Seul Gi Cha¹, Mi Kyung Song¹, Sang Yun Lee¹, Gi Beom Kim¹, Jae Gun Kwak², Woong Han Kim², Eun Jung Bae¹

Congenital Heart Disease, Vol.14, No.5, pp. 684-690, 2019, DOI:10.1111/chd.12810

Abstract Objective: Cardiovascular lesions are the leading cause of morbidity and mortality in patients with Williams syndrome. Recent studies have rebutted conventional reports about the natural course of cardiovascular anomalies in Williams syndrome.
Design: Retrospective study.
Setting: Single tertiary center.
Patients: Eighty patients with Williams syndrome followed up for more than 5 years.
Interventions: Not applicable.
Outcome Measures: Long‐term outcome of cardiovascular lesions, peak velocity change in obstructive cardiovascular lesions over time, post‐interventional courses of disease‐specific intervention, and intervention‐free survival of obstructive cardio‐ vascular lesions.
Results: The median follow‐up duration was 11.0 (5.1‐28.3) years. Among 80 pa‐ tients, supravalvular aortic stenosis… More >

Brena Sue Haughey¹, Shelby Coral White², Michael David Seckeler²

Congenital Heart Disease, Vol.14, No.5, pp. 811-813, 2019, DOI:10.1111/chd.12809

Abstract Objective: Catheter‐associated bloodstream infections complicate and prolong hos‐ pitalizations. The incidence of catheter‐associated bloodstream infections in children undergoing congenital cardiac surgery has not been reported. This study sought to define the incidence of catheter‐associated bloodstream infections after congenital cardiac surgery in neonates and infants ≤12 months old and compare hospital out‐ comes and costs to those who underwent surgery and did not have a catheter‐associ‐ ated bloodstream infections.
Design: Retrospective review of hospital admissions between October 2013 and November 2015 for neonates and infants ≤12 months old at admission with ICD‐9 codes for congenital cardiac surgery from discharge data from… More >

Sonia A. Monteiro¹, Faridis Serrano¹, Rocky Tsang¹, Eboni Smith Hollier¹, Danielle Guffey², Lisa Noll¹, Robert G. Voigt¹, Nancy Ghanayem¹, Lara Shekerdemian¹

Congenital Heart Disease, Vol.14, No.5, pp. 797-802, 2019, DOI:10.1111/chd.12789

Abstract Objective: Neurodevelopmental impairment is common after surgery for congeni‐ tal heart disease (CHD) in infancy. While neurodevelopmental follow‐up of high‐risk patients has increased, the referral patterns for ancillary services following initial evaluation have not been reported. The aim of this study is to describe the rates and patterns of referral at the initial visit to our outcomes clinic of patients who under‐ went surgery for CHD during infancy.
Outcomes Measures: The Cardiac Developmental Outcomes Program clinic at Texas Children’s Hospital provides routine longitudinal follow‐up with developmental pedi‐ atricians and child psychologists for children who required surgery for CHD within the… More >

Katherine Awh^1,2, Morgan A. Venuti^1,2, Lacey P. Gleason^1,2, Rachel Rogers³, Srinivas Denduluri¹, Yuli Y. Kim^1,2

Congenital Heart Disease, Vol.14, No.5, pp. 726-734, 2019, DOI:10.1111/chd.12784

Abstract Objective: To determine the prevalence and predictors of nonattendance in an ACHD outpatient clinic, and to examine the relationship between nonattendance and emergency department (ED) visits, hospitalizations, and death.
Methods: Patients ≥ 18 years who had scheduled appointments at an ACHD outpatient clinic between August 1, 2014 and December 31, 2014 were included. The primary outcome of interest was nonattendance of the first scheduled appointment of the study period, defined as “no-show” or “same-day cancellation.” Secondary outcomes of interest were ED visits, hospitalizations, and death until December 2017.
Results: Of 527 scheduled visits, 55 (10.4%) were nonattended. Demographic and socioeconomic… More >

Yuli Y. Kim^1,2, Leah A. Goldberg², Katherine Awh², Tanmay Bhamare^1,2, David Drajpuch², Adi Hirshberg³, Sara L. Partington^1,2, Rachel Rogers⁴, Emily Ruckdeschel^1,2, Lynda Tobin¹, Morgan Venuti², Lisa D. Levine³

Congenital Heart Disease, Vol.14, No.3, pp. 470-478, 2019, DOI:10.1111/chd.12750

Abstract Objective: To assess performance of risk stratification schemes in predicting adverse cardiac outcomes in pregnant women with congenital heart disease (CHD) and to compare these schemes to clinical factors alone.
Design: Single‐center retrospective study.
Setting: Tertiary care academic hospital.
Patients: Women ≥18 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating CHD who delivered between 1998 and 2014. CARPREG I and ZAHARA risk scores and modified World Health Organization (WHO) criteria were applied to each woman.
Outcome Measures: The primary outcome was defined by ≥1 of the following: arrhyth‐ mia, heart failure/pulmonary edema, transient ischemic attack, stroke,… More >

Christopher J. Petit¹, Athar M. Qureshi², Andrew C. Glatz³, Courtney E. McCracken¹, Michael Kelleman¹, George T. Nicholson⁴, Jeffery J. Meadows⁵, Shabana Shahanavaz⁶, Jeffrey D. Zampi⁷, Mark A. Law⁸, Joelle A. Pettus¹, Bryan H. Goldstein⁹

Congenital Heart Disease, Vol.14, No.3, pp. 341-349, 2019, DOI:10.1111/chd.12737

Abstract Clinical research in the treatment of patients with congenital heart disease (CHD) is limited by the wide variety of CHD manifestations and therapeutic options as well as the generally low incidence of CHD. The availability of comprehensive, contemporary outcomes studies is therefore limited. This inadequacy may result in a lack of data‐driven medical decision making. In 2013, clinician scientists at two centers begana researchcollaboration,the Congenital Catheterization Research Collaborative (CCRC). Over time, the CCRC has grown to include nine cardiac centers from across the United States, with a common data coordinating center. The CCRC seeks to generate high‐quality, contemporary, statistically robust,… More >

Joseph T. Poterucha¹, Saraschandra Vallabhajosyula², Alexander C. Egbe², Joseph S. Krien³, Devon O. Aganga⁴, Kimberly Holst⁵, Adele W. Golden⁶, Joseph A. Dearani⁵, Sheri S. Crow⁴

Congenital Heart Disease, Vol.14, No.2, pp. 193-200, 2019, DOI:10.1111/chd.12717

Abstract Background: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD).
Methods: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admis‐ sion were examined to determine the relationship between VIS score and poor out‐ come of early mortality, early morbidity, or complication related morbidity.
Results: Overall, 1040 ACHD patients… More >

Nayan T. Srivastava^1,2, Roger Hurwitz³, W. Aaron Kay⁴, George J. Eckert⁵, Alisha Kuhlenhoelter⁶, Nicole DeGrave², Eric S. Ebenroth^2,6

Congenital Heart Disease, Vol.14, No.2, pp. 176-184, 2019, DOI:10.1111/chd.12698

Abstract Objective: For over 20 years, we have followed a cohort of patients who underwent the Mustard procedure for d‐transposition of the great arteries. The current study follows the same cohort from our last study in 2007 to reassess their functional ca‐ pacity and quality of life.
Participants: Of the original 45 patients, six patients have required cardiac transplant and 10 patients have died, including two of the transplanted patients. Twenty‐five of the remaining patients agreed to participate in this current study.
Design: Patients underwent comparable testing to the previous studies when possi‐ ble including exercise stress testing, echocardiography, MRI or… More >

Neil C. Zaki^1,2, Michael S. Kelleman¹, W. James Parks^1,3, Timothy C. Slesnick^1,3, Michael E. McConnell^1,3, Matthew E. Oster^1,3

Congenital Heart Disease, Vol.14, No.2, pp. 140-146, 2019, DOI:10.1111/chd.12692

Abstract Objective: Gated cardiac MRI offers the most detailed and accurate noninvasive method of assessing cardiac anatomy, particularly in patients with complex congenital heart disease. The proposed benefits of using cMRI as a routine screening tool in the Fontan population include early recognition of asymptomatic, postoperative anatomic and physiologic changes. In 2011, we therefore instituted at our center a recommended practice of cMRI screening in patients with Fontan physiology at 3 and 8 years postFontan operation. The purpose of this study was to determine the impact of this standardized practice of cMRI screening on the clinical management of a Fontan population.
… More >

Georges Ephrem¹, Camden Hebson², Anitha John³, Estella Moore⁴, Maan Jokhadar⁴, Ryan Ford⁵, Gruschen Veldtman⁶, Yoav Dori⁷, Michelle Gurvitz^8,9, Brian Kogon¹⁰, Adrienne Kovacs¹¹, Meghan Roswick, Michael McConnell¹², Wendy M. Book⁴, Fred Rodriguez III^4,12

Congenital Heart Disease, Vol.14, No.2, pp. 128-137, 2019, DOI:10.1111/chd.12685

Abstract The initial “Frontiers in Fontan Failure” conference in 2015 in Atlanta, Georgia, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with “Failing Fontan” physiology. Four types of “Fontan Failure” were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan proce‐ dure “imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation.” The purpose of the second “Frontiers in Fontan Failure” was to… More >