Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead
Endothelial dysfunction is characterized by a vasoconstricted, pro-coagulative, and pro-inflammatory phenotype and is known to play a role in several chronic non-communicable diseases. Several biophysical and biochemical markers have been developed to assess endothelial function clinically. Its rele...
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| Format: | Article |
| Language: | English |
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Elsevier
2025-06-01
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| Series: | International Journal of Cardiology Congenital Heart Disease |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2666668525000254 |
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| author | Raksheeth Agarwal Louise E. Coats Ali N. Zaidi |
| author_facet | Raksheeth Agarwal Louise E. Coats Ali N. Zaidi |
| author_sort | Raksheeth Agarwal |
| collection | DOAJ |
| description | Endothelial dysfunction is characterized by a vasoconstricted, pro-coagulative, and pro-inflammatory phenotype and is known to play a role in several chronic non-communicable diseases. Several biophysical and biochemical markers have been developed to assess endothelial function clinically. Its relevance in individuals born with single-ventricle congenital heart disease (SV-CHD) is increasingly recognized. Endothelial dysfunction has been observed in all stages of palliation in SV-CHD patients. Several mechanisms possibly contribute, including genetic factors, hypoxia, loss of pulsatility of blood flow, neurohormonal and sympathetic overactivation, and oxidative stress. Clinically, it possibly contributes to impaired pulmonary flow, exercise limitation, thromboembolisms, liver dysfunction, and adverse pregnancy outcomes. Based on this information, several therapeutic targets have been proposed such as early surgical and exercise interventions, pulmonary vasodilators, and other common pharmacological agents. However, much remains unknown and future studies must unravel the relationship of endothelial dysfunction with this complex patient group, ultimately improving their clinical care. |
| format | Article |
| id | doaj-art-926c2bd1803c4affa3982a596d5f2b75 |
| institution | Kabale University |
| issn | 2666-6685 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | International Journal of Cardiology Congenital Heart Disease |
| spelling | doaj-art-926c2bd1803c4affa3982a596d5f2b752025-08-20T03:52:43ZengElsevierInternational Journal of Cardiology Congenital Heart Disease2666-66852025-06-012010058910.1016/j.ijcchd.2025.100589Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies aheadRaksheeth Agarwal0Louise E. Coats1Ali N. Zaidi2Department of Medicine, Jacobi Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA; Corresponding author.Adult Congenital Heart Unit, Freeman Hospital, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK; Population Health Sciences Institute, Newcastle University, Newcastle Upon Tyne, UKMount Sinai Adult Congenital Heart Disease Center, Mount Sinai Heart, New York, NY, USAEndothelial dysfunction is characterized by a vasoconstricted, pro-coagulative, and pro-inflammatory phenotype and is known to play a role in several chronic non-communicable diseases. Several biophysical and biochemical markers have been developed to assess endothelial function clinically. Its relevance in individuals born with single-ventricle congenital heart disease (SV-CHD) is increasingly recognized. Endothelial dysfunction has been observed in all stages of palliation in SV-CHD patients. Several mechanisms possibly contribute, including genetic factors, hypoxia, loss of pulsatility of blood flow, neurohormonal and sympathetic overactivation, and oxidative stress. Clinically, it possibly contributes to impaired pulmonary flow, exercise limitation, thromboembolisms, liver dysfunction, and adverse pregnancy outcomes. Based on this information, several therapeutic targets have been proposed such as early surgical and exercise interventions, pulmonary vasodilators, and other common pharmacological agents. However, much remains unknown and future studies must unravel the relationship of endothelial dysfunction with this complex patient group, ultimately improving their clinical care.http://www.sciencedirect.com/science/article/pii/S2666668525000254Single ventricle congenital heart diseaseEndothelial dysfunctionGlenn circulationFontan circulation |
| spellingShingle | Raksheeth Agarwal Louise E. Coats Ali N. Zaidi Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead International Journal of Cardiology Congenital Heart Disease Single ventricle congenital heart disease Endothelial dysfunction Glenn circulation Fontan circulation |
| title | Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead |
| title_full | Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead |
| title_fullStr | Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead |
| title_full_unstemmed | Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead |
| title_short | Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead |
| title_sort | endothelial dysfunction in single ventricle physiology and the fontan circulation what lies ahead |
| topic | Single ventricle congenital heart disease Endothelial dysfunction Glenn circulation Fontan circulation |
| url | http://www.sciencedirect.com/science/article/pii/S2666668525000254 |
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