Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome
Significant surgical and medical advances over the past several decades have resulted in a growing number of infants and children surviving with hypoplastic left heart syndrome (HLHS) and other congenital heart defects associated with a single systemic right ventricle (RV). However, cardiac dysfunct...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2022-05-01
|
| Series: | Journal of Cardiovascular Development and Disease |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2308-3425/9/5/152 |
| _version_ | 1797498935357472768 |
|---|---|
| author | Angela N. Baybayon-Grandgeorge Ashley E. Pietra Shelley D. Miyamoto Anastacia M. Garcia |
| author_facet | Angela N. Baybayon-Grandgeorge Ashley E. Pietra Shelley D. Miyamoto Anastacia M. Garcia |
| author_sort | Angela N. Baybayon-Grandgeorge |
| collection | DOAJ |
| description | Significant surgical and medical advances over the past several decades have resulted in a growing number of infants and children surviving with hypoplastic left heart syndrome (HLHS) and other congenital heart defects associated with a single systemic right ventricle (RV). However, cardiac dysfunction and ultimately heart failure (HF) remain the most common cause of death and indication for transplantation in this population. Moreover, while early recognition and treatment of single ventricle-related complications are essential to improving outcomes, there are no proven therapeutic strategies for single systemic RV HF in the pediatric population. Importantly, prototypical adult HF therapies have been relatively ineffective in mitigating the need for cardiac transplantation in HLHS, likely due to several unique attributes of the failing HLHS myocardium. Here, we discuss the most commonly used medical therapies for the treatment of HF symptoms in HLHS and other single systemic RV patients. Additionally, we provide an overview of potential novel therapies for systemic ventricular failure in the HLHS and related populations based on fundamental science, pre-clinical, clinical, and observational studies in the current literature. |
| first_indexed | 2024-03-10T03:40:24Z |
| format | Article |
| id | doaj.art-48a35e960bb54a1e83b4bc200a8fae38 |
| institution | Directory Open Access Journal |
| issn | 2308-3425 |
| language | English |
| last_indexed | 2024-03-10T03:40:24Z |
| publishDate | 2022-05-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Cardiovascular Development and Disease |
| spelling | doaj.art-48a35e960bb54a1e83b4bc200a8fae382023-11-23T11:32:53ZengMDPI AGJournal of Cardiovascular Development and Disease2308-34252022-05-019515210.3390/jcdd9050152Medical Therapies for Heart Failure in Hypoplastic Left Heart SyndromeAngela N. Baybayon-Grandgeorge0Ashley E. Pietra1Shelley D. Miyamoto2Anastacia M. Garcia3Department of Medicine, Division of Cardiology, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USADepartment of Pediatrics, Section of Cardiology, University of Colorado Anschutz Medical Campus, Children’s Hospital Colorado, Aurora, CO 80045, USADepartment of Pediatrics, Section of Cardiology, University of Colorado Anschutz Medical Campus, Children’s Hospital Colorado, Aurora, CO 80045, USADepartment of Pediatrics, Section of Cardiology, University of Colorado Anschutz Medical Campus, Children’s Hospital Colorado, Aurora, CO 80045, USASignificant surgical and medical advances over the past several decades have resulted in a growing number of infants and children surviving with hypoplastic left heart syndrome (HLHS) and other congenital heart defects associated with a single systemic right ventricle (RV). However, cardiac dysfunction and ultimately heart failure (HF) remain the most common cause of death and indication for transplantation in this population. Moreover, while early recognition and treatment of single ventricle-related complications are essential to improving outcomes, there are no proven therapeutic strategies for single systemic RV HF in the pediatric population. Importantly, prototypical adult HF therapies have been relatively ineffective in mitigating the need for cardiac transplantation in HLHS, likely due to several unique attributes of the failing HLHS myocardium. Here, we discuss the most commonly used medical therapies for the treatment of HF symptoms in HLHS and other single systemic RV patients. Additionally, we provide an overview of potential novel therapies for systemic ventricular failure in the HLHS and related populations based on fundamental science, pre-clinical, clinical, and observational studies in the current literature.https://www.mdpi.com/2308-3425/9/5/152congenital heart diseaseheart failurehypoplastic left heart syndrome |
| spellingShingle | Angela N. Baybayon-Grandgeorge Ashley E. Pietra Shelley D. Miyamoto Anastacia M. Garcia Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome Journal of Cardiovascular Development and Disease congenital heart disease heart failure hypoplastic left heart syndrome |
| title | Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome |
| title_full | Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome |
| title_fullStr | Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome |
| title_full_unstemmed | Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome |
| title_short | Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome |
| title_sort | medical therapies for heart failure in hypoplastic left heart syndrome |
| topic | congenital heart disease heart failure hypoplastic left heart syndrome |
| url | https://www.mdpi.com/2308-3425/9/5/152 |
| work_keys_str_mv | AT angelanbaybayongrandgeorge medicaltherapiesforheartfailureinhypoplasticleftheartsyndrome AT ashleyepietra medicaltherapiesforheartfailureinhypoplasticleftheartsyndrome AT shelleydmiyamoto medicaltherapiesforheartfailureinhypoplasticleftheartsyndrome AT anastaciamgarcia medicaltherapiesforheartfailureinhypoplasticleftheartsyndrome |