Table 2 Disease presentation of MYLK PV carriers

From: MYLK pathogenic variants aortic disease presentation, pregnancy risk, and characterization of pathogenic missense variants

Variable

Frequency

Median age of onset in years (IQR)

Aortic event

38% (23/60)

48 (28)

 Aortic dissection

87% (20/23)

51 (27.5)

 Type A

85% (17/20)

48 (26)

 Type B

15% (3/20)

56 (62)

 Aneurysm repair

13% (3/23)

42 (31)

Without aortic event

53% (32/60)

39.5 (36.5)

Sudden

8% (5/60)

61 (16)

  1. IQR, interquartile range.
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