Xiaozhuo Xu^1,2, Yilin Huang^1,2, Xu Han^2,*

Congenital Heart Disease, Vol.19, No.2, pp. 233-246, 2024, DOI:10.32604/chd.2024.050231 - 16 May 2024

Abstract Background: Hypoplastic left heart syndrome (HLHS) is one of the most challenging congenital heart diseases in clinical treatment. In cardiac tissues, resident macrophages fulfill critical functions in maintaining a stable cardiac state and have strong regenerative capacity and organ specificity. However, the molecular mechanisms of macrophages in HLHS remained unclear. Methods: Single-nucleus RNA sequencing (snRNA-seq) data of HLHS and healthy control (donors) samples obtained from the Gene Expression Omnibus (GEO) database were normalized and clustered using the Seurat package. The “FindMarkers” function was used to screen differentially expressed genes (DEGs) between the HLHS and donor… More > Graphic Abstract

Eleni P. Asimacopoulos^*, Steven J. Staffa, Peter C. Laussen, Kirsten C. Odegard

Congenital Heart Disease, Vol.17, No.5, pp. 579-590, 2022, DOI:10.32604/chd.2022.020334 - 06 September 2022

Abstract Background: Thromboembolic events are a cause of significant morbidity and mortality in the Fontan population. We previously reported on coagulation profile changes in a cohort of patients with hypoplastic left heart syndrome (HLHS) from Stage I through Fontan completion. In this report, we examine their clinical status, anticoagulation and incidence of thromboembolic events up to 20 years post Fontan. Methods: A retrospective chart review was conducted for twenty (20) surviving patients, from 1998 through December 2020. Patients who underwent orthotopic heart transplantation (OTx) were followed until their transplant. Patients who were found in the original study… More >

Alyssia Venna¹, Kathleen Reid², Sarah Davis², Jiaxiang Gai³, Yves d’Udekem¹, Sarah Clauss^2,*

Congenital Heart Disease, Vol.17, No.5, pp. 505-518, 2022, DOI:10.32604/chd.2022.021571 - 06 September 2022

Abstract Background: Patients with single ventricle anatomy are at increased risk of growth failure and malnutrition. Amongst cardiac centers, there is little standardization of feeding practices in this complex population. We hypothesized that initiation of our center’s preoperative feeding protocol would result in decreased gastrostomy tube (G-tube) use, decreased length of stay and would not result in increased Necrotizing Enterocolitis (NEC) rates. Methods: A single institution review of 52 patients who had undergone stage I single ventricle palliative repair was performed. Patient diagnoses were hypoplastic left heart syndrome (39%), atrioventricular canal (15%), and other (46%). Postoperative parameters such… More > Graphic Abstract

Benjamin Zielonka^1,2,*, David M. Harrild^1,2, Sunil J. Ghelani^1,2, Eleni G. Elia^1,2, Christopher W. Baird^3,4, Andrew J. Powell^1,2, Rahul H. Rathod^1,2

Congenital Heart Disease, Vol.17, No.5, pp. 591-603, 2022, DOI:10.32604/chd.2022.021509 - 06 September 2022

Abstract Background: The right ventricle to pulmonary artery conduit (RVPAC) may impair right ventricular (RV) function in patients with functional single right ventricles. Modification of the RVPAC using a ring-reinforced end with dunked insertion into the RV through a limited ventriculotomy may reduce the impact on RV function. We compared RV segmental strain between patients with a traditional RVPAC and ring-reinforced RVPAC using feature tracking cardiovascular magnetic resonance (CMR) imaging. Methods: Patients with CMR examinations after Stage I operation with RVPAC between 2000 and 2018 were reviewed. Ventricular mass, volumes, late gadolinium enhancement (LGE), and peak radial and… More >

Vitaliy Suvorov^1,*, Vladimir Zaitcev¹, Karolina Andrzejczyk²

Congenital Heart Disease, Vol.17, No.3, pp. 365-374, 2022, DOI:10.32604/chd.2022.019126 - 03 May 2022

Abstract Background: Bilateral banding of the branches of the pulmonary artery in patients with hypoplastic left heart syndrome (HLHS) and other duct dependent critical neonatal heart malformations can significantly reduce the incidence of severe complications in the postoperative period, especially in severely unstable patients. In our study we compared different surgical techniques of bilateral pulmonary artery banding (PAB) in respect to their success in balancing systemic and pulmonary blood flow. Methods: We included 44 neonates with a HLHS and congenital heart diseases (CHD) with a functional single ventricle underwent a hybrid operation: bilateral PAB and patent ductus… More >

Nunzia Borrelli^1,2, Jolanda Sabatino^1,3, Martina Avesani^1,4, Josefa Paredes¹, Manjit Josen¹, Alain Fraisse¹, Paolo Guccione², Guido Michielon¹, Giovanni Di Salvo^1,4,*

Congenital Heart Disease, Vol.16, No.1, pp. 27-37, 2021, DOI:10.32604/CHD.2021.012526 - 23 December 2020

Abstract Background: Still little is known about the impact on right ventricle function of the 2 main approaches to Norwood palliation in Hypoplastic left heart syndrome, the right ventricle-pulmonary artery shunt (RVPAS) and the modified Blalock-Taussig shunt (mBTS). Methods: The cohort included 27 patients with Hypoplastic left heart syndrome (10 in the mBTS group, 17 in the RVPAS group). Longitudinal strain, tricuspid annulus peak systolic excursion and fractional area change were evaluated before Norwood and in four different breakpoints in the steady-state after Norwood procedure (30-days, 90-days, 140-days and 200-days after Norwood). Results: No significant differences were found… More >

Clifford L. Cua¹, Melissa Moore‐Clingenpeel¹, Nazia Husain², Ralf Holzer³, John P. Cheatham¹, Janaki Gokhale⁴

Congenital Heart Disease, Vol.14, No.4, pp. 609-613, 2019, DOI:10.1111/chd.12755

Abstract Background: Increased ventricular end‐diastolic pressure (VEDP) is a known risk fac‐ tor for morbidity and mortality in patients with single right ventricle (RV) physiology. Previous studies have shown mixed results correlating echocardiographic measure‐ ments with catheter‐derived VEDP in this population. Goal of this study was to eval‐ uate if echocardiographic systolic/diastolic ratio (S/D) correlated with VEDP.
Methods: Patients with single RV physiology who underwent simultaneous echocar‐ diography and catheterization were evaluated. Systolic and diastolic durations were measured using tricuspid inflow durations from Doppler analysis to calculate the S/D ratio. VEDP was obtained from the catheterization report.
Results: Twenty‐seven… 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 >

Alan F. Riley¹, Elena C. Ocampo¹, Joseph Hagan², M. Regina Lantin‐Hermoso¹

Congenital Heart Disease, Vol.14, No.5, pp. 706-712, 2019, DOI:10.1111/chd.12774

Abstract Background: When performed by cardiologists, hand‐held echocardiography (HHE) can assess ventricular systolic function and valve disease in adults, but its accuracy and utility in congenital heart disease is unknown. In hypoplastic left heart syndrome (HLHS), the echocardiographic detection of depressed right ventricular (RV) systolic function and higher grade tricuspid regurgitation (TR) can identify patients who are at increased risk of morbidity and mortality and who may benefit from additional imaging or medical therapies.
Methods: Children with HLHS after Stage I or II surgical palliation (Norwood or Glenn procedures) were prospectively enrolled. Subjects underwent HHE by a pediatric… More >

Jeannine M. Hoch¹, Oluwatosin Fatusin², Gayane Yenokyan³, W. Reid Thompson², Maureen A. Lefton‐Greif⁴

Congenital Heart Disease, Vol.14, No.3, pp. 438-445, 2019, DOI:10.1111/chd.12742

Abstract Background: Tube feedings are often needed to achieve the growth and nutrition goals associated with decreased morbidity and mortality in patients with single ventricle anat‐ omy. Variability in feeding method through the interstage period has been previously described, however, comparable information following stage 2 palliation is lacking.
Objectives: To identify types of feeding methods following stage 2 palliation and their influence on length of stay.
Design: Secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative registry was performed on 932 patients. Demographic data, medical characteristics, postoperative complications, type of feeding method, and length of stay for stage… More >