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

Pulmonary Circulation

JananiBaskaran¹ 

关键词: Mustard;    congenital;    cyanotic;    right ventricle;    baffle;   

DOI  :  10.1177/2045893217699228

学科分类：医学（综合）

来源: Sage Journals

【 摘 要 】

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.

【 授权许可】

CC BY