10Sep 2023
Clinical Phenotype and Outcomes of Pulmonary Hypertension Associated with Myeloproliferative Neoplasms

Clinical Phenotype and Outcomes of Pulmonary Hypertension Associated with Myeloproliferative Neoplasms

Precapillary pulmonary hypertension (PH) emerges as an uncommon, often overlooked complication in myeloproliferative neoplasms (MPNs) like polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (MF). French PH registry data illuminates this issue, revealing that 90 MPN patients (42 PV, 35 ET, 13 primary MF) suffer from severe precapillary PH, marked by high mean pulmonary arterial pressure and vascular resistance, and impaired functional status. Half of these cases were attributed to chronic thromboembolic PH (CTEPH), with the rest falling under group 5 PH. Recognizing these risks is vital for MPN patients, especially in group 5 PH, where underlying mechanisms remain unclear.

  • #critical care

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