Related Experiment Video
Updated: Sep 21, 2025

Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
Published on: November 18, 2018
Pulmonary Vein Stenosis Associated with Germline PIK3CA Mutation
Delphine Yung1,2, Kaitlyn Freeman1, Ghayda Mirzaa2,3,4
1Department of Pediatrics, Division of Cardiology, University of Washington School of Medicine, Seattle, WA 98195, USA.
Abstract:
Pulmonary vein stenosis is a rare and frequently lethal childhood disease. There are few known genetic associations, and the pathophysiology is not well known. Current treatments include surgery, interventional cardiac catheterization, and more recently, medications targeting cell proliferation, which are not uniformly effective. We present a patient with PVS and a PIK3CA mutation, who demonstrated a good response to the targeted inhibitor, alpelisib.
Related Concept Videos
Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
Pulmonary Embolism I: Introduction
Mitral Stenosis I: Introduction
Mitral Stenosis II: Clinical features and Diagnostic Tests
PI3K/mTOR/AKT Signaling Pathway
Mitral Valve Prolapse III: Nursing Management

