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Treatment of Pulmonary Hypertension in the Setting of Interstitial Lung Disease with Intravenous Prostacyclin Therapy

Michael Duncan

S Krishnan

T Driscoll

April Blakley


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Conference: 2018 PHA International PH Conference & Scientific Sessions

Release Date: 06.28.2018

Presentation Type: Abstracts

File Download: Conference 2018_1066

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2018 International PH Conference and Scientific SeAbstract presented at the 2018 International PH Conference and Scientific Sessions in Orlando, Fla., June 28-July 1, 2018.


Pulmonary hypertension (PH) in the presence of interstitial lung disease (ILD) is associated with an increase in mortality.1-2 PH due to ILD is categorized as PH World Health Organization (WHO) Group 3. Currently, PH specific medications available on the market are approved by the Food and Drug Administration (FDA) only for PH WHO Group 1 and Group 4. Intravenous (IV) prostacyclin therapy has been successfully utilized before in the setting of ILD. Reported here is a case of a patient with PH WHO Group 3 due to ILD and profound treatment response to IV prostacyclin therapy.


61 year old African American male with a history of pulmonary fibrosis was referred with worsening hypoxia for emergent lung transplant evaluation. Upon admission, the patient required Optiflow at 40 L per minute at 80% inspired oxygen to maintain oxygen saturations greater than 90% at rest. An echocardiogram (ECHO) showed severely reduced right ventricular ejection fraction, moderate pericardial effusion and estimated right ventricular systolic pressure (RVSP) of 100 mmHg. Consequently, a right heart catheterization (RHC) was performed which revealed a mean pulmonary artery (PA) pressure of 45 mmHg, cardiac index of 1.6, pulmonary capillary wedge pressure (PCWP) of 11 mmHg, and a pulmonary vascular resistance (PVR) of 11.9 Woods Units. Though the patient had chest computed tomography (CT) findings consistent with moderate ILD, his PH appeared out of proportion to his lung disease. Therefore, treatment was initiated in an intensive care unit (ICU) setting with treprostinil IV, initiated at 2 ng/kg/min and slowly titrated to 14 ng/kg/min, and sildenafil 20 mg by mouth three times daily. Repeat ECHO, 4 weeks after initial, estimated right ventricle (RV) mean pressure to be 40 mmHg with resolution of the pericardial effusion. The patient was successfully discharged to home requiring no supplemental oxygen at rest or with activity.


Pulmonary vasodilator therapy is only FDA approved for PH WHO Group 1 and Group 4. If therapy is used for PH WHO Group 3, IV therapy is usually avoided due to concern for worsening ventilation perfusion mismatch. In our case, we showed that there is a role for IV therapy in the treatment of PH WHO Group 3 in the setting of ILD. Both prostacyclin therapy with treprostinil IV, and phosphodiesterase-5 (PDE-5) enzyme inhibitor therapy with sildenafil, were utilized. This case demonstrates that intravenous pulmonary vasodilator therapy can safely and effectively be used in carefully selected patients with PH WHO Group 3 due to ILD. This case also emphasizes the importance of exploring all potential treatable etiologies of worsening hypoxia in a patient with advanced lung disease who presents for emergent lung transplant evaluation.