and transfer from pediatric to adult care is becoming increasingly important as improvements in the diagnosis and management of congenital heart disease allow patients to live longer. Transition is a complex and continuous process that requires careful planning. Inadequate transition has adverse effects on patients, their families and healthcare delivery systems. Currently, significant gaps exist in patient care as adolescents transfer to adult care and there are little data to drive the informed management of transition and transfer of care in adolescent congenital heart disease patients. Appropriate congenital heart disease care has been… More >

Katlyn Elizabeth McGrattan^1,2,3,4, Heather McGhee^2,3, Allan DeToma⁵, Elizabeth G. Hill⁵, Sinai C. Zyblewski⁶, Maureen Lefton-Greif^7,8,9, Lucinda Halstead^1,2, Scott M. Bradley¹⁰, Bonnie Martin-Harris^1,2,3,4

Congenital Heart Disease, Vol.12, No.3, pp. 382-388, 2017, DOI:10.1111/chd.12456

Abstract Background: Deficits in swallowing physiology are a leading morbidity for infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliation. Despite the high prevalence of this condition, the underlying deficits that cause this post-operative impairment remain poorly understood.
Objective: Identify the physiologic correlates of dysphagia in infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliative surgery.
Methods: Postoperative fiberoptic laryngoscopies and videofluoroscopic swallow studies (VFSS) were conducted sequentially on infants with functional single ventricles following stage 1 palliative surgery. Infants were dichotomized as having normal or impaired laryngeal function… More >

Ritu Sachdeva¹, Pamela S. Douglas², Michael S. Kelleman¹, Courtney E. McCracken¹, Leo Lopez³, Kenan W.D. Stern⁴, Benjamin W. Eidem⁵, Oscar J. Benavidez⁶, Rory B. Weiner⁶, Elizabeth Welch³, Robert M. Campbell¹, Wyman W. Lai⁷

Congenital Heart Disease, Vol.12, No.3, pp. 373-381, 2017, DOI:10.1111/chd.12455

Abstract Objective: The objective of this study was to evaluate effectiveness of educational intervention (EI) in the Pediatric Appropriate Use of Echocardiography (PAUSE) study to improve appropriateness of transthoracic echocardiograms (TTEs) ordered in pediatric cardiology clinics.
Design: Data were prospectively collected after the publication of the Appropriate Use Criteria (AUC) document during 2 phases: the pre-EI phase (1/1/15 to 4/30/15) and the post-EI phase (7/ 1/15 to 10/30/15). Pre-EI, site-investigators (SI) determined AUC indications, by reviewing the clinic records. Post-EI, providers assigned indications prior to obtaining TTE.
Setting: Pediatric cardiology clinics at six centers.
Patients: Those ≤18 years old, receiving… More >

Koert de Waal¹, Nilkant Phad¹, Nick Collins², Andrew Boyle²

Congenital Heart Disease, Vol.12, No.3, pp. 364-372, 2017, DOI:10.1111/chd.12454

Abstract Background: Sustained volume load due to a patent ductus arteriosus (PDA) leads to cardiac remodeling. Remodeling changes can become pathological and are associated with cardiovascular disease progression. Data on remodeling changes in preterm infants is not available.
Methods: Clinical and echocardiography data were collected in preterm infants <30 weeks gestation on postnatal day 3 and then every 7–14 days until closure of the ductus arteriosus. Images were analyzed using conventional techniques and speckle tracking. Remodeling changes of infants with prolonged (>14 days) exposure to a PDA were compared to control infants without a PDA.
Results: Thirty out of… More >