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

PVRI Member Authors: Thenappan Thenappan

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.

Read the full article online

Topics

Pulmonary Arterial Hypertension
Right Ventricle: Structure, Function and Dysfunction

Authors

Janani Baskaran, Kimara S. March, Thenappan Thenappan

Published in:

Pulmonary Circulation Vol 7: No 2 cover image

May 2017

Pulmonary Circulation Vol 7: No 2

View this journal

Our research platform is the world.

Through worldwide collaboration, we can begin to answer the question of a global disease.

Join the PVRI
standard-example-image.jpg