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Article on Cardiac Surgery for Loeys-Dietz Patients |
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Written by Administrator
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Wednesday, 25 April 2007 |
A new article from the Society of Thoracic Surgeons is now available for download by clicking
lds-syndrome.net/download_center_lite/index.php?CVSsurgery-1.pdf. An abstract follows:
Background. Loeys-Dietz syndrome (LDS) is a recently
described genetic aortic aneurysm syndrome resulting
from mutations in receptors for the cytokine transforming
growth factor-. Phenotypic features include a bifid
uvula, hypertelorism, cleft palate, and generalized arterial
tortuosity, but risk of thoracic aortic rupture and
dissection is the principle focus of management and
exceeds that of most known connective tissue disorders.
Our surgical experience with LDS was reviewed to assess
outcomes and develop guidelines for management of this
aggressive disease.
Methods. We retrospectively reviewed medical records
of all LDS patients from two institutions and obtained
follow-up data from medical records and patient
contacts.
Results. Clinical criteria and genotyping were used to
identify 71 patients. Before surgical intervention, 6 patients
(9%) died from aneurysm rupture or dissection,
which occurred in several patients with aortic diameters
of less than 4.5 cm and as early as 6 months of age.
Thoracic aortic aneurysm surgery was performed in 14
children and 7 adults. Operations included valve-sparing
root replacement (VSRR) in 13, Bentall procedure in 5,
arch replacement in 2, and VSRR with arch replacement
in 1. There were no deaths at the primary operation,
although 3 patients died 2, 5, and 11 years after surgery
from rupture of the descending thoracic (n 2) or
abdominal aorta (n 1).
Conclusions. LDS is an aggressive aortic aneurysm
disease with a propensity toward rupture and dissection
at a younger age and smaller aortic diameters than in
other connective tissue disorders, particularly in the
ascending aorta. Early recognition of the phenotype,
prophylactic intervention, and meticulous surveillance of
the distal aorta and vascular tree are warranted for
optimal management.
(Ann Thorac Surg 2007;83:S757– 63)
© 2007 by The Society of Thoracic Surgeons |
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Last Updated ( Friday, 27 April 2007 )
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