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 >

Hieu Lan Nguyen^1,2, Quang Tan Phan^3,4, Linh Huynh Dinh², Hieu Ba Tran², Hoyoun Won⁴, Julian Johny Thottian^4,5, Dung Doan Duc¹, Truong Nguyen Quang³, Sang Wook Kim⁴

Congenital Heart Disease, Vol.13, No.4, pp. 584-593, 2018, DOI:10.1111/chd.12613

Abstract Objective: To evaluate the efficacy and safety of Nit-Occlud Le VSD Coil versus Duct Occluders ^ for percutaneous closure of perimembranous ventricular septal defect (pmVSD).
Introduction: VSD closure using conventional pmVSD occluders has been largely abandoned because of an unacceptable high rate of complete heart block (CHB). The advantages of Duct Occluders and VSD Coil are supposed to reduce the drawbacks of previous devices, especially CHB complications.
Method: Patients underwent percutaneous pmVSD closure were divided into Coil group (using VSD Coil, n = 71) and DO group (using Duct Occluders, n = 315). Patient demographics, clinical presentations, echocardiography… More >

Kevin C. Harris¹, Christine Voss¹, Kathryn Rankin², Basmina Aminzadah², Ross Gardner¹, Andrew S. Mackie²

Congenital Heart Disease, Vol.13, No.4, pp. 563-570, 2018, DOI:10.1111/chd.12612

Abstract Objective: Individuals with congenital heart disease (CHD) may be at higher risk of acquired cardiovascular disease than the general population due to their underlying physiology and/or surgical sequelae. We sought to assess the prevalence of cardiovascular disease risk factors in youth and adults with CHD.
Methods: We assessed cardiovascular health as per the Cardiovascular Health in Ambulatory Care Research Team (CANHEART) health index in patients with CHD aged 15+ years who attended cardiology outpatient clinics. Participants self-reported smoking behavior, fruit and vegetable consumption, physical activity, and whether they had diabetes and hypertension. Individual health indices were categorized… More >

Michele M. Pasierb, Josiah M. Penalver, Margaret M. Vernon, Bhawna Arya

Congenital Heart Disease, Vol.13, No.4, pp. 571-577, 2018, DOI:10.1111/chd.12611

Abstract Background: Accurate prenatal diagnosis of congenital heart disease (CHD) allows for appropriate delivery and postnatal management. Geographic constraints limit access to fetal cardiology subspecialists. In our approach, general pediatric cardiologists are first line in regional prenatal cardiac screening. We aim to demonstrate the utility of this approach in diagnosing CHD requiring cardiac interventions within 30 days of life.
Methods: This is a retrospective review of fetal echocardiograms performed at Seattle Children’s Hospital regional cardiology sites (SCH-RC) from December 2008 to December 2015. Referrals to Seattle Children’s Hospital Prenatal Program (SCH-PNP) were evaluated for referral timing, indication, diagnostic… More >

Jonathan N. Menachem¹, Edo Y. Birati², Payman Zamani², Anjali T. Owens², Pavan Atluri³, Christian A. Bermudez³, David Drajpuch², Stephanie Fuller⁴, Yuli Y. Kim², Christopher E. Mascio⁴, Vikram Palanivel², J. Eduardo Rame², Joyce Wald², Michael A. Acker³, Jeremy A. Mazurek²

Congenital Heart Disease, Vol.13, No.4, pp. 492-498, 2018, DOI:10.1111/chd.12606

Abstract Background: Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Identification of disease progression and timing of referral for advanced therapies is often delayed. However, increased awareness and understanding of ACHD and improvements in the approach to treatment have led to improved outcomes. Pulmonary hypertension (PH) is a common barrier to HT. In ACHD, the approach to PH and HT is quite complicated, given the anatomic heterogeneity and lower prevalence and experience. However, in some cases, PH is a result of elevated systemic filling pressures and low output.
Methods: We… More >