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, by understanding the pathophysiology, treatment… 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 healthy volunteers with… 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 ACHD patients… 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 to articulate a… 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 antibody level” (TS)… More >

Nayan T. Srivastava^1,2, Roger Hurwitz³, W. Aaron Kay⁴, George J. Eckert⁵, Alisha Kuhlenhoelter⁶, Nicole DeGrave², Eric S. Ebenroth^2,6

Congenital Heart Disease, Vol.14, No.2, pp. 176-184, 2019, DOI:10.1111/chd.12698

Abstract Objective: For over 20 years, we have followed a cohort of patients who underwent the Mustard procedure for d‐transposition of the great arteries. The current study follows the same cohort from our last study in 2007 to reassess their functional ca‐ pacity and quality of life.
Participants: Of the original 45 patients, six patients have required cardiac transplant and 10 patients have died, including two of the transplanted patients. Twenty‐five of the remaining patients agreed to participate in this current study.
Design: Patients underwent comparable testing to the previous studies when possi‐ ble including exercise stress testing, echocardiography, MRI or… More >

Jun Oyamada¹, Chisato Shimizu¹, Jihoon Kim², Matthew R. Williams^1,3, Eileen Png⁴, Martin L. Hibberd⁴, Adriana H. Tremoulet^1,3, James C. Perry^1,3, Jane C. Burns^1,3

Congenital Heart Disease, Vol.14, No.2, pp. 213-220, 2019, DOI:10.1111/chd.12696

Abstract Background: We previously described the association of genetic variants in calcium channel genes and susceptibility to Kawasaki disease (KD), an acute, self‐limited vas‐ culitis, and the most common cause of acquired cardiac disease in children. Abnormal repolarization of cardiomyocytes and changes in T wave morphology have been re‐ ported in KD but have not been studied systematically.
Methods: We analyzed acute and convalescent ECG T wave morphology in two inde‐ pendent cohorts of KD subjects and studied the association between bifid T waves and genetic variants in previously reported genes with SNVs associated with cardiac repolarization.
Results: Bifid T waves… More >

Matthew Lewis¹, William Whang², Angelo Biviano², Kathleen Hickey², Hasan Garan², Marlon Rosenbaum¹

Congenital Heart Disease, Vol.14, No.2, pp. 207-212, 2019, DOI:10.1111/chd.12695

Abstract Background: Catheter ablation is commonly performed to treat atrial arrhythmias in adult congenital heart disease (ACHD). Despite the frequency of ablations in the ACHD population, predictors of recurrence remain poorly defined.
Objective: We sought to determine predictors of arrhythmia recurrence in ACHD patients following catheter ablation for atrial arrhythmias.
Methods: We performed a retrospective study of all catheter ablations for atrial arrhythmias performed in ACHD patients between January 12, 2005 and February 11, 2015 at our institution. Prespecified exposures of interest and time from ablation to recurrence were determined via chart review.
Results: Among 124 patients (mean age: 45 years)… More >

Alessia Callegari^1,2,4, Rhoia Neidenbach², Ornella Milanesi¹, Biagio Castaldi¹, Martin Christmann⁴, Masamichi Ono³, Jan Müller², Peter Ewert², Alfred Hager²

Congenital Heart Disease, Vol.14, No.2, pp. 147-155, 2019, DOI:10.1111/chd.12694

Abstract Aim: The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and qual‐ ity of life is unknown and objective of this study.
Methods: This multicenter retrospective/cross‐sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connec‐ tion). Resting spirometry, cardiopulmonary exercise tests, and quality‐of‐life assess‐ ment (SF‐36 questionnaire) were performed between 2003 and 2015.
Results: Overall, mean forced expiratory volume… More >

Neil C. Zaki^1,2, Michael S. Kelleman¹, W. James Parks^1,3, Timothy C. Slesnick^1,3, Michael E. McConnell^1,3, Matthew E. Oster^1,3

Congenital Heart Disease, Vol.14, No.2, pp. 140-146, 2019, DOI:10.1111/chd.12692

Abstract Objective: Gated cardiac MRI offers the most detailed and accurate noninvasive method of assessing cardiac anatomy, particularly in patients with complex congenital heart disease. The proposed benefits of using cMRI as a routine screening tool in the Fontan population include early recognition of asymptomatic, postoperative anatomic and physiologic changes. In 2011, we therefore instituted at our center a recommended practice of cMRI screening in patients with Fontan physiology at 3 and 8 years postFontan operation. The purpose of this study was to determine the impact of this standardized practice of cMRI screening on the clinical management of a Fontan population.
… More >