Cardiovascular operations for Loeys-Dietz syndrome: Intermediate-term results

Abstract

Growing experience with LDS has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe. Surgical outcomes are favorable, but reintervention rates are high. Meticulous follow-up with cardiovascular surveillance imaging remain important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed.

Loeys–Dietz syndrome: We have come a (Fur)long way

“…6 Even without indexing, there is strong evidence that LDS is a very aggressive disease and without prophylactic proximal aortic repair may lead to early mortality. 7 Considering this, is it time to recommend even a lower threshold for intervention in LDS that may be based on indexing? Because the obvious goal in managing the patients with LDS is to prevent death from rupture or dissection, it would seem intuitive to intervene sooner, but how much sooner?…”

Aortic surgery in Loeys-Dietz syndrome: Excellent results, but close surveillance is necessary

“…The types and results of these interventions are showcased in this month's issue of The Journal of Thoracic and Cardiovascular Surgery , in which Patel and colleagues 2 present their series of 79 patients with LDS who underwent cardiovascular surgical management at Johns Hopkins University. These patients were young; the mean age at first operation was 25 years.…”

“…Given the fragile nature of the aortic and valvular tissues in these patients, these results are a testament to the outstanding surgical care provided by the Johns Hopkins University team, who point out that both technical and patient factors are important considerations for obtaining the best results. In the article of Patel and colleagues, 2 it is stressed that the reimplantation technique is favored relative to the remodeling technique and is the procedure of choice to mitigate the development of late aortic insufficiency caused by annular dilatation. Accompanying this article is an excellent video describing the “Hopkins technique” for reimplantation valve-sparing aortic root replacement, which uses just 3 subannular sutures and encases the aortic valve in a Dacron polyester fabric graft with prefabricated sinuses of Valsalva.…”

“…Patel and colleagues 2 recommend echocardiography every 3 to 6 months for the first year after surgery, which is then extended to every 6 months to 1 year. Full cardiovascular imaging should be obtained preoperatively and 1 year after surgical intervention, with reimaging at least every 2 years thereafter.…”

“…To conclude, the article of Patel and colleagues 2 describes the comprehensive, multidisciplinary, aggressive approach to the surgical treatment of aortic disease in patients with LDS. Because of the fragile nature of the vascular tissues inherent in this disorder, close surveillance is necessary, and it can be expected that about a quarter of the patients followed up will require surgical reintervention.…”