Yajuan Zhang, Jun Yan^*, Qiang Wang, Shoujun Li, Jing Sun, Shuo Dong, Jiachen Li

Congenital Heart Disease, Vol.16, No.4, pp. 383-392, 2021, DOI:10.32604/CHD.2021.015479 - 19 April 2021

Abstract Background: The long-term outcomes of patients treated with a Kawashima procedure and keeping the antegrade pulmonary blood flow (AnPBF) in single-ventricle (SV) and interrupted inferior vena cava (IVC) heart disease is still uncertain as yet. Methods: We investigated 18 patients who underwent the Kawashima procedure with SV physiology and an interrupted IVC between January 2009 and June 2018, perioperative, operative and postoperative characteristics were recorded. Results: A total of 18 patients underwent the Kawashima procedure at a median age of 2.7 years (range 0.5–24.7 years), of which 12 (66.7%) were male and 6 (33.3%) were female. The… More >

Sarah Pradhan^1,*, Eileen Broomall², Russel Hirsch¹

Congenital Heart Disease, Vol.16, No.4, pp. 393-396, 2021, DOI:10.32604/CHD.2021.015257 - 19 April 2021

Abstract The differential diagnosis for irritability in children is broad. In patients with congenital heart disease, one must strongly consider cardiac etiologies such as low cardiac output or elevated central venous pressure (CVP). In patients with single-ventricle physiology, the second stage of palliation includes bidirectional Glenn, which involves anastomosis of the superior vena cava to the pulmonary artery resulting in volume offloading of the single systemic ventricle. Typically, early in the post-operative period, patients may experience a headache due to the acute increase in CVP, and symptoms improve over time. Idiopathic intracranial hypertension (IIH), also known More >

Aparna Kulkarni^1,*, Richard Neugebauer², Shelby Kutty³

Congenital Heart Disease, Vol.16, No.3, pp. 299-307, 2021, DOI:10.32604/CHD.2021.014495 - 02 March 2021

Abstract Objective: The objective of this analysis is to describe the characteristics and morbidity during hospitalizations among adolescents with congenital heart disease (AdoCHD) from the Pediatric Health Information System (PHIS) database. Methods: The PHIS database was queried for all AdoCHD admissions aged 12–18 years (1/1/2004–12/31/2013). Major forms of CHD were identified by their International Classification of Diseases, ninth revision codes, further verified based on their secondary diagnosis and/or procedure codes. Patient characteristics, diagnoses, procedures and vital status were assessed. Results: In total, there were 4,267 adolescents admitted to 42 Children’s Hospitals, 58.3% were males, 24.6% single ventricle (SV)… More >

Sheetal R. Patel^1,2,*, David M. Kwiatkowski³, Adin-Cristian Andrei², Ankita Devareddy², Hangzhi Shi², Catherine D. Krawczeski⁴, Natalie Ebert⁵, Barbara J. Deal^1,2, Craig B. Langman^2,6, Bradley S. Marino^1,2

Congenital Heart Disease, Vol.15, No.4, pp. 181-195, 2020, DOI:10.32604/CHD.2020.012097 - 07 September 2020

Abstract Objectives: The Fontan operation has increased survival in patients with single-ventricle congenital heart defects. However, Fontan survivors are at risk of other organ dysfunctions, such as renal dysfunction (RD). The objectives of this study are to assess the prevalence of and potential risk factors for RD among Fontan survivors. Design, setting, and patients: We performed a two-center, cross-sectional study that included Fontan survivors evaluated in outpatient-clinics for routine follow up between 01/08-12/16. Outcome measures: The primary outcome was the presence of RD defined by estimated glomerular filtration rate (eGFR) of <90 ml/min/1.73 m² derived using the serum… More >

Carter Biewen^1,*, Hitendra Patel², Olaf Reinhartz³, Ginny Gildengorin⁴, Natalie Cvijanovich⁵

Congenital Heart Disease, Vol.15, No.4, pp. 229-238, 2020, DOI:10.32604/CHD.2020.011671 - 07 September 2020

Abstract Purpose: To summarize Fontan procedure data from our institution, UCSF Benioff Children’s Hospital Oakland, in order to better understand outcomes of our surgical and post-operative care. Basic Procedures: This is a retrospective cross-sectional chart review summarizing characteristics and outcomes of patients who underwent the Fontan procedure at our institution between 2005 and 2016. Main Findings: Sixty-five Fontan procedures were performed at our institution between 2005 and 2016, all of which were extracardiac Fontan procedures performed by the same surgical team. At the time of Fontan procedure, the mean patient age was 56.4 ± 18.7 months and mean weight… 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… 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… 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… 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… 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… More >