Shaun Mohan¹, Brady S. Moffett², Wilson Lam², Caridad de la Uz², Christina Miyake², Santiago O. Valdes², Jeffrey J. Kim²

Congenital Heart Disease, Vol.12, No.4, pp. 507-511, 2017, DOI:10.1111/chd.12478

Abstract Objective: As survivors of congenital heart disease (CHD) continue to age, healthcare utilization by this population has increased. It is unknown how often these patients utilize the emergency department (ED) at children’s hospitals and how arrhythmias play a role in their utilization of care.
Design: Using a retrospective cohort design, the Pediatric Hospital Information System (PHIS) database was investigated and we studied adults (≥18 years) with CHD (ACHD) who presented to pediatric EDs from 2004 to 2014.
Setting: Tertiary care pediatric hospitals.
Results: Of the 6310 encounters to pediatric EDs, 1594 (25%) were for arrhythmias. The median age was… More >

Takaya Hoashi¹, Toru Iwasa², Koji Kagisaki¹, Masatoshi Shimada¹, Kenichi Kurosaki², Isao Shiraishi², Hajime Ichikawa¹

Congenital Heart Disease, Vol.12, No.4, pp. 441-447, 2017, DOI:10.1111/chd.12477

Abstract Objective: To review long-term respiratory outcomes for tetralogy of Fallot and absent pulmonary valve (TOF/APV) in respiratory symptomatic populations.
Methods: Of 25 consecutive patients undergoing primary total correction for TOF/APV between 1987 and 2016, Sixteen patients (64%) with a preoperative respiratory disturbance were enrolled. The median age at operation was 1.9 months old, including 4 neonates and 12 infants. Ten patients (62.5%) preoperatively necessitated mechanical ventilator support. During operation, dilated central pulmonary arteries (cPAs) were plicated and retracted anteriorly in all patients, except for the first patient of the study cohort. VSD was completely closed, and the… More >

Karrie F. Downing^1,2, Matthew E. Oster^1,3, Sherry L. Farr¹

Congenital Heart Disease, Vol.12, No.4, pp. 497-506, 2017, DOI:10.1111/chd.12476

Abstract Objective: A substantial percentage of children with congenital heart disease (CHD) fail to transfer to adult care, resulting in increased risk of morbidity and mortality. Transition planning discussions with a provider may increase rates of transfer, yet little is known about frequency and content of these discussions. We assessed prevalence and predictors of transition-related discussions between providers and parents of children with special healthcare needs (CSHCN) and heart problems, including CHD.
Design: Using parent-reported data on 12- to 17-year-olds from the 2009–2010 National Survey of CSHCN, we calculated adjusted prevalence ratios (aPR) for associations between demographic factors… More >

Jennifer K. Peterson¹, Yanjun Chen², Danh V. Nguyen³, Shaun P. Setty¹

Congenital Heart Disease, Vol.12, No.4, pp. 520-532, 2017, DOI:10.1111/chd.12475

Abstract Objective: Despite overall improvements in congenital heart disease outcomes, racial and ethnic disparities have continued. The purpose of this study is to examine the effect of race and ethnicity, as well as other risk factors on congenital heart surgery length of stay and in-hospital mortality.
Design: From the 2012 Healthcare Cost and Utilization Project Kids Inpatient Database (KID), we identified 13 130 records with Risk Adjustment in Congenital Heart Surgery complexity scoreeligible procedures. Multivariate logistic and linear regression modeling with survey weights, stratification and clustering was used to examine the relationships between predictor variables and length of… More >

Magalie Ladouceur^1,2,3, Nadjia Kachenoura⁴, Gilles Soulat^1,3, Emilie Bollache⁴, Alban Redheuil⁴, Michel Azizi³, Christophe Delclaux³, Gilles Chatellier³, Pierre Boutouyrie^1,3, Laurence Iserin³, Damien Bonnet^2,3, Elie Mousseaux^1,3

Congenital Heart Disease, Vol.12, No.4, pp. 458-466, 2017, DOI:10.1111/chd.12472

Abstract Objectives: We aimed (1) determine if systemic right ventricle filling parameters influence systemic right ventricle stroke volume in adult patients with D-transposition of the great arteries (D-TGA) palliated by atrial switch, using cardiac magnetic resonance imaging and echocardiography, and (2) to study relationship of these diastolic parameters with exercise performance and BNP, in patients with preserved systolic systemic right ventricle function.
Design: Single-center, cross-sectional, prospective study.
Setting: In patients with D-TGA palliated by atrial switch, diastolic dysfunction of the systemic right ventricle may precede systolic dysfunction.
Methods: Forty-five patients with D-TGA and atrial switch and 45 age and sex-matched… More >

Aaron R. Prosnitz¹, Jane Leopold², Mira Irons³, Kathy Jenkins¹, Amy E. Roberts¹

Congenital Heart Disease, Vol.12, No.4, pp. 475-483, 2017, DOI:10.1111/chd.12471

Abstract Objective: To describe a group of children with co-incident pulmonary vein stenosis and SmithLemli-Opitz syndrome and to generate hypotheses as to the shared pathogenesis of these disorders.
Design: Retrospective case series.
Patients: Five subjects in a pulmonary vein stenosis cohort of 170 subjects were diagnosed with Smith-Lemli-Opitz syndrome soon after birth.
Results: All five cases were diagnosed with Smith-Lemli-Opitz syndrome within 6 weeks of life, with no family history of either disorder. All cases had pathologically elevated 7-dehydrocholesterol levels and two of the five cases had previously reported pathogenic 7-dehydrocholesterol reductase mutations. Smith-Lemli-Opitz syndrome severity scores ranged from mild… More >

Katie E. Cohen¹, Matthew W. Buelow¹, Jennifer Dixon¹, Ruta Brazauskas², Scott B. Cohen³, Michael G. Earing^1,3, Salil Ginde¹

Congenital Heart Disease, Vol.12, No.4, pp. 435-440, 2017, DOI:10.1111/chd.12470

Abstract Objective: Abnormal lung function characterized by a reduced forced vital capacity (FVC) is common in adults with repaired tetralogy of Fallot (TOF) and is associated with previous thoracotomies and sternotomies. The impact of abnormal lung function on clinical outcomes in adult patients with repaired TOF is unclear. The aim of this study was to determine the impact of abnormal lung function on the outcome of hospitalization and death in adults with repaired TOF when analyzed with other traditional cardiac risk factors.
Design: Retrospective study of adults with repaired TOF, who underwent spirometry between 2000 and 2014. FVC… More >

Sarah A. Twichell¹, Corinna J. Rea¹, Patrice Melvin², Andrew J. Capraro¹, Joshua C. Mandel¹, Michael A. Ferguson¹, Daniel J. Nigrin¹, Kenneth D. Mandl¹, Dionne Graham², Justin P. Zachariah³

Congenital Heart Disease, Vol.12, No.4, pp. 484-490, 2017, DOI:10.1111/chd.12469

Abstract Background: Recognition of high blood pressure (BP) in children is poor, partly due to the need to compute age-sex-height referenced percentiles. This study examined the change in abnormal BP recognition before versus after the introduction of an electronic health record (EHR) app designed to calculate BP percentiles with a training lecture.
Methods and results: Clinical data were extracted on all ambulatory, non-urgent encounters for children 3–18 years old seen in primary care, endocrinology, cardiology, or nephrology clinics at an urban, academic hospital in the year before and the year after app introduction. Outpatients with at least 1… More >

Tarek Alsaied¹, Jouke P. Bokma², Mark E. Engel³, Joey M. Kuijpers², Samuel P. Hanke¹, Liesl Zuhlke⁴, Bin Zhang⁵, Gruschen R. Veldtman⁶

Congenital Heart Disease, Vol.12, No.4, pp. 393-398, 2017, DOI:10.1111/chd.12468

Abstract Background: Reported long-term outcome measures vary greatly between studies in Fontan patients making comprehensive appraisal of mortality hazard challenging. We sought to create a clinical risk score to assist monitoring of Fontan patients in the outpatient setting.
Methods: A systematic review was conducted to evaluate risk factors for long-term (beyond the first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90 patients were included or ≥20 long-term mortalities we reported. Risk factors for long-term mortality were determined. The pooled hazard ratios were used to create components of a clinical score for long-term mortality… More >

Amitabh Thacoor

Congenital Heart Disease, Vol.12, No.4, pp. 430-434, 2017, DOI:10.1111/chd.12467

Abstract Marfan syndrome is a multisystemic genetic condition affecting connective tissue. It carries a reduced life expectancy, largely dependent on cardiovascular complications. More common cardiac manifestations such as aortic dissection and aortic valve incompetence have been widely documented in the literature. Mitral valve prolapse (MVP), however, has remained poorly documented. This article aims at exploring the existing literature on the pathophysiology and diagnosis of MVP in patients with Marfan syndrome, defining its current management and outlining the future developments surrounding it. More >