Right Ventricular Remodeling and Function in Hypoplastic Left Heart Syndrome
The right ventricle (RV) in hypoplastic left heart syndrome (HLHS) becomes the systemic ventricle pumping against systemic afterload. It also has to adapt to an initially increased volume load followed by a decrease in volume load after Fontan completion. Anatomical HLHS subtype, therapeutic strateg...
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| Language: | English |
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Elsevier
2024-12-01
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| Series: | JACC: Advances |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2772963X24006914 |
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| author | Thomas Salaets, MD, PhD Marc Gewillig, MD, PhD Alexander Van De Bruaene, MD, PhD Luc L. Mertens, MD, PhD |
| author_facet | Thomas Salaets, MD, PhD Marc Gewillig, MD, PhD Alexander Van De Bruaene, MD, PhD Luc L. Mertens, MD, PhD |
| author_sort | Thomas Salaets, MD, PhD |
| collection | DOAJ |
| description | The right ventricle (RV) in hypoplastic left heart syndrome (HLHS) becomes the systemic ventricle pumping against systemic afterload. It also has to adapt to an initially increased volume load followed by a decrease in volume load after Fontan completion. Anatomical HLHS subtype, therapeutic strategy, tricuspid valve regurgitation, recoarctation, and genetics influence RV size and function. The resulting remodeling process can be maladaptive and lead to ventricular systolic and diastolic dysfunction. While systolic dysfunction is a strong predictor for mortality before Fontan, there is increasing evidence for the impact of progressive diastolic dysfunction after Fontan. This comprehensive review summarizes the (recent) empirical observations that increased understanding of RV remodeling and function in HLHS. It aims at clinicians and researchers wishing to increase their understanding of the physiology of this disease. It highlights the potential for future scientific work on the assessment and preservation of myocardial health throughout the palliation. |
| format | Article |
| id | doaj-art-1d81972dcbac45d795dea874a9e97d65 |
| institution | Kabale University |
| issn | 2772-963X |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | JACC: Advances |
| spelling | doaj-art-1d81972dcbac45d795dea874a9e97d652024-11-22T07:40:01ZengElsevierJACC: Advances2772-963X2024-12-01312101411Right Ventricular Remodeling and Function in Hypoplastic Left Heart SyndromeThomas Salaets, MD, PhD0Marc Gewillig, MD, PhD1Alexander Van De Bruaene, MD, PhD2Luc L. Mertens, MD, PhD3Division of Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium; The Labatt Family Heart Centre, Division of Cardiology, The Hospital for Sick Children, Department of Paediatrics, University of Toronto, Toronto, CanadaDivision of Pediatric Cardiology, University Hospitals Leuven, Leuven, BelgiumDivision of Adult Congenital Heart Disease, University Hospitals Leuven, Leuven, BelgiumThe Labatt Family Heart Centre, Division of Cardiology, The Hospital for Sick Children, Department of Paediatrics, University of Toronto, Toronto, Canada; Address for correspondence: Dr Luc Mertens, Division of Cardiology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.The right ventricle (RV) in hypoplastic left heart syndrome (HLHS) becomes the systemic ventricle pumping against systemic afterload. It also has to adapt to an initially increased volume load followed by a decrease in volume load after Fontan completion. Anatomical HLHS subtype, therapeutic strategy, tricuspid valve regurgitation, recoarctation, and genetics influence RV size and function. The resulting remodeling process can be maladaptive and lead to ventricular systolic and diastolic dysfunction. While systolic dysfunction is a strong predictor for mortality before Fontan, there is increasing evidence for the impact of progressive diastolic dysfunction after Fontan. This comprehensive review summarizes the (recent) empirical observations that increased understanding of RV remodeling and function in HLHS. It aims at clinicians and researchers wishing to increase their understanding of the physiology of this disease. It highlights the potential for future scientific work on the assessment and preservation of myocardial health throughout the palliation.http://www.sciencedirect.com/science/article/pii/S2772963X24006914congenital cardiologyFontanhypoplastic left heart syndromesingle ventricle diseaseventricular remodeling |
| spellingShingle | Thomas Salaets, MD, PhD Marc Gewillig, MD, PhD Alexander Van De Bruaene, MD, PhD Luc L. Mertens, MD, PhD Right Ventricular Remodeling and Function in Hypoplastic Left Heart Syndrome JACC: Advances congenital cardiology Fontan hypoplastic left heart syndrome single ventricle disease ventricular remodeling |
| title | Right Ventricular Remodeling and Function in Hypoplastic Left Heart Syndrome |
| title_full | Right Ventricular Remodeling and Function in Hypoplastic Left Heart Syndrome |
| title_fullStr | Right Ventricular Remodeling and Function in Hypoplastic Left Heart Syndrome |
| title_full_unstemmed | Right Ventricular Remodeling and Function in Hypoplastic Left Heart Syndrome |
| title_short | Right Ventricular Remodeling and Function in Hypoplastic Left Heart Syndrome |
| title_sort | right ventricular remodeling and function in hypoplastic left heart syndrome |
| topic | congenital cardiology Fontan hypoplastic left heart syndrome single ventricle disease ventricular remodeling |
| url | http://www.sciencedirect.com/science/article/pii/S2772963X24006914 |
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