Pulmonary Hypertension Prevalent Among Patients With Myelofibrosis

Daehyun Lee, MD, Moffitt Cancer Center, Tampa, Florida, shares results from a retrospective cohort study which identified an increased risk for cardiovascular morbidity, including heart failure, among patients with myelofibrosis who also have pulmonary hypertension.

According to Dr Lee, “Although a prospective study is needed for definitive evidence, we would cautiously recommend that our malignant hematologist consider an echocardiogram in patients with preexisting risk factors of cardiovascular disease, including hypertension, diabetes, atrial fibrillation, as well as coronary artery disease.” 

Transcript:

Good morning, everyone. Thank you for the opportunity to talk about our project. My name is Daehyun Lee. I'm a cardio-oncologist at Moffitt Cancer Center and University of South Florida. The title of our abstract is the Prevalence and Outcomes of Pulmonary Hypertension in Myelofibrosis. The first author of this abstract is our Rising Student Doctor, medical student, Alexis Behne Sharma.

I want to start off by saying patients with myelofibrosis often have various cardiovascular disease and risk factors, including pulmonary hypertension. Several studies have already published that pulmonary hypertension is quite common in patients with myelofibrosis, and the risk factors include older age and preexisting cardiovascular disease. Oftentimes, pulmonary hypertension could be considered as a marker of preexisting cardiovascular disease that are not yet diagnosed. The aim of our study is to comprehensively investigate the prevalence, the risk factors, and the clinical outcomes of pulmonary hypertension in patients with myelofibrosis.

We performed a retrospective analysis in 629 patients with myelofibrosis in our Moffitt Cancer Center registry in Tampa, Florida. Of these patients, 208 patients had an echocardiogram performed, which was the method of how we diagnosed pulmonary hypertension. Out of the 208 patients with echocardiogram, there were 61 patients with pulmonary hypertension, which is about 39% of patients who underwent echocardiogram, had pulmonary hypertension. The patients with pulmonary hypertension were older. They had a higher prevalence of hypertension also, and atrial fibrillation. There was no difference in rates of prior VTE or JAK2 inhibitor use. Overall, in patients with myelofibrosis, new onset significant cardiovascular disease was quite common.

We defined significant cardiovascular disease as patients with new onset heart failure, new onset heart attack, and new onset stroke. In the cardiology field, we call them MACE, which is major adverse cardiovascular event, and it is quite striking that 20% of all patients with myelofibrosis develop this MACE. Anyhow, the new onset heart failure was the most common heart disease that occurred after the diagnosis of myelofibrosis with an incidence of 13%, and the total MACE was 20%. Of course, the patients with pulmonary hypertension were twice more likely to develop new onset heart failure.

Lastly, in our study, we investigated the relationship between pulmonary hypertension and overall survival in patients with myelofibrosis. Importantly, we performed a time dependent analysis with pulmonary hypertension status as a time dependent covariate as echocardiograms were not necessarily performed at baseline at the time of myelofibrosis diagnosis, but along the course of the natural history of myelofibrosis. In multi-variable Cox regression analysis, pulmonary hypertension was associated with inferior overall survival with an adjusted hazard ratio of 4.41 and statistically significant. This was after adjustment with clinically relevant covariate of myelofibrosis, which was age sex, DIPSS plus, as well as allogeneic stem cell transplant status. Pulmonary hypertension, inferior overall survival in patients with myelofibrosis.

The important message that I would like to send out from this study is that pulmonary hypertension is quite common in patients with myelofibrosis pulmonary hypertension is associated with increased risk of new onset heart failure, as well as inferior survival in patients with myelofibrosis. Although a prospective study is needed for definitive evidence, we would cautiously recommend that our malignant hematologist consider an echocardiogram in patients with preexisting risk factors of cardiovascular disease, including hypertension, diabetes, atrial fibrillation, as well as coronary artery disease.

Thank you very much for your attention.

Source:

Sharma AB, Wang E, Selvakumar S, et al. Incidence and outcome of pulmonary hypertension in myelofibrosis. Presented at 2025 ASCO Annual Meeting. May 30-June 3, 2025; Chicago, IL. Abstract e24001.