Abstract
Background: Patients with myeloproliferative neoplasms (MPN) are known to complicate pulmonary hypertension (PH). Half of patients with MPN-associated PH have chronic thromboembolic PH (CTEPH); however, the prevalence, clinical characteristics, and treatment response remain unclear.
Methods and Results: Nagoya PH registry database was retrospectively analyzed. Of 129 CTEPH patients, 82 were included. Clinical characteristics and treatment response were compared between CTEPH patients with MPN (MPN group) and those without MPN (non-MPN group). As the treatment course at Nagoya University Hospital, medical treatment was initiated before performing balloon pulmonary angioplasty (BPA)/pulmonary endarterectomy (PEA). Right heart catheterization was performed at pre-medication, post-medication, and post-BPA/PEA. Seven patients had concomitant MPN as the underlying cause for CTEPH, accounting for 5.4% (7/129) of CTEPH cases. Baseline clinical characteristics were comparable between the MPN and non-MPN groups. No significant difference was observed in ∆pulmonary vascular resistance (PVR) from baseline to post-medical treatment (−3.59 WU in the MPN group vs. −3.80 WU in the non-MPN group; P=0.67) and baseline to post-BPA/PEA (−6.92 WU in the MPN group vs. −7.60 WU in the non-MPN group; P=0.35).
Conclusions: As hemodynamic responses to treatment in CTEPH patients with MPN were comparable with those without MPN, their prognosis may improve with multimodal CTEPH treatment similar to non-MPN cases.
