Open AccessPulmonary hypertension as estimated by Doppler echocardiography in adolescent and adult patients with cystic fibrosis and their relationship with clinical, lung function and sleep findings
Author(s) -
Ziegler Bruna,
Perin Christiano,
Casarotto Fernanda Cano,
Fagondes Simone Chaves,
MennaBarreto Sérgio Saldanha,
Dalcin Paulo de Tarso Roth
Publication year - 2018
Publication title -
the clinical respiratory journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.789
H-Index - 33
eISSN - 1752-699X
pISSN - 1752-6981
DOI - 10.1111/crj.12590
Subject(s) - medicine , polysomnography , hypoxemia , cardiology , pittsburgh sleep quality index , cystic fibrosis , pulmonary function testing , univariate analysis , pulmonary hypertension , body mass index , epworth sleepiness scale , sleep study , apnea , multivariate analysis , cognition , psychiatry , sleep quality
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 (SpO 2 ), SpO 2 at 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 SpO 2 (OR = 10.8, CI 95% 1.7‐67.3, P = .011). The cuttoff SpO 2 < 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. Conclusion the present study showed a high rate of PH in adolescent and adult CF patients. At‐rest SpO 2 was associated with PH.