title: Pulmonary hypertension as estimated by Doppler echocardiography in adolescent and adult patients with cystic fibrosis and their relationship with clinical, lung function and sleep findings.
abstract: BACKGROUND
  In cystic fibrosis (CF) patients, end stage of pulmonary disease is characterized by pulmonary hypertension (PH), hypoxemia, decrease in exercise tolerance, and sleep quality.
  
  
  OBJECTIVE
  To evaluate the association between clinical, lung function, sleep quality, and polysomnographic variables with PH in CF patients aged 16 years or older.
  
  
  METHODS
  In a cross-sectional study, 51 clinically stable CF patients underwent a clinical evaluation, an overnight polysomnography and answered sleep questionnaires (Pittsburgh Sleep Quality Index and Epworth sleepiness scale). Also, CF patients had their pulmonary function, 6-minute walk test (6MWT) and echocardiography assessed.
  
  
  RESULTS
  Fifty-one CF patients participated in the study; 47% were female. The mean age was 25.1 ± 8.8 years. Pulmonary artery systolic pressure (PASP) was greater than 35 mm Hg in 11 (27.5%) patients. Variables associated with PASP>35 mm Hg in univariate analysis were Shwachman-Kulczycki clinical score, forced expiratory volume in 1 second % of predicted, Pseudomonas aeruginosa in sputum culture, at-rest peripheral capillary oxygen saturation (SpO2), SpO2at end of 6MWT and time of oxygen desaturation <90% during sleep. These variables were included in the binary logistic regression. The independent variable associated with the PASP > 35 mm Hg was at-rest SpO2(OR = 10.8, CI 95% 1.7-67.3, P = .011). The cuttoff SpO2< 94% had the sensitivity = 7/11 = 64%, specificity = 40/40 = 100%, positive predicted values = 7/7 = 100% and negative predicted values = 40/44 = 91% to the diagnosis of PH.
  
  
  CONCLUSIONS
  the present study showed a high rate of PH in adolescent and adult CF patients. At-rest SpO2was associated with PH.
authors.affiliation: Serviço de Fisioterapia, Physiotherapist, Hospital de Clínicas de Porto Alegre, Postgraduate Program in Ciências Pneumológicas, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.
authors.forename: Bruna
authors.initials: B
authors.lastname: Ziegler
authors.affiliation: Serviço de Pneumologia, Postgraduate Program in Ciências Pneumológicas, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.
authors.forename: Christiano
authors.initials: C
authors.lastname: Perin
authors.affiliation: Serviço de Pneumologia, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.
authors.forename: Fernanda Cano
authors.initials: FC
authors.lastname: Casarotto
authors.affiliation: Serviço de Pneumologia, Postgraduate Program in Ciências Pneumológicas, Universidade Federal do Rio Grande do Sul; Hospital de Cínicas de Porto Alegre, Porto Alegre, Brazil.
authors.forename: Simone Chaves
authors.initials: SC
authors.lastname: Fagondes
authors.affiliation: Serviço de Pneumologia, Professor of Medicine, Universidade Federal do Rio Grande do Sul, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.
authors.forename: Sérgio Saldanha
authors.initials: SS
authors.lastname: Menna-Barreto
authors.affiliation: Professor of Medicine, Faculdade de Medicina, Posgraduate Program, Universidade Federal do Rio Grande do Sul; Serviço de Pneumologia, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil.
authors.forename: Paulo de Tarso Roth
authors.initials: PTR
authors.lastname: Dalcin
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001938:Brazil; D016009:Chi-Square Distribution; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging*; D015150:Echocardiography, Doppler / Q000379:methods; D005080:Exercise Test; D017079:Exercise Tolerance / Q000502:physiology*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000209:etiology*; D015994:Incidence; D008297:Male; D017286:Polysomnography / Q000379:methods; D011237:Predictive Value of Tests; D011379:Prognosis; D012129:Respiratory Function Tests; D018570:Risk Assessment; D012720:Severity of Illness Index; D012737:Sex Factors; D012893:Sleep Wake Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D018709:Statistics, Nonparametric; D062606:Tertiary Care Centers
keywords: cystic fibrosis; doppler echocardiography; polysomnography; pulmonary hypertension