Incidence of Pulmonary Edema After Initiation of Parenteral Prostacyclin Therapy for Group I Pulmonary Arterial Hypertension

Abstract

SESSION TITLE: Pulmonary Hypertension - Advances in Management and Therapy SESSION TYPE: Original Investigation Slide PRESENTED ON: Tuesday, October 31, 2017 at 02:45 PM - 04:15 PM PURPOSE: To determine the incidence of pulmonary edema after initiation of parenteral prostacyclin therapy in patients with Group I PAH without suspicion of pulmonary veno-occlusive disease (PVOD) METHODS: A retrospective cohort study of Group I PAH patients without suspicion of PVOD treated with intravenous prostacyclin therapy at the Cleveland Clinic. We determined the incidence of pulmonary edema after initiation of prostacyclin therapy. New onset pulmonary edema was defined as the presence of at least one symptom of pulmonary congestion (worsening dyspnea, orthopnea) and at least one sign of pulmonary edema (new or worsening rales on pulmonary examination or signs of new or worsening pulmonary congestion on chest radiograph). Chest radiograph signs of pulmonary congestion were defined as at least one of the following: Kerley B lines, interstitial infiltrates, vascular cephalization, alveolar infiltrates or pleural effusions. We also analyzed the association between pulmonary edema and hospital LOS, as well as mortality. RESULTS: A total of 155 patients were included in the study. Median age was 50 years (IQR 38-57), females 127 (82%), Caucasian 132 (85%), etiology was predominantly idiopathic PAH 98 (63%), followed by CTD-PAH 36 (23%). Median left ventricular ejection fraction was 55% (IQR 55-60). On right heart catheterization median PAP was 54 (IQR 47-64), median PCWP 12 (IQR 8-16), median RAP 10 (IQR 5-16), median CI 1.9 (IQR 1.6-2.5) and median DLCO 58% (IQR 40-73). The predominant prostacyclin analogue used was IV epoprostenol in 134 (86%), followed by IV treprostinil in 18 (11.6%) and SQ treprostinil in 3 (2%). Median prostacyclin initiation dose of epoprostenol was 2ng/kg/min (IQR 2-3) and for treprostinil was 2ng/kg/min (IQR 1.25-3.25). Median hospital LOS was 6 days (IQR 4-8). Pulmonary edema occurred in 33 (21%) of patients after initiation of prostacyclin therapy. Median duration to onset of pulmonary edema was 2 days (IQR 1-4). 16 (48%) patients required intravenous diuretic therapy, 8 (24%) required de-escalation/discontinuation of prostacyclin therapy. Two patients required invasive mechanical ventilation. The development of pulmonary edema was associated with a longer hospital LOS with mean increase in 2.2 days, (95% CI 1.0- 3.4, p< 0.001) and increased 6-month mortality (OR 4.3, 95% CI 1.3 - 14.8, p<0.05). CONCLUSIONS: Pulmonary edema occurred in 21% of patients with group 1 PAH without suspicion of PVOD after the initiation of parenteral prostacyclin therapy. The development of pulmonary edema was associated with prolonged hospital LOS and increased 6-month mortality. CLINICAL IMPLICATIONS: Pulmonary edema occurs in 1 in 5 PAH patients treated with parenteral prostacyclin therapy. This is associated with worse outcomes and is in line with recently emerging evidence pointing at a high prevalence of microscopic vein involvement in PAH. DISCLOSURE: The following authors have nothing to disclose: Nauman Khan, Rizwan Khan, Raed Dweik, Gustavo Heresi No Product/Research Disclosure Information