Akutsu Koichi, Watanabe Atsushi, Yamada Takeshi, Sahara Tomoko, Hiraoka Sayuri, Shimizu Wataru
Department of Cardiovascular Medicine, Nippon Medical School, Tokyo, Japan.
Division of Clinical Genetics, Nippon Medical School Hospital, Tokyo, Japan.
CJC Open. 2022 Nov 5;5(1):72-76. doi: 10.1016/j.cjco.2022.11.001. eCollection 2023 Jan.
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.
We retrospectively examined 20 consecutive patients diagnosed with vEDS and examined their clinical events, image findings, and therapies.
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.
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.
血管型埃勒斯-当洛综合征（vEDS）是一种罕见疾病，因其相关的血管并发症，预后较差。然而，vEDS患者中最常见的动脉问题尚不清楚。
我们回顾性研究了连续20例诊断为vEDS的患者，检查了他们的临床事件、影像表现和治疗情况。
首次因并发症需住院治疗的年龄为29±13岁。观察期为67±30个月。20例患者中，17例在最终评估时服用了塞利洛尔。在最后一次随访时，与病变相关且需住院治疗的并发症总数为：肺部病变16例（8例患者），肠道病变16例（8例患者），肌腱/韧带病变5例（2例患者），腹主动脉分支动脉病变18例（10例患者），主动脉病变2例（2例患者），其他动脉病变7例（6例患者）。在54例有症状和无症状的动脉受累（动脉瘤、夹层和破裂）中，43例（80%）位于腹主动脉分支（腹腔干及其分支8例；肠系膜上动脉4例；肾动脉3例；髂动脉及其分支28例），2例（4%）位于主动脉，9例位于其他动脉。主动脉瓣窦直径为29±5mm，在正常范围内。随访期间，3例患者因怀疑腹腔干分支、肠系膜上动脉和主动脉破裂死亡。
我们的研究结果表明，vEDS患者中最常见的病变类型是腹主动脉分支动脉病变，而非主动脉病变。
