Jennifer P. Lundine^1,2, Robert Dempster^3,4, Kirby Carpenito⁵, Holly Miller‐Tate⁵, Wendelin Burdo‐Hartman^4,6, Elizabeth Halpin², Omar Khalid^4,5

Congenital Heart Disease, Vol.13, No.5, pp. 706-712, 2018, DOI:10.1111/chd.12636

Abstract Background: Swallowing dysfunction is a known complication for infants with complex congenital heart disease (CHD), but few studies have examined swallowing outcomes following the hybrid procedure for stage 1 palliation in children with single ventricle physiology.
Objectives: (1) Identify the incidence of aspiration in all infants with single ventricle physiology who underwent the hybrid procedure and (2) Compare results of clinical bedside and instrumental swallowing evaluations to examine the predictive value of a less invasive swallowing assessment for this population of high‐risk infants.
Methods: This was a retrospective cohort chart review study. All patients with single‐ventricle physiology who underwent the… More >

Doris P. Yimgang¹, John D. Sorkin², Charles F. Evans³, Danielle S. Abraham¹, Geoffrey L. Rosenthal^1,4

Congenital Heart Disease, Vol.13, No.4, pp. 533-540, 2018, DOI:10.1111/chd.12622

Abstract Introduction: Angiotensin converting enzyme inhibitors are commonly prescribed medications after the Norwood procedure. There are little data that can be used to determine if angiotensin converting enzyme inhibitors improve interstage outcomes in children with single ventricle defects. The objective of this study was to investigate the relationship between angiotensin converting enzyme inhibitors and interstage failure among infants born with hypoplastic left heart syndrome.
Methods: We conducted a retrospective cohort study using data from the National Pediatric Cardiology Quality Improvement Collaborative database (collected between 2008 and 2015). We used logistic regression models to assess the exposure-outcome associations and propensity score matching… More >

Laura S. Kverneland^1,2, Peter Kramer², Stanislav Ovroutski²

Congenital Heart Disease, Vol.13, No.2, pp. 181-193, 2018, DOI:10.1111/chd.12570

Abstract Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients’ characteristics, Fontan modifications employed, early mortality, long-term survival and frequency of relevant complications was extracted. Ultimately, thirty-one studies published by the largest surgical centers… More >

Christopher R. Broda, Katherine B. Salciccioli, Keila N. Lopez, Peter R. Ermis, Douglas S. Moodie, Heather A. Dickerson

Congenital Heart Disease, Vol.14, No.6, pp. 885-894, 2019, DOI:10.1111/chd.12856

Abstract Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with con‐ genital heart disease (CHD). Little is known about the adult HS population with CHD.
Objective: To describe the outcomes and sociodemographics of the adult CHD popu‐ lation with HS.
Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.
Results: Sixty‐two patients metinclusion criteria.Median agewas 22.7 [IQR19.6‐30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap… More >

Brooke T. Davey¹, Olga H. Toro‐Salazar¹, Naomi Gauthier^2,3, Anne Marie Valente², Robert W. Elder⁴, Fred M. Wu², Norman Berman³, Phyllis Pollack⁵, Ji Hyun Lee¹, Rahul H. Rathod²

Congenital Heart Disease, Vol.14, No.6, pp. 1013-1023, 2019, DOI:10.1111/chd.12854

Abstract Introduction: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures.
Methods: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for pa‐ tients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low‐risk vs high‐risk Fontan patients across regional programs.
Results: Fifty‐six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available… More >

Arene Butto¹, Laura Mercer‐Rosa¹, Christopher Teng¹, Carrie Daymont², Jonathan Edelson¹, Jennifer Faerber¹, Erika Mejia¹, Chitra Ravishankar¹, Meryl S. Cohen¹

Congenital Heart Disease, Vol.14, No.6, pp. 1058-1065, 2019, DOI:10.1111/chd.12843

Abstract Objective: Children with single ventricle cardiac disease (SVCD) have poor growth in early life. Tube‐assisted feeding (TF) is used to improve weight gain, but its impact on long‐term growth remains unknown. We sought to compare the longitudinal growth of SVCD patients receiving TF after initial cardiac surgery with those fed entirely by mouth.
Design: We conducted a retrospective cohort study of SVCD patients who under‐ went initial surgical palliation between 1999 and 2009. We defined TF as the use of nasogastric, gastrostomy, or jejunostomy TF. We compared maximal attained growth z‐scores for each year of life between TF and non‐TF… 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 >

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 >

Jennifer K. Peterson¹, Shaun P. Setty^1,2, Jessica H. Knight³, Amanda S. Thomas⁴, James H. Moller⁵, Lazaros K. Kochilas^4,6

Congenital Heart Disease, Vol.14, No.5, pp. 854-863, 2019, DOI:10.1111/chd.12823

Abstract Objective: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long‐term outcomes in patients with T21 and SV palliation.
Design: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anom‐ aly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ… More >