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Loeys-Dietz Syndrome: Medical and Surgical Management Outcomes
Study Questions:
What are the intermediate-term outcomes after medical and surgical management of patients with Loeys-Dietz syndrome (LDS)?
Methods:
This is a retrospective review of patients with genetically confirmed LDS who were treated at a single institution before April 2017. Data analyzed included family history of LDS, specific gene mutation, maximum aortic diameter, surgical indications and operative details, and postoperative outcomes, with primary endpoints of in-hospital morbidity and mortality and secondary endpoints of aorta-related re-operations and long-term mortality.
Results:
Over a nearly 10-year period, 53 patients (mean age 39 ± 14 years) with LDS aortopathy were treated medically (n = 20) or surgically (n = 33). In the aortic surgery group, valve-sparing root re-implantation was performed in 19 (58%) patients, all in a prophylactic setting; 11 (33%) had multiple aortic operations (on average 3.3 operations per patient over the study period); and there were three deaths (two perioperative deaths from complications of stroke, one late death from aortic rupture). Ten-year survival was 89% (median follow-up 5.2 years). In the nonaortic surgery group, there were no deaths (median follow-up 8.4 years). Follow-up was 100% complete.
Conclusions:
In this relatively young group of patients, aortic surgery and re-intervention were associated with excellent intermediate-term morbidity and mortality.
Perspective:
This is a large series of adult patients with LDS that provides a very detailed report of their surgical indication, index and subsequent aortic operations, and outcomes. The high prevalence of re-intervention, especially if the patient presented with or had a history of DeBakey Type I aortic dissection, supports the importance of persistent and life-long follow-up in order to monitor for aneurysmal degeneration, recurrent aortic dissection, and complications following previous aortic interventions. LDS is an aggressive aortopathy identified relatively recently, and further study is needed to determine how the different aortic phenotypes of LDS behave, the age and diameter at which prophylactic surgery should be performed, and what the extent of the initial operation should be.
Clinical Topics: Cardiac Surgery, Cardiovascular Care Team, Invasive Cardiovascular Angiography and Intervention, Vascular Medicine, Aortic Surgery, Interventions and Vascular Medicine
Keywords: Aneurysm, Dissecting, Aorta, Aortic Rupture, Cardiac Surgical Procedures, Hospital Mortality, Loeys-Dietz Syndrome, Mutation, Outcome Assessment, Health Care, Phenotype, Stroke, Thrombolytic Therapy, Vascular Diseases
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