Jill M. Steiner¹, James N. Kirkpatrick¹, Susan R. Heckbert², James Sibley³, James A. Fausto³, Ruth A. Engelberg³, J. Randall Curtis³

Congenital Heart Disease, Vol.13, No.5, pp. 721-727, 2018, DOI:10.1111/chd.12638

Abstract Objective: Overall health care resource utilization by adults with congenital heart disease has increased dramatically in the past two decades, yet little is known about utilization patterns at the end of life. The objective of this study is to better under‐ stand the patterns and influences on end‐of‐life care intensity for adults with con‐ genital heart disease.
Methods: We identified a sample of adults with congenital heart disease (n = 65), cancer (n = 10 784), or heart failure (n = 3809) who died between January 2010 and December 2015, cared for in one multi‐hospital health care… More >

Cheryl L. Brosig^1,2, Laurel Bear^1,2, Sydney Allen¹, Pippa Simpson¹, Liyun Zhang¹, Michele Frommelt^1,2, Kathleen A. Mussatto²

Congenital Heart Disease, Vol.13, No.5, pp. 700-705, 2018, DOI:10.1111/chd.12632

Abstract Background and Objectives: Children with congenital heart disease (CHD) are at risk for neurodevelopmental (ND) delays. The purpose of this study is to compare the ND testing results of children with CHD at 2 and 4 years of age and determine if rates of ND delays change over time.
Methods: Children with CHD completed the Bayley Scales of Infant Development‐III (BSID‐III) at 2 years of age, and standardized neuropsychological measures at 4 years. Scores were compared with test norms and were classified as: average (within one SD of test mean); at risk (1‐2 SDs from the test… More >

Ketina Arslani^1*, Lukas Notz^1*, Marzena Zurek¹, Matthias Greutmann², Markus Schwerzmann³, Judith Bouchardy⁴, Reto Engel⁵, Christine Attenhofer Jost⁶, Daniel Tobler¹

Congenital Heart Disease, Vol.13, No.5, pp. 678-684, 2018, DOI:10.1111/chd.12627

Abstract Background: In adults with congenital heart disease (CHD) and atrial arrhythmias, recommendations for thromboprophylaxis are vague and evidence is lacking. We aimed to identify factors that influence decision-making in daily practice.
Methods: From the Swiss Adult Congenital HEart disease Registry (SACHER) we identified 241 patients with either atrial fibrillation (Afib) or atrial flutter/intraatrial reentrant tachycardia (Aflut/ IART). The mode of anticoagulation was reviewed. Logistic regression models were used to assess factors that were associated with oral anticoagulation therapy.
Results: Compared with patients with Aflut/IART, patients with Afib were older (51 ± 16.1 vs 37 ± 16 years, P… More >

Juan Lizandro Rodríguez‐Hernández¹, Fayna Rodríguez‐González², Marta Riaño‐Ruiz³, Efrén Martínez‐Quintana^1,4

Congenital Heart Disease, Vol.13, No.5, pp. 655-662, 2018, DOI:10.1111/chd.12620

Abstract Introduction: Hyperuricemia has been associated with cardiovascular risk factors but it remains controversial if uric acid is an independent predictor of cardiac mortality.
Methods: A total of 503 CHD patients (457 nonhypoxemic and 46 hypoxemic) and 772 control patients fulfilled inclusion criteria. Demographic, clinical, and analytical data [serum uric acid and 24h urine uric acid levels, N‐terminal pro‐B‐type natriuretic peptide (NT‐pro‐BNP), and C‐reactive‐protein (CRP) concentrations] were studied. Survivals curves to determine cardiac death and arterial thrombosis in CHD patients were also examined.
Results: Noncyanotic and cyanotic CHD patients had significant higher serum uric acid concentration (5.2 ± 1.5 vs 4.9 ± 1.3mg/dL,… More >

Mi Kyoung Song¹, Gi Beom Kim¹, Eun Jung Bae¹, Young Ah Lee¹, Hyun-Young Kim², Seung-Kee Min³, Jung Hee Kim⁴, Jae-Kyung Won⁵

Congenital Heart Disease, Vol.13, No.4, pp. 608-616, 2018, DOI:10.1111/chd.12625

Abstract Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a possible cause, and a strong link between cyanotic congenital heart disease and these tumors has been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation at a single tertiary center.
Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children’s Hospital.
Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence… More >

William N. Evans^1,2, Ruben J. Acherman^1,2, Dean Berthoty³, Gary A. Mayman^1,2, Michael L. Ciccolo^1,4, Sergio A. Carrillo^1,4, Humberto Restrepo^1,2

Congenital Heart Disease, Vol.13, No.4, pp. 624-627, 2018, DOI:10.1111/chd.12623

Abstract Patients: We reviewed all patients evaluated at our center with situs solitus, levocardia, and a right aortic arch that were born between January 2000 and January 2018. Results: From our databases, we identified 204 patients. We excluded patients with a double aortic arch from analysis. Of the 204 patients, 103 (50%) were male. Of the 204 patients, 95 (47%) had an isolated right aortic arch. Of the 95 with an isolated right aortic arch, 4 (4%) had chromosome 22q11.2 deletion syndrome, and 89 (94%) had a vascular ring. Of the 204 patients, 109 (53%) had a… More >

Sherry L. Farr¹, Karrie F. Downing^1,2, Tiffany Riehle-Colarusso¹, Ginnie Abarbanell^1,3

Congenital Heart Disease, Vol.13, No.4, pp. 633-639, 2018, DOI:10.1111/chd.12621

Abstract Objective: To examine how cognitive and motor limitations in children with heart disease are associated with education and participation in extracurricular activities.
Design: Using 2009–2010 parent-reported data from the National Survey of Children with Special Health Care Needs (NS-CSHCN), we examined prevalence of five functional limitations (learning/ concentration, communication, self-care, gross motor skills, and fine motor skills) by diagnosed heart disease status using chi-square tests and multivariable logistic regression. Among CSHCN with heart disease, we examined the associations between severity of each functional limitation and missing ≥11 days of school in the past year, receiving special education… More >

Elizabeth C. Caris¹, Nicole Dempster², Gil Wernovsky³, Yongjie Miao², Melissa Moore-Clingenpeel², Trent Neely², Rachel Fonseca², Holly Miller-Tate², Robin Allen², Samantha Fichtner², Jamie Stewart², Clifford L. Cua²

Congenital Heart Disease, Vol.13, No.4, pp. 528-532, 2018, DOI:10.1111/chd.12619

Abstract Objectives: Siblings of children with chronic medical conditions endorse a lower quality of life compared to age-matched peers. Caregiver and sibling-self report of adjustment are often discordant. Congenital heart disease significantly affects family life. To date, there have been no studies addressing the functioning of siblings of children with hypoplastic left heart syndrome, one of the most severe forms of congenital heart disease. The goal of this study was to assess the impact of hypoplastic left heart syndrome on sibling’s quality of life as well as the caregiver’s perception of this effect.
Study Design: Cross-sectional study using… More >

Timur Mese¹, Baris Guven², Murat Muhtar Yilmazer¹, Cem Karadeniz³, Rahmi Ozdemir¹, Onder Doksoz¹

Congenital Heart Disease, Vol.13, No.4, pp. 506-511, 2018, DOI:10.1111/chd.12616

Abstract Background: Mean platelet volume (MPV), platecrit, and platelet distribution width (PDW) are markers of platelet activation. Previous studies have found that platelet activation occurs in patients with pulmonary arterial hypertension. Platelet indices including MPV, PDW, and platecrit have not been studied in children with congenital heart disease associated pulmonary arterial hypertension (APAH-CHD) who survived and those who died.
Objective: The objective of this study to investigate the value of platelet indices with clinical and hemodynamic indicators predicting the disease severity and survival in children with APAH-CHD.
Methods: This was a nested case-control study. MPV, platecrit, and PDW levels… More >

Natasha G. Boyes¹, Michael K. Stickland², Stephanie Fusnik¹, Elizabeth Hogeweide¹, Josie T.J. Fries¹, Mark J. Haykowsky³, Chantelle L. Baril¹, Shonah Runalls¹, Ashok Kakadekar⁴, Scott Pharis⁴, Charissa Pockett⁴, Timothy J. Bradley⁴, Kristi D. Wright⁵, Marta Erlandson¹, Corey R. Tomczak¹

Congenital Heart Disease, Vol.13, No.4, pp. 578-583, 2018, DOI:10.1111/chd.12614

Abstract Children with congenital heart disease are at risk for developing increased arterial stiffness and this may be modulated by physical activity. Objective: To compare arterial stiffness in high- and low-physically active children with congenital heart disease and healthy age- and sex-matched controls.
Patients: Seventeen children with congenital heart disease (12 ± 2 years; females = 9), grouped by low- and high-physical activity levels from accelerometry step count values, and 20 matched controls (11 ± 3 years; females = 9) were studied.
Outcome Measures: Carotid-radial pulse wave velocity was assessed with applanation tonometry to determine arterial stiffness. Body composition… More >