MYELOPROLIFERATIVE NEOPLASMS AND PULMONARY HYPERTENSION: AN ASSOCIATION REDEFINED

Abstract

SESSION TITLE: Pulmonary Vascular Disease 2 SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/10/2018 01:00 PM - 02:00 PM PURPOSE: The association between pulmonary arterial hypertension (PAH) and myeloproliferative neoplasms (MPNs) is described for each of the 4 distinct MPN subtypes; essential thrombocythemia (ET), polycythemia vera (PCV), primary myelofibrosis (PMF) and chronic myelogenous leukemia (CML). These associations are largely based on small studies, often using echocardiographic parameters as the basis for PH diagnosis. No study has closely evaluated the hemodynamic features of patients with CMPD associated PH. We sought to characterize the hemodynamic features of this cohort by right heart catheterization and compare those to echocardiogram (TTE) findings. We also evaluated whether hemodynamic differences exist among distinct MPN subtypes. METHODS: This single-center retrospective analysis included patients over 18 years old with an MPN and a documented right heart catheterization (RHC) between the years of 2004 and 2017. The MPN subtype was determined by ICD-9 code. Clinical characteristics nearest the time of right heart catheterization were recorded. PH was diagnosed by TTE if right ventricular systolic pressure (RVSP) was >35mmHg and by RHC if the mean pulmonary artery (PA) pressure was >25mmHg and PA occlusion pressure (PAOP) <15mmHg. Linear regression was used to determine association between TTE and RHC RVSP. One-way ANOVA was used to determine hemodynamic differences between CMPD subtypes. RESULTS: 74 subjects met inclusion criteria. They were predominantly male (58.1%) and Caucasian (87.8%). Median age was 63 years (+/-13yrs). MPN subtypes included PCV (35.1%), ET (24.3%), CML (22.9%), and PMF (16.2%). RHC indices showed mean PA pressure 36.3 (+/- 12 mmHg), PA occlusion pressure 18 (+/-7.4 mmHg), and cardiac output 5.3 (+/- 1.8 l/min). Median time between RHC and TTE was 13 months (IQR 2.5-39). The mean RVSP derived by TTE was 60 (+/-22) mmHg). This did not differ from RHC derived mean RVSP of 57 (+/-19) mmHg. By TTE, 90.6% of patients had PH. Although 84% of patients had mean PA pressure >25mmHg on RHC, a majority of those had PAOP > 15mmHg. Only 28.5% of subjects met full criteria for PAH by RHC. No differences in RVSP (on TTE) or hemodynamic indices were identified between subtypes of MPN. CONCLUSIONS: Although echocardiographic evaluation closely reflects PA catheter derived RVSP, many of these patients demonstrate pulmonary venous hypertension. Thus, echo may not be a reliable substitution for RHC to study or diagnose PAH in this cohort. Among patients with MPN and PAH, hemodynamics do not differ by MPN subtype. CLINICAL IMPLICATIONS: Echo while useful to evaluate PH in patients with MPN, is not a reliable alternative to diagnose PAH and echo criteria likely significantly overestimate the actual prevalence of PAH in clinical practice. DISCLOSURES: Advisory Committee Member relationship with Bayer Please note: $5001 - $20000 Added 03/04/2018 by Neal Chaisson, source=Web Response, value=Consulting fee Speaker/Speaker's Bureau relationship with Gilead Please note: $5001 - $20000 Added 03/04/2018 by Neal Chaisson, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Actelion Please note: $5001 - $20000 Added 03/04/2018 by Neal Chaisson, source=Web Response, value=Consulting fee Consultant relationship with American College of Physicians Please note: $1001 - $5000 Added 03/04/2018 by Neal Chaisson, source=Web Response, value=Honoraria Removed 03/04/2018 by Neal Chaisson, source=Web Response Consultant relationship with Schlesimger Please note: $1-$1000 Added 03/04/2018 by Neal Chaisson, source=Web Response, value=Consulting fee Removed 03/04/2018 by Neal Chaisson, source=Web Response Consultant relationship with Putnam Associates Please note: $1-$1000 Added 03/04/2018 by Neal Chaisson, source=Web Response, value=Consulting fee Removed 03/04/2018 by Neal Chaisson, source=Web Response No relevant relationships by Nauman Khan, source=Web Response