Transplantation |

Independent Association Between Cystic Fibrosis and Pulmonary Hypertension in Patients Listed for Lung Transplant FREE TO VIEW

Nona Mei, MD; Alex Balekian, MD
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University of Southern California, Los Angeles, CA

Chest. 2014;145(3_MeetingAbstracts):640A. doi:10.1378/chest.1826323
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SESSION TITLE: Transplantation

SESSION TYPE: Slide Presentations

PRESENTED ON: Sunday, March 23, 2014 at 12:15 PM - 01:15 PM

PURPOSE: Cor pulmonale can complicate end-stage lung disease in chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), and cystic fibrosis (CF). Although the prevalence of pulmonary hypertension (PH) has been described in CF patients, these studies often drew small sample sizes from single centers. We aimed to describe the independent association between CF and PH compared to COPD and IPF.

METHODS: Retrospective, cross-sectional review of UNOS data for all patients listed for lung transplant between 1991 and 2012. We excluded patients with idiopathic PH and prior lung transplants. We used analysis of covariance to calculate our main outcome, mean pulmonary artery pressure (mPAP) at time of listing, in CF, COPD, and IPF patients after adjusting for lung allocation score (LAS), age, body mass index (BMI), and sex; we also used multivariable logistic regression to report the association between PH (mPAP>= 25 mm Hg) and CF, COPD, and IPF at time of listing after similar adjustment.

RESULTS: Of the 12,470 patients with complete data, 56% were male, 37% had COPD, 11% had CF, and 52% had IPF; mean age was 54.7 years, and mean BMI was 25.4. Unadjusted mPAP was 25.8 vs 26.2 vs 27.2 mm Hg for COPD, CF, and IPF, respectively (p<0.001). The prevalence of PH was 53%, 55%, and 51% for COPD, CF, and IPF (p<0.02). After adjustment for age, BMI, and LAS, mPAP was 27.3 vs 25.0 vs 26.4 mm Hg for COPD, CF, and IPF, respectively (p<0.001). When using IPF patients as a baseline comparator in univariate analysis, PH was associated with a diagnosis of COPD (OR 1.06, 95% CI 1.01 - 1.12) and CF (OR 1.13, 95% CI 1.03 - 1.23). After adjustment for age, sex, LAS, and BMI, this association with PH became stronger for both COPD (OR 1.72, 95% CI 1.58 - 1.88) and CF (OR 1.40, 95% CI 1.18 - 1.65).

CONCLUSIONS: PH occurs in over half of patients with COPD, CF, and IPF at time of lung transplant listing. CF and COPD have independent associations with PH when compared to IPF.

CLINICAL IMPLICATIONS: Because PH has been associated with higher lung transplant wait list mortality, CF patients might be considered for PH treatment similar to patients with COPD or IPF.

DISCLOSURE: The following authors have nothing to disclose: Nona Mei, Alex Balekian

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