Navigating a Case of Swyer Syndrome with Congenital Heart Disease

A 38-year-old female underwent septal closure for a large ostium secundum atrial septal defect (ASD) as she had progressive dyspnea. She was also found to have primary amenorrhea, for which she was evaluated. The clinical examination and investigations revealed a female phenotype and a male karyotyp...

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Main Authors: K. Subramanyam, Dilip Johny, Amita Rao, Aditya Ojha
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2024-10-01
Series:Heart Views
Subjects:
Online Access:https://journals.lww.com/10.4103/heartviews.heartviews_139_24
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author K. Subramanyam
Dilip Johny
Amita Rao
Aditya Ojha
author_facet K. Subramanyam
Dilip Johny
Amita Rao
Aditya Ojha
author_sort K. Subramanyam
collection DOAJ
description A 38-year-old female underwent septal closure for a large ostium secundum atrial septal defect (ASD) as she had progressive dyspnea. She was also found to have primary amenorrhea, for which she was evaluated. The clinical examination and investigations revealed a female phenotype and a male karyotype (46 XY). She was diagnosed with Swyer syndrome and subsequently started on hormone replacement therapy. This case highlights the presence of congenital heart disease and large ostium secundum ASD with left to right shunt in patients with Swyer syndrome, which has not been reported in the literature.
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publisher Wolters Kluwer Medknow Publications
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series Heart Views
spelling doaj-art-a7276dbb9d234b01a54adfcf548541822025-08-20T02:32:00ZengWolters Kluwer Medknow PublicationsHeart Views1995-705X0976-51232024-10-0125428328710.4103/heartviews.heartviews_139_24Navigating a Case of Swyer Syndrome with Congenital Heart DiseaseK. SubramanyamDilip JohnyAmita RaoAditya OjhaA 38-year-old female underwent septal closure for a large ostium secundum atrial septal defect (ASD) as she had progressive dyspnea. She was also found to have primary amenorrhea, for which she was evaluated. The clinical examination and investigations revealed a female phenotype and a male karyotype (46 XY). She was diagnosed with Swyer syndrome and subsequently started on hormone replacement therapy. This case highlights the presence of congenital heart disease and large ostium secundum ASD with left to right shunt in patients with Swyer syndrome, which has not been reported in the literature.https://journals.lww.com/10.4103/heartviews.heartviews_139_24asdcongenital heart diseaseswyer syndrome
spellingShingle K. Subramanyam
Dilip Johny
Amita Rao
Aditya Ojha
Navigating a Case of Swyer Syndrome with Congenital Heart Disease
Heart Views
asd
congenital heart disease
swyer syndrome
title Navigating a Case of Swyer Syndrome with Congenital Heart Disease
title_full Navigating a Case of Swyer Syndrome with Congenital Heart Disease
title_fullStr Navigating a Case of Swyer Syndrome with Congenital Heart Disease
title_full_unstemmed Navigating a Case of Swyer Syndrome with Congenital Heart Disease
title_short Navigating a Case of Swyer Syndrome with Congenital Heart Disease
title_sort navigating a case of swyer syndrome with congenital heart disease
topic asd
congenital heart disease
swyer syndrome
url https://journals.lww.com/10.4103/heartviews.heartviews_139_24
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AT dilipjohny navigatingacaseofswyersyndromewithcongenitalheartdisease
AT amitarao navigatingacaseofswyersyndromewithcongenitalheartdisease
AT adityaojha navigatingacaseofswyersyndromewithcongenitalheartdisease