Impact of echocardiographic hemodynamics on outcomes among patients with polycythemia vera or essential thrombocythemia.

INTRODUCTION

Pulmonary hypertension (PH) and high-output heart failure (HOHF) are associated with myeloproliferative neoplasms (MPNs), including essential thrombocythemia (ET) and polycythemia vera (PV). Transthoracic echocardiography (TTE) is a non-invasive modality that can be used for the screening of PH and HOHF. However, the impact of echocardiographic HOHF on TTE on outcomes of patients with ET or PV is unclear.

METHODS

This was a multicenter, retrospective cohort study of patients with ET or PV with ≥ 1 TTE. Estimated pulmonary artery systolic pressure (PASP) and cardiac index were calculated using doppler TTE. Patients were categorized based on TTE hemodynamics as echocardiographic HOHF (PASP > 40 mmHg and cardiac index > 3.54 L/min/m2), elevated PASP alone, elevated cardiac index alone, and normal PASP and cardiac index. Primary outcome was all-caused death or MPN disease progression.

RESULTS

A total of 272 patients (150 PV, 122 ET) were included, 16 (5.9%) with echocardiographic HOHF, 31 (11.4%) elevated cardiac index alone, 63 (23.2%) elevated PASP alone, and 147 (54.0%) with normal hemodynamics. After multivariable Cox proportional hazards regression, echocardiographic HOHF (adjusted HR 3.35, 95% CI 1.53-7.34) and elevated PASP alone (adjusted HR 2.89, 95% CI 1.70-4.92) were associated with increased of primary outcome. Increased risk of HF hospitalizations was seen across all abnormal hemodynamic vs. normal hemodynamics.

CONCLUSIONS

Among patients with ET or PV, echocardiographic HOHF and elevated PASP alone were associated with increased risk of death or MPN disease progression. Prospective studies are needed to identify patients at risk of HOHF and PH.