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Late-onset pulmonary arterial hypertension in repaired D-transposition of great arteries: an uncommon complication

Late-onset pulmonary arterial hypertension (PAH) is a rare but fatal complication in patients with childhood surgical repair of dextro-transposition of great arteries (D-TGA), especially with the Mustard and Senning procedures. The pathogenic mechanisms of PAH in patients with repaired D-TGA are not...

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Detalles Bibliográficos
Autores principales: Baskaran, Janani, March, Kimara S., Thenappan, Thenappan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5467927/
https://www.ncbi.nlm.nih.gov/pubmed/28597760
http://dx.doi.org/10.1177/2045893217699228
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author Baskaran, Janani
March, Kimara S.
Thenappan, Thenappan
author_facet Baskaran, Janani
March, Kimara S.
Thenappan, Thenappan
author_sort Baskaran, Janani
collection PubMed
description Late-onset pulmonary arterial hypertension (PAH) is a rare but fatal complication in patients with childhood surgical repair of dextro-transposition of great arteries (D-TGA), especially with the Mustard and Senning procedures. The pathogenic mechanisms of PAH in patients with repaired D-TGA are not well understood and treatment is not standardized. In this manuscript, we present a case of late-onset PAH in an adult D-TGA patient after Mustard repair and discuss the pathogenic mechanisms, diagnosis, and treatment of pulmonary hypertension in repaired D-TGA.
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spelling pubmed-54679272017-06-20 Late-onset pulmonary arterial hypertension in repaired D-transposition of great arteries: an uncommon complication Baskaran, Janani March, Kimara S. Thenappan, Thenappan Pulm Circ Case Reports Late-onset pulmonary arterial hypertension (PAH) is a rare but fatal complication in patients with childhood surgical repair of dextro-transposition of great arteries (D-TGA), especially with the Mustard and Senning procedures. The pathogenic mechanisms of PAH in patients with repaired D-TGA are not well understood and treatment is not standardized. In this manuscript, we present a case of late-onset PAH in an adult D-TGA patient after Mustard repair and discuss the pathogenic mechanisms, diagnosis, and treatment of pulmonary hypertension in repaired D-TGA. SAGE Publications 2017-05-30 /pmc/articles/PMC5467927/ /pubmed/28597760 http://dx.doi.org/10.1177/2045893217699228 Text en © The Author(s) 2017 http://creativecommons.org/licenses/by-nc/3.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 3.0 License (http://www.creativecommons.org/licenses/by-nc/3.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Reports
Baskaran, Janani
March, Kimara S.
Thenappan, Thenappan
Late-onset pulmonary arterial hypertension in repaired D-transposition of great arteries: an uncommon complication
title Late-onset pulmonary arterial hypertension in repaired D-transposition of great arteries: an uncommon complication
title_full Late-onset pulmonary arterial hypertension in repaired D-transposition of great arteries: an uncommon complication
title_fullStr Late-onset pulmonary arterial hypertension in repaired D-transposition of great arteries: an uncommon complication
title_full_unstemmed Late-onset pulmonary arterial hypertension in repaired D-transposition of great arteries: an uncommon complication
title_short Late-onset pulmonary arterial hypertension in repaired D-transposition of great arteries: an uncommon complication
title_sort late-onset pulmonary arterial hypertension in repaired d-transposition of great arteries: an uncommon complication
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5467927/
https://www.ncbi.nlm.nih.gov/pubmed/28597760
http://dx.doi.org/10.1177/2045893217699228
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