Chest
Clinical InvestigationsPULMONARY VASCULATUREPulmonary Hypertension and Cardiac Function in Adult Cystic Fibrosis: Role of Hypoxemia
Section snippets
Patient Population
Adult patients (> 18 years old) with CF confirmed by an abnormal sweat test result were recruited from the Adult CF program at the Wellesley Hospital in Toronto. We recruited 18 patients with severe lung disease (FEV1 < 40% of the predicted normal value) and 15 patients with moderately severe lung disease (FEV1 40 to 65% predicted). All patients were in clinically stable condition over the month prior to the study with no exacerbations requiring antibiotic therapy or hospitalization. Patients
Patients With Severe Lung Disease
Four of the 18 patients studied were female and 14 were male. The average age was 30.2 years (range, 21 to 40) and the mean body mass index (BMI) was 21.9 kg/m2 (range, 18.3 to 31.2). Indices of pulmonary function are summarized in Table 1. All patients had severe, stable lung disease. Mild hypoxemia was present at rest, and two of our patients had evidence of CO2 retention with arterial Pco2 of 66 and 53 mm Hg.
Mean Sao2 and tco2 during wakefulness and sleep are summarized in Table 2. Mild
Discussion
Clinical cor pulmonale occurs in the terminal stages of CF and its onset carries a grave prognosis.28 The mean survival time from the onset of clinically apparent cor pulmonale has been reported to be as short as 8 months.2 The nearly universal finding of severe hypoxemia in association with clinical cor pulmonale is, therefore, not surprising. We chose to study patients with severe CF (FEV1 < 40% predicted) prior to the development of hypoxemia or clinically apparent cor pulmonale to
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Supported by the Canadian Cystic Fibrosis Foundation and the Ontario Thoracic Society.