Computed tomographic findings in TBX4 mutation: a common cause of severe pulmonary artery hypertension in children.

Abstract excerpt

BACKGROUND: Mutations in the T-Box 4 (TBX4) gene are a lesser-known cause of heritable pulmonary arterial hypertension (PAH). Patients with heritable PAH typically have worse outcomes when compared with patients with idiopathic PAH, yet little is known about the phenotypical presentation of this mutation. OBJECTIVE: This article reviews the pattern of chest CT findings in pediatric patients with PAH and TBX4...

Topics

Share this publication in a Topic to start or enrich a Post.

• Share to Child, Preschool
• Share to Familial Primary Pulmonary Hypertension