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 >

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 >

Andrew D. Spearman

Congenital Heart Disease, Vol.12, No.6, pp. 828-833, 2017, DOI:10.1111/chd.12543

Abstract A genetic basis of congenital heart disease (CHD) has been known for decades. In addition to the sequence of the genome, the contribution of epigenetics to pediatric cardiology is increasingly recognized. Multiple epigenetic mechanisms, including DNA methylation, histone modification, and RNA-based regulation, are known mediators of cardiovascular disease, including both development and progression of CHD and its sequelae. Basic understanding of the concepts of epigenetics will be essential to all pediatric cardiologists in order to understand mechanisms of pathophysiology, pharmacotherapeutic concepts, and to understand the role of epigenetics in precision medicine. More >

Juan Villafane¹, Thomas C. Edwards², Karim A. Diab³, Gary M. Satou⁴, Elizabeth Saarel⁵, Wyman W. Lai⁶, Gerald A. Serwer⁷, Peter P. Karpawich⁸, Russell Cross⁹, Russell Schiff¹⁰, Devyani Chowdhury¹¹, Thomas J. Hougen¹²

Congenital Heart Disease, Vol.12, No.6, pp. 762-767, 2017, DOI:10.1111/chd.12523

Abstract Objective: The objective of this study was to develop quality metrics (QMs) relating to the ambulatory care of children after complete repair of tetralogy of Fallot (TOF).
Design: A workgroup team (WT) of pediatric cardiologists with expertise in all aspects of ambulatory cardiac management was formed at the request of the American College of Cardiology (ACC) and the Adult Congenital and Pediatric Cardiology Council (ACPC), to review published guidelines and consensus data relating to the ambulatory care of repaired TOF patients under the age of 18 years. A set of quality metrics (QMs) was proposed by the… More >

Raysa Morales-Demori

Congenital Heart Disease, Vol.12, No.6, pp. 820-827, 2017, DOI:10.1111/chd.12521

Abstract Congenital heart disease (CHD) is present in approximately 50% of patients with trisomy 21 (T21) and Turner syndrome (TS). According to the American Academy of Pediatrics, every patient with these genetic disorders should have a postnatal echocardiogram. T21 is usually associated with atrioventricular (30%–60%), atrial (16%-21%), or ventricular septal defects (14%–27%). TS is usually associated with left-sided heart disease. However, the spectrum of CHD in these genetic disorders is wider than those mentioned lesions. More cardiac surgical procedures are offered to these patients and that has influenced positively their life expectancy for some CHD conditions. More >

Joseph M. Krepp¹, Mary J. Roman², Richard B. Devereux², Adrienne Bruce¹, Siddharth K. Prakash³, Shaine A. Morris⁴, Dianna M. Milewicz³, Kathryn W. Holmes⁵, William Ravekes⁵, Ralph V. Shohet⁶, Reed E. Pyeritz⁷, Cheryl L. Maslen⁸, Barbara L. Kroner⁹, Kim A. Eagle¹⁰, Liliana Preiss⁹, GenTAC Investigators^*, Federico M. Asch¹

Congenital Heart Disease, Vol.12, No.6, pp. 740-745, 2017, DOI:10.1111/chd.12520

Abstract Background: Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe, leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously performed.
Methods: We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions. Clinical and echocardiographic data from the GenTAC Registry were compared between 17… More >

Michael D. Seckeler¹, Katri Typpo², Jendar Deschenes², Ruth Higgins³, Ricardo Samson¹, Peter Lichtenthal⁴

Congenital Heart Disease, Vol.12, No.6, pp. 815-819, 2017, DOI:10.1111/chd.12517

Abstract Objective: To determine the accuracy of a continuous cardiac output monitor (FloTrac sensor) for measuring cardiac index in children with congenital heart disease undergoing cardiac catheterization. Cardiac index is a critical hemodynamic parameter measured during catheterizations in children with congenital heart disease. This has been challenging to measure accurately and many clinicians rely on predictive equations for calculating cardiac index.
Design: Prospective, nonrandomized trial.
Setting: Tertiary care congenital heart center.
Patients: Consecutive participants ≤18 years old undergoing clinically indicated cardiac catheterizations from September 2014 through August 2015.
Interventions: Oxygen consumption was measured using the Vmax Encore 229 monitor attached to… More >

Constanze Pfitzer^1,2,3, Paul C. Helm⁴, Hannah Ferentzi^1,5, Lisa-Maria Rosenthal¹, Ulrike M. M. Bauer^4,6, Felix Berger^1,3,7, Katharina R. L. Schmitt^1,3

Congenital Heart Disease, Vol.12, No.6, pp. 787-793, 2017, DOI:10.1111/chd.12515

Abstract Objective: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender.
Design: Cross-sectional registry study.
Setting: We analyzed data from patients with CHD born between 1996 and 2015.
Patients: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic… More >

Brian A. Boe¹, Mark D. Norris², Jeffrey D. Zampi², Albert P. Rocchini², Gregory J. Ensing²

Congenital Heart Disease, Vol.12, No.6, pp. 733-739, 2017, DOI:10.1111/chd.12514

Abstract Objective: We sought to identify a time during cardiac ejection when the instantaneous pressure gradient (IPG) correlated best, and near unity, with peak-to-peak systolic ejection gradient (PPSG) in patients with congenital aortic stenosis. Noninvasive echocardiographic measurement of IPG has limited correlation with cardiac catheterization measured PPSG across the spectrum of disease severity of congenital aortic stenosis. A major contributor is the observation that these measures are inherently different with a variable relationship dependent on the degree of stenosis.
Design: Hemodynamic data from cardiac catheterizations utilizing simultaneous pressure measurements from the left ventricle (LV) and ascending aorta (AAo)… More >

Dmitry Tumin^1,2, Helen Chou¹, Don Hayes Jr^1,3,4, Joseph D. Tobias^1,2,5, Mark Galantowicz^6,7, Patrick I. McConnell^6,7

Congenital Heart Disease, Vol.12, No.6, pp. 794-799, 2017, DOI:10.1111/chd.12513

Abstract Objective: Adults with congenital heart disease may require heart transplantation for end-stage heart failure. Whereas heart transplantation potentially allows adults with congenital heart disease to resume their usual activities, employment outcomes in this population are unknown. Therefore, we investigated the prevalence and predictors of work participation after heart transplantation for congenital heart disease.
Design: Retrospective review of a prospective registry.
Setting: United Network for Organ Sharing registry of transplant recipients in the United States.
Patients: Adult recipients of first-time heart transplantation with a primary diagnosis of congenital heart disease, performed between 2004 and 2015.
Interventions: None.
Outcome measures: Employment status reported… More >