Predictors of Pulmonary Edema in PAH Following Prostacyclin Therapy

x-ray of lungs with edema
x-ray of lungs with edema
In patients with pulmonary arterial hypertension, high baseline right atrial pressure, left heart disease, and pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis are valuable predictors of pulmonary edema.

High baseline right atrial pressure (RAP), left heart disease (LHD), and pulmonary veno-occlusive disease (PVOD) or pulmonary capillary hemangiomatosis (PCH) are valuable predictors of pulmonary edema, according to a study published in CHEST.

In this retrospective cohort study, researchers sought to determine the characteristics of patients with pulmonary arterial hypertension (PAH) that are predictive of pulmonary edema. Patients had been diagnosed with group 1 PAH and enrolled in the Cleveland Clinic Pulmonary Hypertension Registry at the initiation of parenteral prostacyclin between 1997 and 2015 (N=155; median age, 51 years; 72% women). The study’s primary outcome was the development of pulmonary edema ≤7 days after starting parenteral prostacyclin therapy. Secondary outcomes were the length of stay in the intensive care unit and hospital, mortality at 3 and 6 months, and long-term transplant-free survival.

After prostacyclin therapy was initiated, 21.3% (n=33; 95% CI, 15.6%-28.7%) of the patients developed pulmonary edema. Connective tissue disease-associated PAH was significantly more prevalent in the patients who developed pulmonary edema (39.4% vs 18.9%; P =.045). These patients also had significantly higher RAP (17 mm Hg vs 10 mm Hg; P =.005) as well as a higher presence of ≥3 LHD risk factors (P =.031).

Pulmonary edema was associated with a mean increase of 4.5 days (95% CI, 1.4-7.5 days; P <.001) in length of hospital stay, a >4-fold increase in 6-month mortality (odds ratio, 4.3; 95% CI, 1.28-14.36; P =.031), and a 1.5-fold increased risk for death or lung transplant (hazard ratio, 1.54; 95% CI, 0.97-2.36; P =.06).

Limitations of this study included the inability to calculate the true incidence of pulmonary edema within the parameters of this retrospective cohort study design and few participants had chest computed tomography images for review. Investigators could not rule out some misclassifications occurring from nonspecific symptoms and radiologic signs.

Based on these findings, the researchers stressed the importance of higher suspicion for pulmonary edema venous involvement in patients with group 1 PAH. They further cautioned that LHD, found to be of predictive value for pulmonary edema, is often underdiagnosed in PAH.

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Disclosures: Multiple authors declare affiliations with the pharmaceutical industry. Please refer to reference for a complete list of authors’ disclosures.


Khan NA, Khan RA, Tonelli AR, et al. Pulmonary edema following initiation of a parenteral prostacyclin therapy for pulmonary arterial hypertension: a retrospective study [published online February 15, 2019]. CHEST. doi:10.1016/j.chest.2019.02.005