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 >

Andrew D. Spearman¹, Steven J. Kindel¹, Ronald K. Woods², Salil Ginde^1,3

Congenital Heart Disease, Vol.14, No.6, pp. 1199-1206, 2019, DOI:10.1111/chd.12828

Abstract Background: Hypoxia is a common and sometimes severe morbidity of single ven‐ tricle congenital heart disease (CHD). Creation of an arteriovenous fistula (AVF) is occasionally performed for patients after superior or total cavopulmonary connec‐ tion (SCPC or TCPC) in an attempt to improve oxygen saturations. Despite previ‐ ous reports, AVF creation is a rare palliation with inadequately defined benefits and risks. We sought to determine changes in peripheral oxygen saturation (SpO₂) and risk of adverse event after AVF creation in children with single ventricle CHD at our institution.
Methods: We conducted a retrospective chart review of patients with a history… More >

Line Marie Holst¹, Faridis Serrano², Lara Shekerdemian², Hanne Berg Ravn¹, Danielle Guffey³, Nancy S. Ghanayem², Sonia Monteiro⁴

Congenital Heart Disease, Vol.14, No.6, pp. 1207-1213, 2019, DOI:10.1111/chd.12827

Abstract Objective: To investigate the impact of feeding mode on neurodevelopmental outcomes in children with congenital heart defects.
Design: A retrospective cohort study of 208 children with congenital heart disease (CHD), who had surgery from 1 January 2013 until 31 December 2016 at Texas Children’s Hospital, Houston, TX, US.
Settings: University Hospital, Developmental Outcome Clinic.
Outcomes measures: Standardized cognitive scores were assessed with Capute Scales and motor development with Revised Gesell Developmental Schedules. We analyzed anthropometrics, mode of feeding, surgical complexity, syndrome, and gen‐ der as predictors of developmental outcomes at four time points: hospital discharge, and 6, 12, and 24… More >

Uri Pollak^1,2,3,4,*, Tatyana Ruderman^5,*, Sharon Borik‐Chiger^5,6, David Mishaly^5,7, Alain Serraf^5,7, Amir Vardi^5,8

Congenital Heart Disease, Vol.14, No.6, pp. 968-977, 2019, DOI:10.1111/chd.12825

Abstract Objective: The final common pathway of single ventricle patients is the Fontan procedure. Among the immediate postoperative complications is acute hepatic injury presented by marked elevation of liver enzymes (alanine transaminase [ALT] and aspartate transaminase [AST]). We aimed to determine the contribution of blood products transfusion to acute hepatic injury.
Design: Single center retrospective cohort study.
Setting: Pediatric Cardiac Intensive Care Unit at a tertiary medical center.
Patients: Ninety‐nine pediatric patients undergoing the Fontan procedure between January 2009 and December 2016.
Interventions: None.
Measurements and Main Results: Out of the four types of blood products, transfusion of platelets was found… More >

Rachel L. Hansen^1,2, Iman Naimi³, Hongyue Wang¹, Nader Atallah^3,4, Frank Smith^3,4, Craig Byrum^3,4, Daniel Kveselis^3,4, Glenn Leonard^1,5, Rajiv Devanagondi¹, Matthew Egan^3,4

Congenital Heart Disease, Vol.14, No.6, pp. 1037-1045, 2019, DOI:10.1111/chd.12788

Abstract Objective: Evaluate long‐term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS).
Background: Long‐term data following BPV is limited to small, single center studies.
Methods: BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis.
Results: Among 254 patients, mean age at BPV was 3.8 years (range 1 day‐67 years), initial PS catheter gradient was 56 mm Hg (IQR 40‐70), 19% had… More >

Aura A. Sanchez^1,2, Sara K. Sexson Tejtel¹, Myriam E. Almeida‐Jones^1,3, Douglas K. Feagin¹, Carolyn A. Altman¹, Ricardo H. Pignatelli¹

Congenital Heart Disease, Vol.14, No.6, pp. 1024-1031, 2019, DOI:10.1111/chd.12787

Abstract Objective: Children with Kawasaki disease (KD) with persistent coronary artery aneurysms (CAAs) can develop chronic vasculopathy and subsequent myocardial ischemia. Early detection of this process is challenging. Myocardial deformation anal‐ ysis can detect early alterations in myocardial performance. We aim to determine whether there are differences in myocardial deformation between KD patients with and without CAAs.
Design: This is a cross‐sectional study of 123 echocardiograms performed on 103 children with KD. Myocardial deformation was measured with two‐dimensional speckle tracking (2DSTE). The echocardiograms were divided into groups according to the KD phase in which they were performed: acute, subacute, and convalescent/… More >