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

Janani Baskaran; Kimara S. March; Thenappan Thenappan

doi:10.1177/2045893217699228

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

Janani Baskaran, Kimara S. March, Thenappan Thenappan

Medicine - Cardiology Division

Research output: Contribution to journal › Article › peer-review

3 Scopus citations

Abstract

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.

Original language	English (US)
Pages (from-to)	547-550
Number of pages	4
Journal	Pulmonary Circulation
Volume	7
Issue number	2
DOIs	https://doi.org/10.1177/2045893217699228
State	Published - Jun 2017

Bibliographical note

Funding Information:
TT was funded by an AHA Scientist Development Grant 15SDG25560048 and a Lillehei Heart Institute High Risk and High Reward Grant.

Publisher Copyright:
© The Author(s) 2017.

Keywords

Baffle
Congenital
Cyanotic
Mustard
Right ventricle

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1177/2045893217699228

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5467927

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abstract = "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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AU - March, Kimara S.

AU - Thenappan, Thenappan

N1 - Funding Information: TT was funded by an AHA Scientist Development Grant 15SDG25560048 and a Lillehei Heart Institute High Risk and High Reward Grant. Publisher Copyright: © The Author(s) 2017.

PY - 2017/6

Y1 - 2017/6

N2 - 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.

AB - 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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KW - Cyanotic

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KW - Right ventricle

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

Abstract

Bibliographical note

Keywords

UN SDGs

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