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 >

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 >

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 >

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 >