Adult diagnosis of Swyer-James-MacLeod syndrome: a case report

• Author(s): Capela, C ; Gouveia, P ; Sousa, M ; Regadas, MJ
• Date: 2011
• Persistent ID: http://hdl.handle.net/10400.23/98
• Origin: Repositório Científico do Hospital de Braga
• Subject(s): Síndrome de Swyer-James-MacLeod
• Document Type: Article
• Language: English

Description

INTRODUCTION: Swyer-James-MacLeod syndrome or unilateral hyperlucent lung syndrome is a rare entity associated with postinfectious bronchiolitis obliterans occurring in childhood. It is characterized by hypoplasia and/or agenesis of the pulmonary arteries resulting in pulmonary parenchyma hypoperfusion. CASE PRESENTATION: Here we report the case of a 53-year-old Caucasian woman with Swyer-James-MacLeod syndrome found in the differential diagnosis workup for a new onset of heart failure, secondary to pulmonary arterial hypertension complicated by a patent ductus arteriosus. CONCLUSION: Typically, this disorder is diagnosed in childhood after evaluation for recurrent respiratory infections, but sometimes an indolent course means diagnosis is not made until adulthood.