Stephanie M. Ford¹, Matthew T. McPheeters², Yves T. Wang³, Pei Ma¹³, Shi Gu³, James Strainic⁴, Christopher Snyder⁴, Andrew M. Rollins³, Michiko Watanabe², Michael W. Jenkins²

Congenital Heart Disease, Vol.12, No.3, pp. 322-331, 2017, DOI:10.1111/chd.12443

Abstract Background: The relationship between changes in endocardial cushion and resultant congenital heart diseases (CHD) has yet to be established. It has been shown that increased regurgitant flow early in embryonic heart development leads to endocardial cushion defects, but it remains unclear how abnormal endocardial cushions during the looping stages might affect the fully septated heart. The goal of this study was to reproducibly alter blood flow in vivo and then quantify the resultant effects on morphology of endocardial cushions in the looping heart and on CHDs in the septated heart.
Methods: Optical pacing was applied to create… More >

Waldemar F. Carlo¹, James F. Cnota², Robert J. Dabal³, Jeffrey B. Anderson²

Congenital Heart Disease, Vol.12, No.3, pp. 315-321, 2017, DOI:10.1111/chd.12442

Abstract Objective: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) was established in 2008 to improve outcomes of hypoplastic left heart syndrome (HLHS) during the interstage period. They evaluated changes in patient variables and practice variation between early and late eras.
Design: Data including demographic, operative, discharge, and follow-up variables from the first 100 patients (6/2008–1/2010) representing 18 centers were compared with the most recent 100 patients (1/2014–11/2014) from these same centers.
Results: Prenatal diagnosis increased from 69% to 82% (P = .05). There were no differences in gestational age or weight at Norwood. A composite of any preoperative… More >

Paul Cooper¹, Morgan Hindes², Timothy M. Maul^3,4, Stephen C. Cook⁵

Congenital Heart Disease, Vol.12, No.3, pp. 309-314, 2017, DOI:10.1111/chd.12441

Abstract Objective: Medical residents are exposed to increasing numbers of adults with congenital heart disease (ACHD). While inadequate ACHD knowledge may lead to inappropriate practice, this educational deficit has not been investigated. Our aim was to analyze residents’ attitudes, perceived ability, and knowledge of ACHD medicine.
Design, Methods, Outcome Measures: A single center, multiprogram cross-sectional study was conducted in 2015 using an electronic survey to assess 472 medical residents’ perceived knowledge and self-assessed skills related to ACHD medicine. Demographic data obtained included age, gender, level of training and program type.
Results: The survey was completed by 25% of surveyed… More >

Maryanne Caruana¹, Victor Grech²

Congenital Heart Disease, Vol.12, No.3, pp. 301-308, 2017, DOI:10.1111/chd.12439

Abstract Objectives: To determine overall and reintervention-free survival for repaired Maltese tetralogy of Fallot patients and to investigate the potential impact of gender, age at repair, genetic syndromes, previous palliation, and type of repair on these outcomes.
Design: All 130 tetralogy of Fallot patients born before the end of 1997 included in the local database were extracted. Surgical repair type, age at repair and operative survival were analyzed among the 103/130 repaired patients. Kaplan–Meier survival analyses were performed on the 75 repair survivors with complete follow-up data (mean follow-up 26.37 ± 9.27 (range 9.95–51.21) years).
Results: Patients born after… More >

Antonio R. Mott¹, Steven R. Neish², Melissa Challman³, Timothy F. Feltes⁴

Congenital Heart Disease, Vol.12, No.3, pp. 294-300, 2017, DOI:10.1111/chd.12438

Abstract Background: The treatment of children with cardiac disease is one of the most prevalent and costly pediatric inpatient conditions. The design of inpatient medical services for children admitted to and discharged from noncritical cardiology care units, however, is undefined. North American Pediatric Cardiology Programs were surveyed to define noncritical cardiac care unit models in current practice.
Method: An online survey that explored institutional and functional domains for noncritical cardiac care unit was crafted. All questions were multi-choice with comment boxes for further explanation. The survey was distributed by email four times over a 5-month period.
Results: Most programs… More >

Xinghua Gu¹, Qiuwang Zhang², Hourong Sun¹, Jianchun Fei¹, Xiquan Zhang¹, Michael J. Kutryk²

Congenital Heart Disease, Vol.12, No.3, pp. 289-293, 2017, DOI:10.1111/chd.12437

Abstract Objective: To present our experience in transcatheter closure of calcified patent ductus arteriosus (PDA) in older adult patients, which has rarely been reported.
Patients: From 2009 to 2014, a total of 16 patients (median age 58 years) with calcified PDA underwent transcatheter closure in our center. All patients were symptomatic with major symptoms being exertional dyspnea (in 12), palpitations (in 8), and fatigue (in 5). A continuous murmur was heard in all patients. The median ductus diameter was 4 mm (range 3–7 mm). The median Qp/Qs was 1.6 (range 1.4–2.9).
Interventions: Transcatheter closure was performed for all patients.… More >

Janet M. Simsic, Kirby-Rose Carpenito, Kristin Kirchner, Stephanie Peters, PNP-AC, Holly Miller-Tate, Brian Joy, Mark Galantowicz

Congenital Heart Disease, Vol.12, No.3, pp. 275-281, 2017, DOI:10.1111/chd.12435

Abstract Objective: Enteral feeding is associated with decreased infection rates, decreased mechanical ventilation, decreased hospital length of stay, and improved wound healing. Enteral feeding difficulties are common in congenital heart disease. Our objective was to develop experience-based newborn feeding guidelines for the initiation and advancement of enteral feeding in the cardiothoracic intensive care unit.
Design: This is a retrospective analysis of a quality improvement project.
Setting: This quality improvement project was performed in a cardiothoracic intensive care unit.
Patients: Newborns admitted to the cardiothoracic intensive care unit for cardiac surgery from January 2011 to May 2015 were retrospectively reviewed.
Intervention: Newborn… More >

Kari L. Turkowski¹, David J. Tester^2,3, J. Martijn Bos^2,4, Kristina H. Haugaa⁵, Michael J. Ackerman^2,3,4

Congenital Heart Disease, Vol.12, No.2, pp. 226-235, 2017, DOI:10.1111/chd.12462

Abstract Background: Arrhythmogenic cardiomyopathy (ACM) is a heritable disease characterized by fibrofatty replacement of cardiomyocytes, has a prevalence of approximately 1 in 5000 individuals, and accounts for approximately 20% of sudden cardiac death in the young (≤35 years). ACM is most often inherited as an autosomal dominant trait with incomplete penetrance and variable expression. While mutations in several genes that encode key desmosomal proteins underlie about half of all ACM, the remainder is elusive genetically. Objective: Here, whole exome sequencing (WES) was performed with genomic triangulation in an effort to identify a novel explanation for a… More >

Anushree Agarwal, Fatima Samad, Lindsey Kalvin, Michelle Bush, A. Jamil Tajik

Congenital Heart Disease, Vol.12, No.2, pp. 143-152, 2017, DOI:10.1111/chd.12453

Abstract Introduction: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital disease that frequently remains undiagnosed until adulthood, especially when there is an absence of other congenital anomalies. Adults with ccTGA may remain asymptomatic and their diagnosis could be missed on initial evaluation, or it could be diagnosed incidentally as an evaluation of murmur. We aim to report the different presentations of ccTGA in eight adult patients and review the key features required to suspect the diagnosis during an initial visit.
Cases: We present some illustrative cases of ccTGA patients who had diverse presentations ranging… More >

R. Thomas Collins II^1,2, Margaret G. Collins³, Michael L. Schmitz^2,4, Justin T. Hamrick⁴

Congenital Heart Disease, Vol.12, No.2, pp. 133-142, 2017, DOI:10.1111/chd.12447

Abstract Williams syndrome (WS) is a congenital, multisystem disorder affecting the cardiovascular, connective tissue, and central nervous systems in 1 in 10 000 live births. Cardiovascular involvement is the most common cause of morbidity and mortality in patients with WS, and noninvasive and invasive procedures are common. Sudden cardiovascular collapse in patients with WS is a well-known phenomenon, especially in the peri-procedural period. Detailed guidelines for peri-procedural management of patients with WS are limited. The goal of this review is to provide thoughtful, safe and effective management strategies for the peri-procedural care of patients with WS More >