Fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum: How to counsel the parents?

Isolated absent pulmonary valve syndrome with an intact ventricular septum (APVS/IVS) is an extremely rare lesion. The prenatal diagnosis was described in a few reports. In the current work, we report a unique fetal case with this rare anomaly. The family counseling for isolated APVS/IVS is a differ...

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Detalles Bibliográficos
Autores principales: Rakha, Shaimaa, Alkhushi, Naif
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2020
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7331835/
https://www.ncbi.nlm.nih.gov/pubmed/32641885
http://dx.doi.org/10.4103/apc.APC_101_19
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author Rakha, Shaimaa
Alkhushi, Naif
author_facet Rakha, Shaimaa
Alkhushi, Naif
author_sort Rakha, Shaimaa
collection PubMed
description Isolated absent pulmonary valve syndrome with an intact ventricular septum (APVS/IVS) is an extremely rare lesion. The prenatal diagnosis was described in a few reports. In the current work, we report a unique fetal case with this rare anomaly. The family counseling for isolated APVS/IVS is a different challenging process than with fetal Fallot type diagnosis. Moreover, 22q11 deletion has never been found as an association. Parents must also be aware of the expected early heart failure in cases with large patent ductus arteriosus and the need for its closure as early as possible.
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spelling pubmed-73318352020-07-07 Fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum: How to counsel the parents? Rakha, Shaimaa Alkhushi, Naif Ann Pediatr Cardiol Case Report Isolated absent pulmonary valve syndrome with an intact ventricular septum (APVS/IVS) is an extremely rare lesion. The prenatal diagnosis was described in a few reports. In the current work, we report a unique fetal case with this rare anomaly. The family counseling for isolated APVS/IVS is a different challenging process than with fetal Fallot type diagnosis. Moreover, 22q11 deletion has never been found as an association. Parents must also be aware of the expected early heart failure in cases with large patent ductus arteriosus and the need for its closure as early as possible. Wolters Kluwer - Medknow 2020 2020-03-30 /pmc/articles/PMC7331835/ /pubmed/32641885 http://dx.doi.org/10.4103/apc.APC_101_19 Text en Copyright: © 2020 Annals of Pediatric Cardiology http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Rakha, Shaimaa
Alkhushi, Naif
Fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum: How to counsel the parents?
title Fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum: How to counsel the parents?
title_full Fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum: How to counsel the parents?
title_fullStr Fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum: How to counsel the parents?
title_full_unstemmed Fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum: How to counsel the parents?
title_short Fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum: How to counsel the parents?
title_sort fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum: how to counsel the parents?
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7331835/
https://www.ncbi.nlm.nih.gov/pubmed/32641885
http://dx.doi.org/10.4103/apc.APC_101_19
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AT alkhushinaif fetaldiagnosisofisolatedabsentpulmonaryvalvewithintactinterventricularseptumhowtocounseltheparents

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