Wenjie Dong^1,2,#, Zhibin Hong^1,#, Aqian Wang², Kaiyu Jiang², Hai Zhu², Fu zhang², Zhaoxia Guo², Hongling Su^2,*, Yunshan Cao^3,*

Congenital Heart Disease, Vol.19, No.3, pp. 325-339, 2024, DOI:10.32604/chd.2024.052267 - 26 July 2024

Abstract Background: Current guidelines for managing pulmonary arterial hypertension (PAH) recommend a risk stratification approach. However, the applicability and accuracy of these strategies for PAH associated with congenital heart disease (PAH-CHD) require further validation. This study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year follow-up. Additionally, new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD patients. Methods: This retrospective study included 126 PAH-CHD patients. Clinical and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s… More > Graphic Abstract

LIHUA CHEN^1,2,#, HONGTIAN ZHANG^1,2,#, YONG XIA^1,2, KAI SUN¹, WENJIN CHEN¹, RUXIANG XU^1,2,*

BIOCELL, Vol.47, No.5, pp. 1065-1073, 2023, DOI:10.32604/biocell.2023.025924 - 10 April 2023

Abstract Purpose: The present study summarized cases of children (n = 32) with medulloblastoma (MB) who were treated using stratified therapy based on risk grading and also discussed the factors affecting prognosis. Methods: According to the risk stratification criteria, the cases were divided into the following four risk groups: low, standard, high, and very high. The 5-year overall survival (OS) and progression-free survival (PFS) rates were summarized. Further, the effects on the prognosis of tumor size, tumor stage, degree of resection, treatment mode, metastatic recurrence, molecular typing, and risk stratification were analyzed. Results: In the present study, following… More >

Liping Wang, Mingjie Zhang, Xi Chen, Yachang Pang, Jiaqi Liu, Zhuoming Xu^*

Congenital Heart Disease, Vol.17, No.1, pp. 87-97, 2022, DOI:10.32604/CHD.2022.018452 - 26 October 2021

Abstract Background: Reversibility of pulmonary hypertension (PH) is closely related to the treatment options for and prognosis of children with congenital heart disease. Objective: We combined patient-specific clinical features including diagnosis, age and echocardiographic results, and biomarkers of pulmonary vascular dysfunction to explore the noninvasive methods that can be used to accurately evaluate the reversibility of pulmonary hypertension in congenital heart disease (PH-CHD). Methods: Based on the preoperative systolic pulmonary arterial pressure (sPAP), 70 CHD patients were divided into normal, PH-CHD suspected, and confirmed groups. Additionally, biomarkers of circulating endothelial cells (CECs), endothelin-1 (ET-1), and endothelial nitric oxide… More >

Alexander C. Egbe, William R. Miranda, Patricia A. Pellikka, Sorin V. Pislaru, Barry A. Borlaug, Srikanth Kothapalli, Sindhura Ananthaneni, Harigopal Sandhyavenu, Maria Najam, Mohamed Farouk Abdelsamid, Heidi M. Connolly

Congenital Heart Disease, Vol.14, No.4, pp. 657-664, 2019, DOI:10.1111/chd.12768

Abstract Background: We hypothesized that echocardiographic indices of right ventricular to pulmonary artery (RV‐PA) coupling were comparable to cardiac magnetic resonance imaging (CMRI)‐derived RV volumetric indices in predicting disease severity in chronic pulmonary regurgitation (PR).
Methods: Patients with ≥ moderate PR (2003‐2015) with and without prior CMRI scans were enrolled into the study cohort and validation cohort, respectively. Endpoint was to determine the association between noninvasive RV‐PA coupling in‐ dices (tricuspid annular plane systolic excursion/right ventricular systolic pressure [TAPSE/RVSP] and fractional area change [FAC]/RVSP ratio) and markers of disease severity, and compared this association to that of CMRI‐derived… More >

Sara Bobillo‐Perez^1,2, Joan Sanchez‐de‐Toledo^3,4, Susana Segura², Monica Girona‐Alarcon², Maria Mele⁵, Anna Sole‐Ribalta², Debora Cañizo Vazquez⁶, Iolanda Jordan^2,7, Francisco Jose Cambra^1,2

Congenital Heart Disease, Vol.14, No.6, pp. 1066-1077, 2019, DOI:10.1111/chd.12846

Abstract Objective: Three scores have been proposed to stratify the risk of mortality for each cardiac surgical procedure: The RACHS‐1, the Aristotle Basic Complexity (ABC), and the STS‐EACTS complexity scoring model. The aim was to compare the ability to predict mortality and morbidity of the three scores applied to a specific population.
Design: Retrospective, descriptive study.
Setting: Pediatric and neonatal intensive care units in a referral hospital.
Patients: Children under 18 years admitted to the intensive care unit after surgery.
Interventions: None.
Outcome measures: Demographic, clinical, and surgical data were assessed. Morbidity was considered as prolonged length of stay (LOS > 75… More >

Yuli Y. Kim^1,2, Leah A. Goldberg², Katherine Awh², Tanmay Bhamare^1,2, David Drajpuch², Adi Hirshberg³, Sara L. Partington^1,2, Rachel Rogers⁴, Emily Ruckdeschel^1,2, Lynda Tobin¹, Morgan Venuti², Lisa D. Levine³

Congenital Heart Disease, Vol.14, No.3, pp. 470-478, 2019, DOI:10.1111/chd.12750

Abstract Objective: To assess performance of risk stratification schemes in predicting adverse cardiac outcomes in pregnant women with congenital heart disease (CHD) and to compare these schemes to clinical factors alone.
Design: Single‐center retrospective study.
Setting: Tertiary care academic hospital.
Patients: Women ≥18 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating CHD who delivered between 1998 and 2014. CARPREG I and ZAHARA risk scores and modified World Health Organization (WHO) criteria were applied to each woman.
Outcome Measures: The primary outcome was defined by ≥1 of the following: arrhyth‐ mia, heart failure/pulmonary edema, transient ischemic attack, stroke,… More >

R. Thomas Collins II^1,2, Margaret G. Collins³, Michael L. Schmitz^2,4, Justin T. Hamrick⁴

Congenital Heart Disease, Vol.12, No.2, pp. 133-142, 2017, DOI:10.1111/chd.12447

Abstract Williams syndrome (WS) is a congenital, multisystem disorder affecting the cardiovascular, connective tissue, and central nervous systems in 1 in 10 000 live births. Cardiovascular involvement is the most common cause of morbidity and mortality in patients with WS, and noninvasive and invasive procedures are common. Sudden cardiovascular collapse in patients with WS is a well-known phenomenon, especially in the peri-procedural period. Detailed guidelines for peri-procedural management of patients with WS are limited. The goal of this review is to provide thoughtful, safe and effective management strategies for the peri-procedural care of patients with WS More >