Not seen in primary pulmonary hypeension-

Primary pulmonary hypeension (PPH) is a rare but impoant disease that affects young people, predominantly women, aged between 20 and 30 years. Familial disease is rarer still, but is known to be associated with mutations in the gene encoding type II bone morphogenetic protein receptor (BMPR2), a member of the TGF-bsuperfamily. Mutations in this gene have been identified in some patients with sporadic pulmonary hypeension. Pathological features include hyperophy of both the media and intima of the vessel wall, and a clonal expansion of endothelial cells which take the appearance of plexiform lesions. There is marked narrowing of the vessel lumen and this together with the frequently observed in situ thrombosis leads to an increase in pulmonary vascular resistance and pulmonary hypeension.pulmonary ejection click,single s2,as well as left parasternal heave are the other associated findings. ref:davidson&;s principles and practice of medicine,ed 21,pg no 550