Nicholas Y. Tan¹, Christine H. Attenhofer Jost¹, Murray D. Polkinghorne¹, Emily R. Vargas², David O. Hodge², Joseph A. Dearani³, Samuel J. Asirvatham^1,4, Heidi M. Connolly¹, Christopher J. McLeod¹

Congenital Heart Disease, Vol.14, No.6, pp. 1157-1165, 2019, DOI:10.1111/chd.12841

Abstract Introduction: Mechanisms and risk factors for cerebrovascular accidents (CVAs) in Ebstein’s anomaly (EA) are not well understood; hence, we aimed to clarify these in a large cohort of EA patients.
Methods: Patients with a confirmed diagnosis of EA were retrospectively reviewed. Baseline characteristics were compared between patients with and without a prior history of CVA using logistic regression modeling. Cox regression analysis was used to identify predictors of CVA following initial evaluation. CVA incidence from birth and following tricuspid valve surgery were estimated using the Kaplan‐Meier method.
Results: Nine hundred sixty‐eight patients (median age 21.1 years, 41.5% male) were included,… More >

Munes Fares¹, Paul J. Critser², Maria J. Arruda¹, Carolyn M. Wilhelm¹, Mantosh S. Rattan³, Sean M. Lang^2,4, Tarek Alsaied^2,4

Congenital Heart Disease, Vol.14, No.6, pp. 1166-1175, 2019, DOI:10.1111/chd.12840

Abstract Pharmacologic stress cardiovascular magnetic resonance (PSCMR) is a wellestablished and reliable diagnostic tool for evaluation of coronary artery disease in the adult population. Stress imaging overall and PSCMR in particular is less utilized in the pediatric population with limited reported data. In this review, we highlight the potential use of PSCMR in specific pediatric cohorts with congenital and acquired heart disease, and we review the reported experience. A suggested protocol is presented in addition to two case examples of patients with Kawasaki disease where PSCMR aided decision making. More >

Bradley Johnson^1,2, Matthew Buelow^1,2, Michael Earing^1,2, Scott Cohen^1,2, Peter Bartz^1,2, Salil Ginde^1,2

Congenital Heart Disease, Vol.14, No.6, pp. 895-900, 2019, DOI:10.1111/chd.12839

Abstract Objective: As adults with congenital heart disease (CHD) grow older, preoperative screening for coronary artery disease (CAD) may be indicated prior to CHD surgery. Data regarding the indications for preoperative CAD screening in this population are limited. Current practice is to follow guidelines for patients with valvular heart dis‐ ease; however, the risk for CAD in certain congenital heart diagnoses may be higher than the general population. This study aimed to assess the results of preoperative CAD screening in patients prior to CHD surgery.
Design: Retrospective study.
Setting: Single tertiary center.
Patients: Patients ≥35 years that had CHD surgery from… More >

Sok‐Leng Kang¹, Aimee Armstrong², Gregor Krings³, Lee Benson¹

Congenital Heart Disease, Vol.14, No.6, pp. 1046-1057, 2019, DOI:10.1111/chd.12838

Abstract Three‐dimensional rotational angiography (3D‐RA) enables volumetric imaging through rotation of the C‐arm of an angiographic system and real‐time 3D recon‐ struction during cardiac catheterization procedures. In the field of congenital heart disease (CHD), 3D‐RA has gained considerable traction, owing to its capability for en‐ hanced visualization of spatial relationships in complex cardiac morphologies and real time image guidance in an intricate interventional environment. This review provides an overview of the current applications, strengths, and limitations of 3D‐RA acquisi‐ tion in the management of CHD and potential future directions. In addition, issues of dosimetry, radiation exposure, and optimization strategies will be… More >

Rohit S. Loomba¹, Saul Flores²

Congenital Heart Disease, Vol.14, No.6, pp. 1176-1184, 2019, DOI:10.1111/chd.12837

Abstract Objective: The main goal of this study is to examine the variation in vasoactive agent prescription patterns across a large cohort of patients. In addition, we sought to determine the association between the number of vasoactive agents used during admissions and characteristics of admissions utilizing varying numbers of vasoactive agents.
Methods: This was a multi-institutional, cross-sectional study of the pediatric health information system database of patients who underwent congenital heart surgery and received vasoactive agents from 2004 to 2015. The international classification of disease-9 (ICD-9) codes were used to select admissions to those only pertaining to cardiac patients. The vasoactive… More >

Paolo D’Ambrosio^1,2, Derek Tran^1,2, Charlotte E. Verrall^3,4, Chantal Attard⁵, Maria Fiatarone Singh^6,7, Julian Ayer^3,4,8, Yves d’Udekem^5,9,10, Stephen Twigg^2,11, David S. Celermajer^1,2,12, Rachael Cordina^1,2,5

Congenital Heart Disease, Vol.14, No.6, pp. 987-995, 2019, DOI:10.1111/chd.12836

Abstract Objective and Patients: This study aimed to characterize bone mineral density abnormalities and pathophysiological associations in young adults living with a Fontan circulation.
Design: Participants underwent bone mineral density measurement using dual‐energy X‐ray absorptiometry and serum biochemical analysis, cardiopulmonary exercise and strength testing and transthoracic echocardiography.
Results: In our cohort (n = 28), 29% had osteopenic‐range bone mineral density and one patient was osteoporotic (average hip t score: −0.6 ± 1.1; spine t score: −0.6 ± 0.9). Four patients (14%) had z scores < −2.0. Parathyroid hormone levels were increased compared with laboratory median (6.1 ± 3.5 vs 4 pmol/L,… More >

Andrew S. Kim¹, Colleen M. Witzenburg², Mark Conaway³, Jeffrey E. Vergales¹, Jeffrey W. Holmes^2,4, Thomas J. L’Ecuyer¹, Peter N. Dean¹

Congenital Heart Disease, Vol.14, No.6, pp. 1185-1192, 2019, DOI:10.1111/chd.12834

Abstract Background: Children with hypoplastic left heart syndrome (HLHS) have risk for mortality and/or transplantation. Previous studies have associated right ventricular (RV) indices in a single echocardiogram with survival, but none have related serial measurements to outcomes. This study sought to determine whether the trajectory of RV indices in the first year of life was associated with transplant‐free survival to stage 3 palliation (S3P).
Methods: HLHS patients at a single center who underwent stage 1 palliation (S1P) between 2000 and 2015 were reviewed. Echocardiographic indices of RV size and function were obtained before and following S1P and stage 2 palliation (S2P).… More >

Benjamin Zielonka¹, Yuli Y. Kim^2,3, Gregory E. Supple², Sara L. Partington^2,3, Emily S. Ruckdeschel^2,3, Francis E. Marchlinski², David S. Frankel²

Congenital Heart Disease, Vol.14, No.6, pp. 931-938, 2019, DOI:10.1111/chd.12833

Abstract Objective: Atrial arrhythmias and atrioventricular valve regurgitation (AVVR) are common causes of morbidity among adults with congenital heart disease (ACHD). The impact of rhythm control on AVVR in this population is unknown. We sought to determine whether a rhythm control strategy is associated with greater freedom from AV valve surgery than a rate control strategy.
Design: Patients evaluated by both ACHD and electrophysiology specialists at a single academic center were screened for atrial arrhythmias and at least moderate‐severe AVVR. Clinical and electrographic data were abstracted. All echocardiograms were in‐ terpreted by a single echocardiographer blinded to treatment strategy. Patients were… More >

Alexander Lemmer¹, Lisa VanWagner^1,2, Zaira Gasanova³, Steve Helmke^4,5, Gregory T. Everson^4,5, Daniel Ganger¹

Congenital Heart Disease, Vol.14, No.6, pp. 978-986, 2019, DOI:10.1111/chd.12831

Abstract Background & Aims: Fontan surgery for single ventricle congenital heart disease leads to Fontan‐associated liver disease (FALD). Typical laboratory tests, imaging, and histopathology cannot predict clinical severity in FALD. HepQuant SHUNT is a proprietary serum test of hepatic function and physiology that has not yet been evaluated in FALD.
Methods: Fourteen adult FALD patients at a single urban tertiary care center who underwent a Fontan procedure in childhood received HepQuant SHUNT testing between September 2015 and April 2018. The HepQuant SHUNT disease severity index (DSI) assesses global liver function and physiology from systemic and portal hepatic filtration rates (HFRs, clearances… More >

Jeremy M. Steele^1,2, Rukmini Komarlu², Sarah Worley³, Tarek Alsaied¹, Christopher Statile¹, Francine G. Erenberg²

Congenital Heart Disease, Vol.14, No.6, pp. 1193-1198, 2019, DOI:10.1111/chd.12829

Abstract Objective: Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success.
Design: Infants born between July 2015 and August 2017 with ≥2 left heart obstructive lesions with no prior interventions were identified (n = 19). Patients with aortic or mitral valve (MV) atresia and critical aortic stenosis were excluded. Initial echocardiograms were reviewed for aortic, MV, tricuspid valve annulus size,… More >