Pulmonary Hypertension Suggest Worse Patient Outcomes in MPNs, CV Diseases

Findings from a single-center retrospective study investigated the prognostic implication of pulmonary hypertension (PH) in a high-risk myeloproliferative neoplasms (MPNs) patient population, particularly to analyze those at risk for cardiovascular disease. All data were shared at the 2021 Annual American Society of Hematology (ASH) Meeting.

“Cardiovascular (CV) disease, including atrial fibrillation (AF), heart failure (HF), and pulmonary hypertension (PH), are underappreciated causes of morbidity and mortality in MPNs. Additionally, MPNs harbor mutations commonly seen in clonal hematopoiesis of indeterminate potential (CHIP) which can be associated with CV risk,” explained Orly Leiva, MD, New York University Langone Health, NY, and co-investigators.

The study identified and enrolled 197 patients with MPNs, or subtypes of essential thrombocythemia (ET), polycythemia (PV), or primary myelofibrosis (PMF), who had CV disease and had undergone right heart catheterization (RHC) or transthoracic echocardiography (TTE).

“PH was defined as either a mean pulmonary artery pressure ≥ 20 mmHg on RHC or estimated right ventricular systolic pressure (RVSP) ≥ 50 mmHg on TTE,” continued Dr Leiva and co-authors.

The primary outcome was CV death. Secondary outcomes included all-cause mortality, HF hospitalization, arterial (ATE), and venous thrombosis (VTE). A Fine-Gray model was used to determine the effect of PH on CV death using other causes of death as competing events.

“To investigate the effect of PH on our outcomes we also used a multivariable logistic regression model using age, sex, MPN type, MPN treatment, ATE and VTE prior to and after MPN diagnosis, diabetes, prior HF diagnosis, HF hospitalization, AF, hyperlipidemia, leukemic or secondary MF transformation as co-variables,” said Dr Leiva and co-investigators.

In all, 92 patients with PH and 105 patients without PH were analyzed with a median age at MPNs diagnosis being higher in PH vs non-PH patients (73 versus 71, p=0.038). The median time to diagnosis of PH from MPNs was 69 months (IQR 43, 133).

Notably, patients with PH expressed higher rates of prior HF (54% vs 32%, P=0.001) and hypertension (83% vs 64%, P=0.004), but lower rates of AF (64% vs 79%, P=0.026) compared with patients without PH. Further, MPNs types and driver mutations were not statistically significant between PH and non-PH groups.

Other findings showed more patients with PH had leukemic or secondary MF transformation compared to patients without PH (23% vs 11%, P=0.037). Patients with PH had higher rates of CV death (35% vs 9%, P<0.0001), all-cause death (58% vs 37%, P=0.004), HF hospitalization (73% vs 36%, P<0.0001), and VTE (25% vs 12%, P=0.027), but not ATE (39% vs 41%, P=0.88).

“In patients with MPNs and CV diseases, PH is associated with worse outcomes including CV death, VTE, HF hospitalization and leukemic/secondary MF transformation compared with patients without PH. More investigation is needed but our results suggest early screening for PH may be beneficial in this patient population,” concluded Dr Levia, et al.—Alexa Stoia

Leiva O, Ren S, Neuberg D, et al. Pulmonary Hypertension Is Associated with Poor Outcomes in Patients with Myeloproliferative Neoplasms in Cardiovascular DIsease. Presented at: Presented at: the 2021 ASH Annual Meeting; Dec. 11-14, 2021; Abstract 3653.