Related Experiment Video
Updated: Jan 12, 2026

Full-root Aortic Valve Replacement by Stentless Aortic Xenografts in Patients with Small Aortic Roots
Published on: May 21, 2017
Valve Sparing Aortic Root Replacement in Loeys-Dietz Syndrome Type II: Is It Ever Too Early?
Bushray Almiqlash1, Muhammad Faateh1, Awais Ashfaq1
1The Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
Background:
Loeys-Dietz syndrome (LDS) is an autosomal-dominant connective tissue disorder that is associated with aggressive vascular disease, including predisposition to aortic root aneurysms and dissection.
Case Summary:
An 18-month-old boy was referred to our hospital with a diagnosis of LDS with TGFBR2 mutation because of concern for progressive aortic dilation. Despite antihypertensive therapy, aortic root dilation advanced significantly to 3.7 cm (z score: +19.2) at a rate of 1.2 cm/y, and an aortic annulus to 16 mm (z score: +6.5), mandating early valve-sparing aortic root replacement.
Discussion:
We demonstrate successful valve-sparing aortic root replacement in an extremely early case of an 18-month-old with LDS. Children with LDS with high-risk features may benefit from closer follow-up of aortic root growth.
Take-Home Messages:
Patients with high-risk LDS need closer follow-ups and genetic consultation to determine the association with aggressive patterns because earlier surgical intervention may become mandatory in this group.
Related Concept Videos
Aortic Regurgitation III: Medical Management
Aortic Regurgitation I: Introduction
Mitral Stenosis III: Medical Management
Aortic Regurgitation II: Clinical Features and Diagnostic Tests
Mitral Regurgitation III: Medical Management
Mitral Stenosis I: Introduction

