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

Abstract

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 under-recognized. 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.