Primary whose mechanisms are incompletely known, is another vascular disease entity recently described in chronic kidney disease particularly in patients undergoing haemodialysis. It corresponds to 5th subtype of World Symposium on Pulmonary Hypertension classification established in 2008 in Dana Point and updated in 2013 in Nice. Pulmonary hypertension is defined by Pulmonary arterial pressure mean ≥25 mm Hg at rest measured by right heart catheterization. Its pathophysiological mechanism is controversy maybe explained by vascular calcification, blood flow in arteriovenous fistula and fluid overload. Primary pulmonary hypertension is a major problem of haemodialysis patients because of its high prevalence, sometimes reaching 68% and by its significant morbidity and mortality.