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Pulmonary Vascular Disease |

Initiation of Pulmonary Arterial Hypertension Specific Therapy Is Well Tolerated in Patients With Severe Lung Disease and Associated WHO Group III Pulmonary Hypertension

Justin Roberts, DO; Rebecca Cogswell, MD; Jason Rasmussen, MD; Cathy O'Brien, NP-C; Emily Aviles, NP-C; Alethea Anderson, RN; Batul Mehdi, RN; Anna Krogman; Marc Pritzker, MD
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University of Minnesota, Minneapolis, MN


Chest. 2013;144(4_MeetingAbstracts):862A. doi:10.1378/chest.1705303
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Abstract

SESSION TITLE: DVT/PE/Pulmonary Hypertension Posters

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 30, 2013 at 01:30 PM - 02:30 PM

PURPOSE: Patients with severe lung disease and secondary pulmonary hypertension (WHO Group III PH) have not been well studied in clinical practice. Concern exists about worsening hypoxia and ventilation-perfusion (V/Q) mismatch with initiation of pulmonary arterial hypertension (PAH) specific therapies in this population. We sought to determine the safety profile of these therapies in an inpatient, monitored setting.

METHODS: Serial WHO Group III PH patients (n=22) were electively admitted for initiation of PAH therapy to determine acute tolerance. The etiology of lung disease was IPF, ILD and COPD. No patients with connective tissue or chronic thromboembolic disease were included. Clinical and hemodynamic variables were monitored and analyzed.

RESULTS: The patients enrolled had a mean FEV1 of 1.6 ± 0.6 L, a percent predicted DLCO of 25 ± 11.1, a baseline oxygen requirement of 3.5 ± 2.2 L and peripheral saturation of 94.7 ± 2.6%. The baseline PVR was 9.2 ± 4.3 Wood Units and the mean cardiac index was 2 ± 0.6 L/min/m2. Therapy consisted of PDE5 inhibitors (n=17) and inhaled or intravenous prostacyclin (n=5). Ninety-one percent (20/22) of the patients tolerated therapy and were discharged on PH medications. During initiation of therapy, there was an average increase in oxygen requirement of 2 L (p<0.005) from baseline, which decreased to non-significant by the time of discharge (+0.5 L, p = 0.24), and oxygen saturations at the time of discharge were unchanged from prior (0, p= 0.5). Mean arterial pressure dropped by an average of 8.5 mmHg (p<0.005). Two patients did not tolerate initiation of therapy due to shortness of breath (n=1) or desaturation of more than 10 % from baseline (n=1).

CONCLUSIONS: In this series, patients with severe lung disease and associated PH tolerated conventional PH therapy. While oxygen requirements temporarily increased during initiation, these returned to baseline at steady state of therapy.

CLINICAL IMPLICATIONS: Patients with severe underlying lung disease and severe PH tolerate therapy without significant changes in oxygen requirements.

DISCLOSURE: The following authors have nothing to disclose: Justin Roberts, Rebecca Cogswell, Jason Rasmussen, Cathy O'Brien, Emily Aviles, Alethea Anderson, Batul Mehdi, Anna Krogman, Marc Pritzker

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