Persistent Pulmonary Hypertension as Clue for Swyer-James-MacLeod Syndrome

Introduction

Swyer-James-MacLeod syndrome (SJMS) is a rare condition occurring after childhood bronchiolitis obliterans or pneumonitis (Sen HS et al. Respir Care 2014 Apr; 59(4): e51–4). Different viral (e. g. adenovirus, respiratory syncytial virus) and bacterial (bordetella pertussis, mycoplasma pneumoniae) triggers have been implicated in the development of SJMS (Lucaya J et al., J Comput Assist Tomogr 1998 & Trimis G et al. Scand J Infect Dis 2003; 35: 197). In SJMS hyperactive or misdirected repair processes lead to formation of granulation tissue and submucosal fibrosis that cause airway obstruction (Dirweesh et al., Respir Med Case Rep 2017; 20: 104–106). Interalveolar fibrosis impairs pulmonary capillary blood flow and overinflation of alveolae due to bronchoconstriction further reduces pulmonary perfusion. As this process mainly occurs in early childhood pulmonal arterial development is impaired (Sulaiman A et al. Interact Cardiovasc Thorac Surg 2009 Apr; 8(4): 482–4). Reduced ventilation and blood flow restrict lung growth. The combination of emphysema and rarefication of the pulmonary vasculature lead to the typical radiographic presentation with unilateral pulmonary hyperlucency and volume-reduction of the affected pulmonary areas.

Publikationsverlauf

Artikel online veröffentlicht:
15. Dezember 2021