Primary Pulmonary Hypertension and Moyamoya Disease: Case Presentation and Review of Literature

Introduction: primary pulmonary hypertension (pph) is an uncommon disease in children. this pulmonary vascular disease may be idiopathic, familial or a kind of hereditary systemic vasculopathy. multisystemic vascular diseases in children are rare and may be underrecognized. a few reports have described the involvement of cerebral vessels such as moyamoya disease (md) in patients with pph. md is a unique cerebrovasculopathy, characterized by occlusion of the internal carotid arteries. case presentation: we reported a 2-year-old boy with pph, who presented unusual and intractable seizure which suggested the diagnosis of md. we also reviewed a few previous reports about the association of pph with moyamoya disease. discussion: moyamoya disease is a kind of cerebral vasculopathy and should be considered as a differential diagnosis for patients with unusual seizure attacks who are suffering from primary pulmonary hypertension.