[Pulmonary Hypertension and Polycythemia vera]

Chronic myeloproliferative diseases are rare causes of PH class 5 according to Nice classification 2018. The present case reports show different courses, on the one hand with a primary manifestation of a PH and subsequently a PV, on the other hand with the development of a PH in the context of a PV.

1) At first contact, a 75-year-old female patient who complained progressive dyspnea and had evidence of stress-PH in the right heart catheter. During the course she developed a resting PH of up to 70 mmHg systolic despite initial monotherapy and subsequent dual therapy for PH. After 5 years she had the diagnosis of polycythemia vera, treated with hydroxycarbamide and subsequent phlebotomies. In the further course increasing cardiac decompensation and death. 2) 74-year-old female patient at the time of diagnosis of chronic megakaryocytic-granulocytic myelosis. After 7 years, evidence of polycythemia vera (V617F mutation in the JAK2 gene), a monoclonal gammopathy. In the case of splenomegaly, irradiation of the spleen was carried out and, after 1 year, therapy with ruxolitinib was started. After another 2 years, with increasing dyspnea, pulmonary hypertension (CTEPH) with a PA-mean of 43 mmHg and a PVR of 4.5 WE were detected. With anticoagulation and riociguat therapy exercise capacity and PA pressures were only temporarily improved. Within 1 year restrictive ventilation, hypoxemia, heart failure (EF 45 %) with leading right heart decompensation and cardiorenal syndrome developed. Dialysis showed only short-term recompensation, and the patient died.

The case reports are characterized by a combination of PV and PH, with different temporal sequence, as well as only a low influence of PH-specific therapy, with subsequent progressive cardiac decompensation. Thus, they reflect the different etiologies, clinical manifestations, and the low therapeutic influence of PH in myeloproliferative disorders. The value of PH-specific therapy remains unclear, especially in view of different pathomechanisms in the genesis of PH.

Patients with myeloproliferative diseases require screening for PH. In the course of PH, myeloproliferative disease can unmask or develop. The therapeutic influence on PH is limited.