Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Mohan, Jagdish C., Mohan, Vishwas, Shukla, Madhu, Sethi, Arvind
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2016
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
_version_ 1782460703647465472
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
work_keys_str_mv AT mohanjagdishc hypoplasticrightheartsyndromeabsentpulmonaryvalveandnoncompactedleftventricleinanadult
AT mohanvishwas hypoplasticrightheartsyndromeabsentpulmonaryvalveandnoncompactedleftventricleinanadult
AT shuklamadhu hypoplasticrightheartsyndromeabsentpulmonaryvalveandnoncompactedleftventricleinanadult
AT sethiarvind hypoplasticrightheartsyndromeabsentpulmonaryvalveandnoncompactedleftventricleinanadult