Rahul H. Rathod^1,2, Brittney Jurgen^1,2, Rose A. Hamershock³, Kevin G. Friedman^1,2, Audrey C. Marshall^1,2, Mihail Samnaliev⁴, Dionne A. Graham³, Kathy Jenkins^1,2, James E. Lock^1,2, Andrew J. Powell^1,2

Congenital Heart Disease, Vol.12, No.6, pp. 768-776, 2017, DOI:10.1111/chd.12508

Abstract Background: Standardized Clinical Assessment and Management Plans (SCAMPs) are a quality improvement initiative designed to reduce unnecessary utilization, decrease practice variation, and improve patient outcomes. We created a novel methodology, the SCAMP managed episode of care (SMEOC), which encompasses multiple encounters to assess the impact of the arterial switch operation (ASO) SCAMP on total costs.
Methods: All ASO SCAMP patients (dates March 2009 to July 2015) were compared to a control group of ASO patients (January 2001 to February 2009). Patients were divided into “younger” (<2 years) and “older” (2–18 years) subgroups. Utilization included all cardiology visits,… More >

Hitesh Agrawal^1,2, Oriana K. Wright³, Kathleen E. Carberry^1,4, S. Kristen Sexson Tejtel^1,2, Carlos M. Mery^1,5, Silvana Molossi^1,2

Congenital Heart Disease, Vol.12, No.6, pp. 721-725, 2017, DOI:10.1111/chd.12473

Abstract Background: Long-term outcome data on patients with anomalous aortic origin of coronary arteries (AAOCA) is sparse and they are often managed in a nonuniform manner. There is subjective perception of anxiety and unmet needs in these patients and families.
Methods: An online survey of 13 questions was sent to 74 families of patients with AAOCA between May and October 2015. Descriptive statistics were performed.
Results: A total of 31 (47%) families responded. Of these, 27 expressed the need to interact with other patients/families with AAOCA. The majority were interested in either face-to-face meetings (77%) or online support groups… More >

Hitesh Agrawal^1,2, Carlos M. Mery^1,3, Patrick E. Day⁴, S. Kristen Sexson Tejtel^1,2, E. Dean McKenzie^1,3,5, Charles D. Fraser Jr^1,3, Athar M. Qureshi^1,2, Silvana Molossi^1,2

Congenital Heart Disease, Vol.12, No.5, pp. 610-614, 2017, DOI:10.1111/chd.12511

Abstract Background: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in young athletes in the USA. Long-term outcome data for these patients are lacking to date. There is insufficient knowledge on the best approach to these patients and they are managed in a nonuniform manner.
Methods: An online survey of 15 questions regarding management of AAOCA was sent out to 198 cardiac healthcare providers. The goal was to define gaps in knowledge to justify a dedicated scientific forum for discussion of AAOCA. Descriptive statistics were performed.
Results: A total of… More >

Silvana Molossi, Hitesh Agrawal

Congenital Heart Disease, Vol.12, No.5, pp. 607-609, 2017, DOI:10.1111/chd.12505

Abstract The clinical evaluation of patients with an anomalous aortic origin of a coronary artery (AAOCA), a congenital abnormality of the origin or course of a coronary artery that arises from the aorta, is challenging given its first presentation being sudden cardiac arrest in about half of the patients. Symptoms of chest pain, shortness of breath and syncope during exertion should be of concern in evaluating young athletes and nonathletes. The lack of abnormal signs on the physical exam and electrocardiogram further adds to the difficulty in establishing the diagnosis. Additional imaging with echocardiography, computed tomography More >

Julie A. Brothers^1,2

Congenital Heart Disease, Vol.12, No.5, pp. 600-602, 2017, DOI:10.1111/chd.12497

Abstract Anomalous aortic origin of a coronary artery (AAOCA) occurs when both coronary arteries arise from the same aortic sinus from a single ostium or two separate ostia. While most coronary anomalies are benign, the two most common subtypes that predispose to sudden cardiac death in the young are interarterial anomalous right coronary artery and interarterial anomalous left coronary artery. Practitioners face many challenges with AAOCA. Diagnosing patients may be difficult because children and adolescents are often asymptomatic and first presentation may be sudden death or sudden cardiac arrest. Risk stratification is also challenging as determining More >

Elise D. Pieterman^1,2, Ricardo P. J. Budde², Danielle Robbers-Visser^1,2, Ron T. van Domburg³, Willem A. Helbing^1,2

Congenital Heart Disease, Vol.12, No.5, pp. 561-569, 2017, DOI:10.1111/chd.12484

Abstract Objective: Follow-up of right ventricular performance is important for patients with congenital heart disease. Cardiac magnetic resonance imaging is optimal for this purpose. However, observerdependency of manual analysis of right ventricular volumes limit its use. Knowledge-based reconstruction is a new semiautomatic analysis tool that uses a database including knowledge of right ventricular shape in various congenital heart diseases. We evaluated whether knowledge-based reconstruction is a good alternative for conventional analysis.
Design: To assess the inter- and intra-observer variability and agreement of knowledge-based versus conventional analysis of magnetic resonance right ventricular volumes, analysis was done by two observers… More >

Tyler H. Harris¹, Mark Adler², Sharon M. Unti³, Mary E. McBride⁴

Congenital Heart Disease, Vol.12, No.4, pp. 546-553, 2017, DOI:10.1111/chd.12483

Abstract Background: Training guidelines state that pediatricians should be able to diagnose, manage, and triage patients with heart disease. Acutely ill cardiac patients present infrequently and with high acuity, yet residents receive less exposure to acute cardiac conditions than previous generations. Trainees must learn to manage these situations despite this gap. Simulation has been used successfully to train learners to provide acute care. We hypothesized that a simulation-based cardiac curriculum would improve residents’ ability to manage cardiac patients.
Methods: Pediatric residents completed 4 simulation cases followed by debriefing and a computer presentation reviewing the learning objectives. Subjects returned… More >

Waldemar F. Carlo¹, Steven T. Clark², Santiago Borasino³, Jeffrey A. Alten³

Congenital Heart Disease, Vol.12, No.4, pp. 540-545, 2017, DOI:10.1111/chd.12482

Abstract Objective: Acute kidney injury (AKI) is a frequent complication after cardiopulmonary bypass (CBP) for cardiac surgery in neonates. It is unclear if exposure to computed tomography angiography (CTA) in the preoperative period increases the risk of AKI. We hypothesized a short interval between CTA and CPB surgery would be associated with higher rates of AKI in infants.
Design: In this single center retrospective review of patients between 2012 and 2015, neonates less than one month old were analyzed if they had CTA prior to cardiac surgery with CPB. Baseline, demographic, fluid balance, and laboratory data was analyzed.… More >

Shankar Baskar¹, Philippa Horne², Samantha Fitzsimmons³, Philip R. Khoury¹, Joseph Vettukattill⁴, Koichiro Niwa⁵, Teiji Agaki⁶, Mark Spence⁷, Hisanori Sakazaki⁸, Gruschen Veldtman¹

Congenital Heart Disease, Vol.12, No.4, pp. 512-519, 2017, DOI:10.1111/chd.12481

Abstract Background: Patients with Eisenmenger syndrome have a shorter lifespan than the general population. A significant proportion develop arrhythmia and some, sudden death.
Objective: The aims of this study were to characterize the frequency, type and effects of arrhythmias in adult patients with Eisenmenger’s syndrome and to identify risk factors for arrhythmias.
Methods: This retrospective study included patients aged ≥18 years of age with Eisenmenger’s syndrome from three institutions. Arrhythmias were noted from electrocardiograms and Holter study reviews.
Results: A total of 167 patients, 96 females, 63 males (gender not available in 9 patients) were included in this study. The… More >

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 >