title: Burkholderia cepacia is associated with pulmonary hypertension and increased mortality among cystic fibrosis patients.
abstract: The aim of the study was to evaluate the impact of Burkholderia cepacia on cardiovascular status and mortality in cystic fibrosis. Seven patients infected with B. cepacia were matched with 31 patients not infected with this organism for gender, age, height, weight, genotype, and percent predicted forced expiratory volume in one second, partial arterial oxygen pressure, and pancreatic sufficiency status. The pulmonary artery systolic pressure, as assessed by transthoracic echocardiography, was significantly higher in patients infected with B. cepacia (61.3 +/- 17.2 mm Hg) than in controls (37.3 +/- 13.9 mm Hg; P = 0.02), and the mean acceleration time was significantly lower (77 +/- 33 ms versus 108 +/- 25 ms; P = 0.02). The 6-month mortality was significantly higher in patients infected with B. cepacia (57% versus 16%; P = 0.02). Six of the seven patients infected with B. cepacia harbored the same ribotype (genomovar II, B. multivorans). Pulmonary hypertension was significantly more frequent in patients infected by B. cepacia and could contribute to the increased mortality rate.
authors.affiliation: Paediatric Pulmonary Department and Research Unit INSERM E 213, Armand Trousseau Hospital, 28 avenue du Docteur Arnold Netter, 75012 Paris, France. brigitte.fauroux@trs.ap-hop-paris.fr
authors.forename: Brigitte
authors.initials: B
authors.lastname: Fauroux
authors.forename: Nicholas
authors.initials: N
authors.lastname: Hart
authors.forename: Samira
authors.initials: S
authors.lastname: Belfar
authors.forename: Michèle
authors.initials: M
authors.lastname: Boulé
authors.forename: Isabelle
authors.initials: I
authors.lastname: Tillous-Borde
authors.forename: Damien
authors.initials: D
authors.lastname: Bonnet
authors.forename: Edouard
authors.initials: E
authors.lastname: Bingen
authors.forename: Annick
authors.initials: A
authors.lastname: Clément
mesh_terms: D000293:Adolescent; D019121:Burkholderia Infections / Q000150:complications* / Q000382:microbiology; D016956:Burkholderia cepacia / Q000145:classification / Q000235:genetics / Q000302:isolation & purification*; D002648:Child; D003550:Cystic Fibrosis / Q000382:microbiology* / Q000401:mortality*; D004452:Echocardiography; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008297:Male; D012129:Respiratory Function Tests