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Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation

Holt-Oram syndrome is a rare inherited disorder involving the hands, arms, and the heart. The defects involve carpal bones of the wrist and the thumb and the associated cardiac anomalies like atrial or ventricular septal defects. Congenital cardiac and upper-limb malformations frequently occur toget...

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Autores principales: Kumar, Rupesh, Mahapatra, Subhendu Sekhar, Datta, Monalisa, Hoque, Amanul, Datta, Swarnendu, Ghosh, Soumyajit, Datta, Santanu, Bhattacharjee, Subhankar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4006581/
https://www.ncbi.nlm.nih.gov/pubmed/24826304
http://dx.doi.org/10.1155/2014/130617
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author Kumar, Rupesh
Mahapatra, Subhendu Sekhar
Datta, Monalisa
Hoque, Amanul
Datta, Swarnendu
Ghosh, Soumyajit
Datta, Santanu
Bhattacharjee, Subhankar
author_facet Kumar, Rupesh
Mahapatra, Subhendu Sekhar
Datta, Monalisa
Hoque, Amanul
Datta, Swarnendu
Ghosh, Soumyajit
Datta, Santanu
Bhattacharjee, Subhankar
author_sort Kumar, Rupesh
collection PubMed
description Holt-Oram syndrome is a rare inherited disorder involving the hands, arms, and the heart. The defects involve carpal bones of the wrist and the thumb and the associated cardiac anomalies like atrial or ventricular septal defects. Congenital cardiac and upper-limb malformations frequently occur together and are classified as heart-hand syndromes. The most common amongst the heart-hand disorders is the Holt-Oram syndrome, which is characterized by septal defects of the heart and preaxial radial ray abnormalities. Its incidence is one in 100,000 live births. Approximately three out of four patients have some cardiac abnormality with common associations being either an atrial septal defect or ventricular septal defect. Herein, we report a rare sporadic case of Holt-Oram syndrome with atrial septal defect with symptoms of heart failure in a forty-five-year-old lady who underwent emergency cardiac surgery for the symptoms.
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spelling pubmed-40065812014-05-13 Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation Kumar, Rupesh Mahapatra, Subhendu Sekhar Datta, Monalisa Hoque, Amanul Datta, Swarnendu Ghosh, Soumyajit Datta, Santanu Bhattacharjee, Subhankar Case Rep Cardiol Case Report Holt-Oram syndrome is a rare inherited disorder involving the hands, arms, and the heart. The defects involve carpal bones of the wrist and the thumb and the associated cardiac anomalies like atrial or ventricular septal defects. Congenital cardiac and upper-limb malformations frequently occur together and are classified as heart-hand syndromes. The most common amongst the heart-hand disorders is the Holt-Oram syndrome, which is characterized by septal defects of the heart and preaxial radial ray abnormalities. Its incidence is one in 100,000 live births. Approximately three out of four patients have some cardiac abnormality with common associations being either an atrial septal defect or ventricular septal defect. Herein, we report a rare sporadic case of Holt-Oram syndrome with atrial septal defect with symptoms of heart failure in a forty-five-year-old lady who underwent emergency cardiac surgery for the symptoms. Hindawi Publishing Corporation 2014 2014-03-23 /pmc/articles/PMC4006581/ /pubmed/24826304 http://dx.doi.org/10.1155/2014/130617 Text en Copyright © 2014 Rupesh Kumar et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Kumar, Rupesh
Mahapatra, Subhendu Sekhar
Datta, Monalisa
Hoque, Amanul
Datta, Swarnendu
Ghosh, Soumyajit
Datta, Santanu
Bhattacharjee, Subhankar
Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation
title Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation
title_full Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation
title_fullStr Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation
title_full_unstemmed Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation
title_short Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation
title_sort holt-oram syndrome in adult presenting with heart failure: a rare presentation
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4006581/
https://www.ncbi.nlm.nih.gov/pubmed/24826304
http://dx.doi.org/10.1155/2014/130617
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