Researchers Investigate the Effect of Pulmonary Hypertension Risk on Survival Outcomes in PV

A research team at Weill Cornell Medical College, New York, New York, observed in a study of patients with polycythemia vera (PV) that pulmonary hypertension (PH) risk is linked to reduced overall survival (OS). The team published their findings in Blood Advances.

“Based upon these results and receiver operator characteristic optimization, we recommend echocardiographic screening for patients with PV aged >70 years or with duration of PV of >8 years,” wrote first author Alexander J Gazda, DO, and colleagues.

In this study, the researchers defined PH risk according to echocardiography (ECHO)–assessed tricuspid regurgitant jet velocity (TRV) measurements, with mild risk defined as TRV greater than 2.8 m/s up to 3.4 m/s, and high risk, as TRV greater than 3.4 m/s. Increased PH risk in binary analysis was defined as TRV greater than 2.8 m/s.

From the electronic medical records of 637 patients with PV at a single center, 134 patients were identified as being at risk for PH. The cohort was 53% women with a median age of 73 years at ECHO assessment and median BMI of 25. In the cohort, 37% of patients had history of either arterial or venous thrombosis, 57% had systemic hypertension, and 47% had hyperlipidemia. A wide range of PV management approaches were being used, but the most common were antiplatelet, anticoagulant, and cytoreductive therapies.

The researchers found that patients with increased PH risk, as compared with those with normal risk, were older with a median age of 78 years versus 70 years, respectively (P<0.001) and had a much longer median duration of PV before ECHO assessment at 11 years versus 3 years, respectively (P=0.002). However, there were no statistically significant differences in PH risk found among the 3 risk groups based on sex or medical history, including history of thrombosis.

“Similarly, neither PH risk tertile, nor patient age was linked to the cumulative incidence of thrombosis during the follow-up period,” mentioned Gazda and colleagues.

Over the study period, the mortality rate was greater among patients with increased PH risk than among those with normal risk, at 67% versus 33%, respectively (P=0.001).

The researchers’ Kaplan-Meier survival curve analysis showed that patients with normal PH risk had higher 10-year OS than patients with increased risk, at 57% versus 18%, respectively (P=0.0024). The 10-year OS for patients with normal, mild, and high PH risk was 57%, less than 21%, and less than 25%, respectively (P=0.003). Median survival was 1.8 years among patients with high PH risk, 3.7 years among those with mild PH risk, and not yet reached for patients with normal PH risk.

“Despite its importance in assessing future thrombosis risk in PV, we did not find thrombosis history predictive of OS in our cohort,” Gazda and colleagues noted.

The Cox proportional hazards analysis conducted by the researchers revealed that having any increase in PH risk was associated with mortality risk, with a hazard ratio of 2.5. Their multivariate Cox model revealed that high PH risk was associated with a 3 times greater risk of mortality that was independent of age and thrombosis history. Logistic regression showed that patient age and duration of PV diagnosis were strongly linked to risk for PH, with odds ratios of 6.9 and 5.4, respectively.

“These results suggest that ECHO-based PH risk in PV is largely driven by mechanisms other than thrombosis, such as increased blood viscosity, diastolic dysfunction, and inflammatory changes, and that the excess mortality because of PH risk in PV is not from thrombotic events and CTEPH [chronic thromboembolic PH],” Gazda and colleagues summarized.

Reference

Gazda AJ, Pan D, Erdos K, et al. High pulmonary hypertension risk by echocardiogram shortens survival in polycythemia vera. Blood Adv. 2025;9(6):1320-1329. doi:10.1182/bloodadvances.2024013970