Session IAscending aortaEarly Surgical Experience With Loeys-Dietz: A New Syndrome of Aggressive Thoracic Aortic Aneurysm Disease
Section snippets
Patient Selection
After obtaining Institutional Review Board approval, we retrospectively reviewed the medical records of all patients with the diagnosis of LDS. Informed consent was waived because of the retrospective nature of this study. In some instances, patients were evaluated and treated before the initial description of LDS, and some initially had been referred to our institutions as patients with Marfan syndrome.
Patients from The Johns Hopkins Medical Institutions, Baltimore, Maryland, and the Ghent
Results
Clinical criteria and genotyping identified 71 patients with LDS, of whom 32 (45%) were children, and 39 (55%) were adults. Six (9%) died before surgery at our institutions from aneurysm rupture or intracerebral hemorrhage. Aortic surgery was performed in 30% of patients (21/71), and 62% (44/71) are medically managed or are awaiting surgery. Of the surgically treated patients, 62% (13/21) had a preoperative diagnosis of LDS; whereas, 38% (8/21) were diagnosed postoperatively when the genetic
Comment
The TGF-β family of cytokines consists of more than two dozen signaling molecules secreted into the extracellular matrix [6]. These molecules act through a complex signaling pathway on multiple cell types to effect growth and development of normal cells [7, 8]. Actions of TGF-β molecules lead to cellular differentiation, proliferation, motility, organization, and death [6]. These cytokines have been specifically implicated in cardiovascular development and function [9]. TGF-β molecules guide
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