Pulmonary Vascular Disease |

Prevalence of Pulmonary Arterial Hypertension (PAH) in Patients With Idiopathic Pulmonary Fibrosis (IPF) FREE TO VIEW

Adolfo Villar, MD; Julio Ancochea, MD; Antonio Xaubet, MD
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Complexo Hospitalario Universitario de Pontevedra, Pontevedra, Spain

Chest. 2014;145(3_MeetingAbstracts):512A. doi:10.1378/chest.1816876
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SESSION TITLE: Pulmonary Hypertension Posters II

SESSION TYPE: Poster Presentations

PRESENTED ON: Saturday, March 22, 2014 at 01:15 PM - 02:15 PM

PURPOSE: The prevalence of PAH in patients with IPF is not well known. There has been no prospective study to date. The purpose of our study was to know the prevalence of PAH in patients with IPF at any stage of severity

METHODS: IPF patients diagnosed by clinical-radiological or pathological criteria were included. All patients underwent echocardiography. If the systolic pulmonary arterial pressure was greater than 40 mmHg, right heart catheterization was performed. A mean pulmonary artery pressure greater than or equal to 25 mmHg with a wedge pressure less than 16 mmHg was considered diagnostic of PAH.

RESULTS: 60 patients (18 women, age at diagnosis 64 years) were included and 56 completed the study protocol. Patient characteristics: FVC 64%, DLCO 41%, 6MWT 357 meters, systolic pulmonary arterial pressure 46 mmHg. 17 patients showed a systolic pulmonary artery pressure greater than 40 mmHg and in 14 of them PAH was confirmed by right heart catheterization with a mean pulmonary arterial pressure of 28.6 mmHg. When comparing various clinical and functional parameters between patients with and without PAH significant differences were seen in FVC, FEV1, DLCO, 6MWT and Functional Class. In a multivariate analysis DLCO was associated with the presence of PAH

CONCLUSIONS: PAH is not infrequent in patients with IPF (around 26%).Patients with more severe disease are more likely to develop PAH. A marked decrease in DLCO is independently associated with the presence of PAH

CLINICAL IMPLICATIONS: PAH is a devastating disease with prognostic and therapeutic implications in patients with IPF. To know the prevalence and associated factors in very important for a better management of this patients because we now have effective treatments in PAH

DISCLOSURE: The following authors have nothing to disclose: Adolfo Villar, Julio Ancochea, Antonio Xaubet

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