Vascular Involvements Are Common in the Branch Arteries of the Abdominal Aorta Rather Than in the Aorta in Vascular Ehlers-Danlos Syndrome.

BACKGROUND

Vascular Ehlers-Danlos syndrome (vEDS) is a rare disorder with poor prognosis, owing to associated vascular complications. However, the most prevalent arterial problems in patients with vEDS are not well known.

METHODS

We retrospectively examined 20 consecutive patients diagnosed with vEDS and examined their clinical events, image findings, and therapies.

RESULTS

The age at first complication requiring admission was 29 ± 13 years. The observational period was 67 ± 30 months. Of the 20 patients, 17 took celiprolol at final assessment. At the final follow-up, the total number of complications relating to lesions and requiring admission was 16 for pulmonary lesions (8 patients), 16 for bowel lesions (8 patients), 5 for tendon/ligament lesions (2 patients), 18 for the branch arteries of the abdominal aorta (10 patients), 2 for the aorta (2 patients), and 7 for other arteries (6 patients). Of 54 arterial involvements (aneurysms, dissections, and ruptures), both with and without symptoms, 43 (80%) were in branches of the abdominal aorta (celiac artery and branches, 8; superior mesenteric artery, 4; renal arteries, 3; iliac arteries and branches, 28), 2 (4%) were in the aorta, and 9 were in other arteries. The diameter of the sinus of Valsalva was 29 ± 5 mm, within the normal range. During follow-up, 3 patients died due to suspected ruptures in a branch of the celiac artery, the superior mesenteric artery, and the aorta.

CONCLUSION

Our findings indicate that lesions involving the branch arteries of the abdominal aorta, rather than aorta, were the most prevalent lesion type in patients with vEDS.