Brian A. Boe, Sharon L. Cheatham, Aimee K. Armstrong, Darren P. Berman, Joanne L. Chisolm, John P. Cheatham

Congenital Heart Disease, Vol.14, No.2, pp. 297-304, 2019, DOI:10.1111/chd.12728

Abstract Objective: We sought to describe the leaflet morphology variation in the Melody Transcatheter Pulmonary Valve (TPV) and evaluate associated outcomes. The Melody TPV is constructed from harvested bovine jugular venous valves which have been rigorously tested. Natural anatomic leaflet variations are seen in the Melody TPV but have not been evaluated.
Design: A Melody TPV leaflet morphology classification system was devised after reviewing a subset of photographed and implanted TPVs. All images were blindly re‐ viewed by implanters and classified. Midterm hemodynamic outcomes and complica‐ tions of the Melody TPVs were compared by leaflet morphology.
Results: Photographed Melody… More >

Jesse Lee, Doaa Abdullah Shahbah, Howaida El‐Said, Rodrigo Rios, Kanishka Ratnayaka, John Moore

Congenital Heart Disease, Vol.14, No.2, pp. 288-296, 2019, DOI:10.1111/chd.12726

Abstract Background: In the modern era, results of the arterial switch operation (ASO) for transposition of the great arteries are excellent. However, because of the LeCompte maneuver, there may be a propensity for development of pulmonary artery stenosis. We encountered atypical complications of pulmonary artery stenting in patients after the ASO, including aorto‐pulmonary fistula and coronary compression.
Methods: We performed a 10‐year retrospective review of catheterizations per‐ formed in patients after ASO in our institution with a focus on adverse events.
Results: Diagnostic and interventional catheterizations were performed in 47 pa‐ tients. In 29 patients, 37 interventional procedures performed,… More >

Charlotte A. Houck^1,2, Reinder Evertz³, Christophe P. Teuwen¹, Jolien W. Roos‐Hesselink¹, Janneke A. E. Kammeraad⁴, Anthonie L. Duijnhouwer³, Natasja M. S. de Groot³, Ad J. J. C. Bogers²

Congenital Heart Disease, Vol.14, No.2, pp. 280-287, 2019, DOI:10.1111/chd.12724

Abstract Objective: Outcomes after surgical repair of complete atrioventricular septal defect (cAVSD) have improved. With advancing age, the risk of development of dysrhythmias may increase. The aims of this study were to (1) examine development of sinus node dysfunction (SND), atrial and ventricular tachyarrhythmias, and (2) study progression of atrioventricular conduction abnormalities in young adult patients with repaired cAVSD.
Study design: In this retrospective multicenter study, 74 patients (68% female) with a cAVSD repaired in childhood were included. Patients’ medical files were evaluated for occurrence of SND, atrioventricular conduction block (AVB), atrial and ventricular tachyarrhythmias.
Results: Median age at… More >

Vanessa Marie Hormaza¹, Mark Conaway², Daniel Scott Schneider¹, Jeffrey Eric Vergales¹

Congenital Heart Disease, Vol.14, No.2, pp. 274-279, 2019, DOI:10.1111/chd.12722

Abstract Objective: Limited information is known on how right ventricular function affects outcomes after stage 2 palliation. We evaluated the impact of different right ventricular indices prior to stage 2 palliation on morbidity and mortality.
Design: Retrospective study design.
Setting: Pediatric Heart Network Single Ventricle Reconstruction Trial Public Data Set.
Patient: Any variant of stage 1 palliation and all anatomic hypoplastic left heart syndrome variants in the trial were evaluated. Echocardiograms prior to stage 2 palliation were analyzed and compared between those who failed and those who survived.
Intervention: None.
Outcome measures: Mortality was defined as death, listed for transplant, or transplanted… More >

Kailyn Anderson¹, James Cnota^2,3, Jeanne James^4,5, Erin M. Miller^2,3, Ashley Parrott³, Valentina Pilipenko^1,2, Kathryn Nicole Weaver^1,2, Amy Shikany³

Congenital Heart Disease, Vol.14, No.2, pp. 264-273, 2019, DOI:10.1111/chd.12721

Abstract Objective: To evaluate the prevalence of Noonan spectrum disorders (NSD) in a pediatric population with valvar pulmonary stenosis (vPS) and identify the clinical characteristics that differentiate those with NSD from those without NSD.
Design: A retrospective chart review of 204 patients diagnosed with vPS between 9/1/2012 and 12/1/2016 at a pediatric medical center was performed. The quantitative features of vPS, genetic diagnosis information, and phenotypic characteristics of Noonan syndrome were collected. Chi‐square test, Fisher’s exact test, t test, Wilcoxon rank‐sum test, and ANOVA were used for comparisons among the groups. Logistic regression was used to test for… More >

Camden L. Hebson¹, Michael E. McConnell², David W. Hannon³

Congenital Heart Disease, Vol.14, No.2, pp. 156-161, 2019, DOI:10.1111/chd.12720

Abstract Dysautonomia is an increasingly recognized yet still poorly understood disease within the field of pediatrics. Symptoms, including dizziness, headaches, fatigue, joint pain, anxiety, and intolerance of heat or cold, are often significant and difficult to sort, especially in terms of their relation to each other. This often leads to referral to multiple subspecialists, who then proceed to treat seemingly familiar symptoms in kind. In the authors’ experience, this leads to more frustration on the part of the patients and their physicians when symptom improvement does not follow (or can even worsen). On the other hand, More >

Ersin Çagrı Şimşek¹, Selcen Yakar Tülüce², Kamil Tülüce³, Sadık Volkan Emren², Serap Çuhadar⁴, Cem Nazlı²

Congenital Heart Disease, Vol.14, No.2, pp. 256-263, 2019, DOI:10.1111/chd.12718

Abstract Objective: The bicuspid aortic valve (BAV) is the most common congenital heart dis‐ ease. The process of aortic dilatation is not completely clear in patients with the BAV. Apelin is a peptide found at high levels in vascular endothelial cells which has a role in vascular regulation and cardiovascular function. The aim of this study was to de‐ termine the relationship between serum apelin levels and ascending aortic dilatation in adult patients with BAV.
Design: This cross‐sectional study included 62 patients with isolated BAV and to an age, gender, and body mass index‐matched control group of 58… More >

Joseph T. Poterucha¹, Saraschandra Vallabhajosyula², Alexander C. Egbe², Joseph S. Krien³, Devon O. Aganga⁴, Kimberly Holst⁵, Adele W. Golden⁶, Joseph A. Dearani⁵, Sheri S. Crow⁴

Congenital Heart Disease, Vol.14, No.2, pp. 193-200, 2019, DOI:10.1111/chd.12717

Abstract Background: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD).
Methods: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admis‐ sion were examined to determine the relationship between VIS score and poor out‐ come of early mortality, early morbidity, or complication related morbidity.
Results: Overall, 1040… More >

Jennifer L. Lapum¹, Suzanne Fredericks¹, Barbara Bailey², Terrence M. Yau^3,4, Jennifer Graham⁵, Ariane J. Marelli^6,7

Congenital Heart Disease, Vol.14, No.2, pp. 185-192, 2019, DOI:10.1111/chd.12716

Abstract Objective: The number of adults with congenital heart disease (CHD) has increased substantially because of medical advances that are extending life expectancy beyond childhood. A historical investigation is timely not only because of this significant demographic shift, but also because stakeholder engagement is increasingly important in shaping research directions. The study questions were: From the perspective of stakeholders, what is known about the medical treatment for the adult CHD cohort in Canada and how has it changed over time?
Design/Methods: We harnessed the use of historical research methods and conducted interviews with 21 key informants in order… More >

Patrick D. Evers^1,*, Tarek Alsaied^1,2,*, Jeffrey B. Anderson¹, James F. Cnota¹, Allison A. Divanovic¹

Congenital Heart Disease, Vol.14, No.2, pp. 221-229, 2019, DOI:10.1111/chd.12713

Abstract Objective: Maternal anti-Ro/SSA and anti-La/SSB antibodies can lead to fetal complete heart block (CHB). Current guidelines recommend weekly echocardiographic screening between 16 and 28 weeks gestation. Given the cost of screening and the rarity of conduction abnormalities in fetuses of mothers with low anti-Ro levels (<50 U/mL), we sought to identify a strategy that optimizes resource utilization.
Design: Decision analysis cost-utility modeling was performed for three screening paradigms: “standard screening” (SS) in which mid-gestation mothers are screened weekly, “limited screening” (LS) in which fetal echocardiograms are avoided unless the fetus develops bradycardia, and “targeted screening by maternal… More >