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Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult
Hypoplastic right heart syndrome is a rare cyanotic congenital heart disease with under-development of the right ventricle, tricuspid, and pulmonary valves leading to right-to-left shunting of the blood through inter-atrial septal defect. Perinatal mortality is high with very few patients surviving...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5067759/ https://www.ncbi.nlm.nih.gov/pubmed/27751299 http://dx.doi.org/10.1016/j.ihj.2016.03.030 |
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author | Mohan, Jagdish C. Mohan, Vishwas Shukla, Madhu Sethi, Arvind |
author_facet | Mohan, Jagdish C. Mohan, Vishwas Shukla, Madhu Sethi, Arvind |
author_sort | Mohan, Jagdish C. |
collection | PubMed |
description | Hypoplastic right heart syndrome is a rare cyanotic congenital heart disease with under-development of the right ventricle, tricuspid, and pulmonary valves leading to right-to-left shunting of the blood through inter-atrial septal defect. Perinatal mortality is high with very few patients surviving to adulthood without corrective surgery. This report describes a 26-year-old young woman, who had recurrent abortions and stillbirths and detected to have marked cyanosis with hypoplastic right heart, sub-arterial ventricular septal defect, absent pulmonary valve, non-compaction of the left ventricle, and bicuspid aortic valve with aortic regurgitation. The patient died owing to progressive heart failure 4 years after the diagnosis was made. |
format | Online Article Text |
id | pubmed-5067759 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-50677592017-09-01 Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult Mohan, Jagdish C. Mohan, Vishwas Shukla, Madhu Sethi, Arvind Indian Heart J Case Report Hypoplastic right heart syndrome is a rare cyanotic congenital heart disease with under-development of the right ventricle, tricuspid, and pulmonary valves leading to right-to-left shunting of the blood through inter-atrial septal defect. Perinatal mortality is high with very few patients surviving to adulthood without corrective surgery. This report describes a 26-year-old young woman, who had recurrent abortions and stillbirths and detected to have marked cyanosis with hypoplastic right heart, sub-arterial ventricular septal defect, absent pulmonary valve, non-compaction of the left ventricle, and bicuspid aortic valve with aortic regurgitation. The patient died owing to progressive heart failure 4 years after the diagnosis was made. Elsevier 2016-09 2016-04-15 /pmc/articles/PMC5067759/ /pubmed/27751299 http://dx.doi.org/10.1016/j.ihj.2016.03.030 Text en © 2016 Cardiological Society of India. Published by Elsevier B.V. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Case Report Mohan, Jagdish C. Mohan, Vishwas Shukla, Madhu Sethi, Arvind Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult |
title | Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult |
title_full | Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult |
title_fullStr | Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult |
title_full_unstemmed | Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult |
title_short | Hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult |
title_sort | hypoplastic right heart syndrome, absent pulmonary valve, and non-compacted left ventricle in an adult |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5067759/ https://www.ncbi.nlm.nih.gov/pubmed/27751299 http://dx.doi.org/10.1016/j.ihj.2016.03.030 |
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