[Pulmonary Hypertension and Polycythemia vera]

Area of Science:

• Cardiology
• Hematology
• Pulmonary Medicine

Background:

• Pulmonary hypertension (PH) is a rare complication of myeloproliferative diseases (MPNs), classified under NICE classification 2018.
• MPNs, such as polycythemia vera (PV), can present with or develop PH, showcasing diverse clinical trajectories.

Observation:

• Two case reports detail elderly female patients with MPNs and subsequent PH development.
• One patient developed PH before PV diagnosis, while the other developed PH in the context of established PV.
• Both cases exhibited progressive dyspnea, right heart catheterization findings, and ultimately, cardiac decompensation despite various therapies.

Findings:

• The combination of PV and PH presented with varied temporal sequences.
• PH-specific therapies demonstrated limited influence on disease course and outcomes.
• Progressive cardiac decompensation was a common outcome in both reported cases.

Implications:

• Screening for PH is crucial in patients diagnosed with myeloproliferative diseases.
• The underlying pathomechanisms of PH in MPNs require further investigation to optimize therapeutic strategies.
• The efficacy of current PH-specific treatments in the context of MPNs remains uncertain, necessitating a re-evaluation of treatment approaches.