Open AccessRespiratory exacerbation in a young adult with cystic fibrosis and tricuspid atresia
Author(s) -
Wood Jamie,
Sawyer Abbey,
Mulrennan Siobhain,
Bullock Andrew
Publication year - 2018
Publication title -
respirology case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.304
H-Index - 9
ISSN - 2051-3380
DOI - 10.1002/rcr2.318
Subject(s) - medicine , tricuspid atresia , cardiology , exacerbation , tricuspid valve , pulmonary atresia , cystic fibrosis , hypoplastic left heart syndrome , pulmonary artery , respiratory system , hypoplasia , ventricle , fontan procedure , endomyocardial fibrosis , surgery , fibrosis , heart disease
Tricuspid atresia (TAt) is a complex congenital heart defect (CHD) characterized by the absence of the tricuspid valve and right ventricular hypoplasia requiring surgery in childhood, the Fontan procedure. We present a case of a 21‐year‐old male with TAt and cystic fibrosis (CF), who underwent a Fontan procedure in childhood, presenting to an adult CF clinic with severe deterioration in his respiratory status and multi‐organ dysfunction associated with CF. This report describes problems associated with the management of a CF respiratory exacerbation and extrapulmonary manifestations of CF in the unique situation of a Fontan circulation, a circulation with absence of a subpulmonary ventricle and pulsatile pulmonary arterial blood flow where maintenance of systemic cardiac output is totally dependent on good respiratory function and low pulmonary artery pressures.