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., , DOI:10.32604/CHD.2020.012097

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 creatinine-based Full Age… More >

Angeles Fuertes-Moure¹, Michael Meyer^2,3, Anna-Luisa Häcker^2,3, Barbara Reiner^2,3, Leon Brudy³, Sonia Pértega-Diaz⁴, Renate Oberhoffer^2,3, Peter Ewert², Jan Müller^2,3,*

Congenital Heart Disease, Vol., , DOI:10.32604/CHD.2020.011771

Abstract Objective: Health-related quality of life (HRQoL) has become an important outcome measure for patients with congenital heart disease (CHD). The aim of this study was to evaluate the natural course of HRQoL from longitudinal assessment in children with CHD. Patients and Methods: From July 2014 to February 2020 this longitudinal study recruited 317 children with CHD (113 girls, 35.6%) aged 6 to 18 years (11.6 ± 2.9 years). HRQoL was assessed with the generic, self-reported and age-adapted KINDL^® questionnaire. During a mean follow-up period of 2.2 ± 1.3 years, 195 patients had one HRQoL reassessment, 70 two, 40 three and… More >

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

Congenital Heart Disease, Vol., , DOI:10.32604/CHD.2020.011671

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 was 16.6… More >

Naveed Rabbani^1,*, Sarah Hofman DeYoung², Ronald L. Gibson², Jeffrey Conwell¹, Jason F. Deen¹

Congenital Heart Disease, Vol., , DOI:10.32604/CHD.2020.011527

Abstract Persistent left superior vena cava (PLSVC) is a relatively common congenital venous anomaly that typically drains into the coronary sinus without hemodynamic significance. Rarely a PLSVC drains directly into the left atrium, forming a right-to-left shunt that can result in hypoxemia and potential paradoxical embolism. We present the case of a 2-year-old medically complex child on chronic mechanical ventilation with eventual diagnosis of episodic hypoxemia due to a PLSVC draining into the left atrium with position-dependent venous flow. The lesion was identified with contrast echocardiography and cardiac MRI. Subsequent occlusion with a vascular plug resulted in resolution of the child’s… More >