Cargando…
Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy
Cardiac abnormalities such as left ventricular hypertrophy, left ventricular dilation, and pulmonary hypertension in sickle cell anemia have been previously described. Hydroxyurea, a disease-modifying therapy for sickle cell anemia, has been used for several decades. Longitudinal assessment of echoc...
Autores principales: | , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
American Society of Hematology
2021
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8579269/ https://www.ncbi.nlm.nih.gov/pubmed/34529023 http://dx.doi.org/10.1182/bloodadvances.2021005076 |
_version_ | 1784596406079586304 |
---|---|
author | Dhar, Arushi Leung, Tung Ming Appiah-Kubi, Abena Gruber, Dorota Aygun, Banu Serigano, Olivia Mitchell, Elizabeth |
author_facet | Dhar, Arushi Leung, Tung Ming Appiah-Kubi, Abena Gruber, Dorota Aygun, Banu Serigano, Olivia Mitchell, Elizabeth |
author_sort | Dhar, Arushi |
collection | PubMed |
description | Cardiac abnormalities such as left ventricular hypertrophy, left ventricular dilation, and pulmonary hypertension in sickle cell anemia have been previously described. Hydroxyurea, a disease-modifying therapy for sickle cell anemia, has been used for several decades. Longitudinal assessment of echocardiographic abnormalities in children and young adults with sickle cell anemia receiving hydroxyurea therapy is lacking. The goal of this retrospective study was to determine the prevalence of echocardiographic abnormalities in children and young adults with sickle cell anemia and to examine the effects of hydroxyurea on reverse cardiac remodeling. We reviewed the records of patients with sickle cell anemia who underwent routine cardiac screening at Cohen Children’s Medical Center between 2010 and 2017, followed by retrospective longitudinal analysis of echocardiograms performed on patients receiving treatment with hydroxyurea. Data on a total of 100 patients with sickle cell anemia were analyzed; 60 (60%) were being treated with hydroxyurea. Twenty-five (41.6%) of the patients on hydroxyurea had been treated for <1 year; these patients had a significantly greater prevalence of left ventricular dilation compared with those who had been on treatment for >1 year. Serial echocardiograms of patients receiving hydroxyurea were then analyzed. Left ventricular dilation and hypertrophy improved significantly with hydroxyurea treatment. In addition, the left ventricular volume and mass correlated negatively with duration of treatment with hydroxyurea. Our study provides evidence that prolonged hydroxyurea therapy may lead to reverse cardiac remodeling. Future studies should attempt to follow up this patient cohort for a longer duration. |
format | Online Article Text |
id | pubmed-8579269 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | American Society of Hematology |
record_format | MEDLINE/PubMed |
spelling | pubmed-85792692021-11-10 Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy Dhar, Arushi Leung, Tung Ming Appiah-Kubi, Abena Gruber, Dorota Aygun, Banu Serigano, Olivia Mitchell, Elizabeth Blood Adv Clinical Trials and Observations Cardiac abnormalities such as left ventricular hypertrophy, left ventricular dilation, and pulmonary hypertension in sickle cell anemia have been previously described. Hydroxyurea, a disease-modifying therapy for sickle cell anemia, has been used for several decades. Longitudinal assessment of echocardiographic abnormalities in children and young adults with sickle cell anemia receiving hydroxyurea therapy is lacking. The goal of this retrospective study was to determine the prevalence of echocardiographic abnormalities in children and young adults with sickle cell anemia and to examine the effects of hydroxyurea on reverse cardiac remodeling. We reviewed the records of patients with sickle cell anemia who underwent routine cardiac screening at Cohen Children’s Medical Center between 2010 and 2017, followed by retrospective longitudinal analysis of echocardiograms performed on patients receiving treatment with hydroxyurea. Data on a total of 100 patients with sickle cell anemia were analyzed; 60 (60%) were being treated with hydroxyurea. Twenty-five (41.6%) of the patients on hydroxyurea had been treated for <1 year; these patients had a significantly greater prevalence of left ventricular dilation compared with those who had been on treatment for >1 year. Serial echocardiograms of patients receiving hydroxyurea were then analyzed. Left ventricular dilation and hypertrophy improved significantly with hydroxyurea treatment. In addition, the left ventricular volume and mass correlated negatively with duration of treatment with hydroxyurea. Our study provides evidence that prolonged hydroxyurea therapy may lead to reverse cardiac remodeling. Future studies should attempt to follow up this patient cohort for a longer duration. American Society of Hematology 2021-11-01 /pmc/articles/PMC8579269/ /pubmed/34529023 http://dx.doi.org/10.1182/bloodadvances.2021005076 Text en © 2021 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved. |
spellingShingle | Clinical Trials and Observations Dhar, Arushi Leung, Tung Ming Appiah-Kubi, Abena Gruber, Dorota Aygun, Banu Serigano, Olivia Mitchell, Elizabeth Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy |
title | Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy |
title_full | Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy |
title_fullStr | Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy |
title_full_unstemmed | Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy |
title_short | Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy |
title_sort | longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy |
topic | Clinical Trials and Observations |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8579269/ https://www.ncbi.nlm.nih.gov/pubmed/34529023 http://dx.doi.org/10.1182/bloodadvances.2021005076 |
work_keys_str_mv | AT dhararushi longitudinalanalysisofcardiacabnormalitiesinpediatricpatientswithsicklecellanemiaandeffectofhydroxyureatherapy AT leungtungming longitudinalanalysisofcardiacabnormalitiesinpediatricpatientswithsicklecellanemiaandeffectofhydroxyureatherapy AT appiahkubiabena longitudinalanalysisofcardiacabnormalitiesinpediatricpatientswithsicklecellanemiaandeffectofhydroxyureatherapy AT gruberdorota longitudinalanalysisofcardiacabnormalitiesinpediatricpatientswithsicklecellanemiaandeffectofhydroxyureatherapy AT aygunbanu longitudinalanalysisofcardiacabnormalitiesinpediatricpatientswithsicklecellanemiaandeffectofhydroxyureatherapy AT seriganoolivia longitudinalanalysisofcardiacabnormalitiesinpediatricpatientswithsicklecellanemiaandeffectofhydroxyureatherapy AT mitchellelizabeth longitudinalanalysisofcardiacabnormalitiesinpediatricpatientswithsicklecellanemiaandeffectofhydroxyureatherapy |