.

https://www.ahajournals.org/doi/10.1161/circulationaha.111.054676

.

Discussion

The main finding of our study is the low rate of aortic events in a population diagnosed with Marfan syndrome according to the international criteria (Ghent nosology)⁹ when current recommendations are applied, ie, systematic β-blockade, advice about sports and physical activity, regular aortic measurements with echocardiography, and prophylactic aortic root surgery for an absolute aortic diameter of 50 mm.¹⁵ With the use of these rules, 7 aortic events occurred among 732 patients during a follow-up of 6.6±4.3 years, leading to an annual risk of 0.17%. This risk can be stratified according to aortic diameter, as shown in Figure 2. When only patients with aortic diameter <50 mm were considered and excluding 1 neonatal patient with Marfan syndrome, a pregnant woman with an aortic diameter of 45 mm, and a 72-year-old woman who had undergone 2 previous surgeries and had had 1 acute myocardial infarction, the annual risk was <0.05%. Preventing aortic dissection is critical because it is well established that previous aortic dissection alters survival, particularly if dissected aorta remains after surgery,¹⁶ and these short- and long-term risks are to be compared with the risk of preventive aortic surgery, which is low in experienced centers.

.

A cirurgia de Aorta no Marfan depende quase que exclusivamente de um acompanhamento rigoroso da ecocardiografia.

.

Indicar o procedimento com 50 mm está escrito como favorável mas causa hesitação em muitos clínicos.

.

Eles precisa confiar no ecocardiografista para decidir.

.

The Root of the Problem

https://www.ahajournals.org/doi/full/10.1161/CIRCIMAGING.119.008860

.

Acima um editorial muito interessante sobre o tema.

Seria a Bivalvulada uma complicação para indicar mais precocemente?

.

The timing of preventive aortic root replacement in MFS is determined by aortic diameter but is informed by other factors including aortic growth rate, family history of aortic dissection, arterial tortuosity, diffuse aortic dilatation, requirement for mitral valve surgery, pregnancy, prior type B dissection, and patient and physician wishes. BAV may coexist in MFS at a rate similar to the general population and, although this has not been associated with an increased risk for dissection, appears to relate to a larger aortic root diameter for a given age and the requirement for preventive surgery at an earlier age based on this important new contribution.

.