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,… 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… 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… 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… 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… More >

Emmanuel Akintoye¹, Gruschen R. Veldtman², William R. Miranda³, Heidi M. Connolly³, Alexander C. Egbe³

Congenital Heart Disease, Vol.14, No.2, pp. 138-139, 2019, DOI:10.1111/chd.12690

Abstract The purpose of this study was to determine the optimal age for performing Fontan operation using data from the National Inpatient Sample. Our results showed that although the Fontan operation was most commonly performed at age 2 in the United States, age 3 is the optimum age for this procedure as evident by lower rate of in‐hospital mortality, procedure‐related complications, and rate of nonroutine home discharge when procedure is performed at age 3 years. More >

Mariana M. Clavé¹, Nair Y. Maeda², Ana M. Thomaz¹, Sergio P. Bydlowski³, Antonio A. Lopes¹

Congenital Heart Disease, Vol.14, No.2, pp. 246-255, 2019, DOI:10.1111/chd.12688

Abstract Background: Ideally, vasodilator therapies for pulmonary arterial hypertension (PAH) should have a favorable impact on markers of vascular dysfunction, in addition to their known effects on hemodynamics, cardiac function, and patient’s physical capacity.
Methods: We analyzed circulating (plasma) markers of endothelial and platelet activation/dys‐ function (enzyme‐linked immunoassays) in the specific setting of advanced PAH associated with congenital heart disease, during the course of sildenafil and tadalafil therapies. Thirty‐one patients were enrolled (age 10‐54 years), most of them with chronic hypoxemia and elevated hematocrit. Drugs were administered orally for 6 months (sildenafil [n = 16], 20 mg t.i.d.;… More >

Soham Dasgupta¹, Shae Anderson¹, Michael Kelleman², Ritu Sachdeva¹

Congenital Heart Disease, Vol.14, No.2, pp. 230-235, 2019, DOI:10.1111/chd.12687

Abstract Introduction: In the pediatric Appropriate Use Criteria (AUC), abnormal electrocardiogram (ECG) in an asymptomatic patient has been rated as an “Appropriate” indication for transthoracic echocardiogram (TTE). We hypothesized that the yield of abnormal findings on TTE for this indication will be low.
Methods: All asymptomatic patients (≤ 18 years) from January 1, 2015 to December 31, 2017 who underwent initial outpatient evaluation at our center and had a TTE ordered for an abnormal ECG, were included. Clinic records were reviewed to obtain ECG and TTE findings.
Results: Of the 199 study patients, 13 (6.5%) had abnormal findings. Incomplete… More >

Samantha C. Butler^1,4, Anjali Sadhwani^1,4, Christian Stopp², Jayne Singer^1,3,4, David Wypij^2,5,6, Carolyn Dunbar‐Masterson², Janice Ware^1,3,4, Jane W. Newburger^2,5

Congenital Heart Disease, Vol.14, No.2, pp. 236-245, 2019, DOI:10.1111/chd.12686

Abstract Objective: Mortality rates for children with congenital heart disease (CHD) have significantly declined, resulting in a growing population with associated neurodevelopmental disabilities. American Heart Association guidelines recommend systematic developmental screening for children with CHD. The present study describes results of inpatient newborn neurodevelopmental assessment of infants after open heart surgery.
Outcome measures: We evaluated the neurodevelopment of a convenience sample of high‐risk infants following cardiac surgery but before hospital discharge using an adaptation of the Newborn Behavioral Observation. Factor analysis examined relationships among assessment items and consolidated them into domains of development.
Results: We assessed 237 infants at… More >

Georges Ephrem¹, Camden Hebson², Anitha John³, Estella Moore⁴, Maan Jokhadar⁴, Ryan Ford⁵, Gruschen Veldtman⁶, Yoav Dori⁷, Michelle Gurvitz^8,9, Brian Kogon¹⁰, Adrienne Kovacs¹¹, Meghan Roswick, Michael McConnell¹², Wendy M. Book⁴, Fred Rodriguez III^4,12

Congenital Heart Disease, Vol.14, No.2, pp. 128-137, 2019, DOI:10.1111/chd.12685

Abstract The initial “Frontiers in Fontan Failure” conference in 2015 in Atlanta, Georgia, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with “Failing Fontan” physiology. Four types of “Fontan Failure” were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan proce‐ dure “imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation.” The purpose of the second “Frontiers… More >