Résumé
The incidence of PAH in CMPD is unknown. When present, PAH in CMPD may be attributed to thrombo–embolism, which is a known complication of CMPD. CMPD and unexplained PAH has been reported in few case reports or few studies with limited cases. Pathophysiology may be related to pulmonary capillary obstruction from increased cellular components, platelet activation and aggregation, micro thrombose formation and stasis. We present the case of a middle aged lady with no previous co–morbidity, who presented with polycythaemia and biventricular failure. Evaluation revealed PAH with no evidence of pulmonary thrombo–embolism. Haematocrit, Serum erythropoietin assay and bone marrow analysis suggested polycythaemia vera. Unexplained PAH in CMPD is discussed.