Marfan syndrome : Cardiovascular features

Marfan syndrome: Cardiovascular features

Features

1) Excessive aortic root dilation ( > 1.7mm per year)

2) Aortic aneurysm

3) Aortic dissection. Features that are suggestive of a higher risk of dissection include:

- Dilatation at the aortic sinotubular junction

- Aortic root diameter >55 mm

- Excessive aortic root dilatation (>1.7 mm/year)

4) Aortic valve regurgitation

- 100% if the aortic root meaures more than 6mm

5) Mitral valve prolapse and regurgitation secondary to myxomatous

6) Tricuspid valve regurgitation can also occur but is less likely than aortic valve regurgitation

Prophylactic surgery at:

- Sinus diameter of 55 mm in adulthood or 45–50 mm in childhood

- Aneurysm expansion of > 0.5–1 mm per year

References

1. Weerakkody Y, Sharma R, Iqbal S, et al. Ma...