Charters Pia Frances Pemberton, McKenzie Daniel Barnaby, Rodrigues Jonathan Carl Luis, Loughborough William Wynn
Royal United Hospitals Bath NHS Foundation Trust, Bath, UK.
BJR Case Rep. 2022 Nov 1;8(6):20210248. doi: 10.1259/bjrcr.20210248.
We describe the case of a 21-year-old female with Cutis Laxa presenting with an acute coronary syndrome. A CT coronary angiogram (CTCA) diagnosed spontaneous coronary artery dissection (SCAD) of the right coronary artery, which was successfully managed with medical therapy. Cutis Laxa is a rare connective-tissue disorder in which the skin becomes inelastic. Lax, redundant skin hangs in folds give a prematurely aged appearance and several forms of the disease have been described. Although certain cardiovascular complications are recognised in Cutis Laxa, to our knowledge SCAD has not been previously described. SCAD is an uncommon cause of acute coronary syndrome and sudden cardiac death. The condition particularly affects young females, those with connective tissue diseases, arteriopathies, pregnant females, contraceptive use and cocaine use. Atherosclerotic risk factors are seldom reported. The condition is underdiagnosed as symptoms may not generate a high index of clinical suspicion in this demographic. Diagnosis is traditionally made on invasive coronary angiogram although the procedure carries risks in SCAD and non-invasive CTCA should be considered in appropriately selected patient cohorts or as an adjunctive measure to assess for extracoronary vascular abnormalities. Our patient was diagnosed on CTCA, avoiding the need for invasive catheter angiogram.
我们描述了一名患有皮肤松弛症的21岁女性出现急性冠状动脉综合征的病例。CT冠状动脉造影（CTCA）诊断为右冠状动脉自发性冠状动脉夹层（SCAD），经药物治疗成功处理。皮肤松弛症是一种罕见的结缔组织疾病，其皮肤会变得缺乏弹性。松弛、多余的皮肤形成褶皱，呈现出早衰的外观，并且已描述了该疾病的几种形式。虽然在皮肤松弛症中某些心血管并发症是公认的，但据我们所知，此前尚未描述过SCAD。SCAD是急性冠状动脉综合征和心源性猝死的罕见原因。这种情况特别影响年轻女性、患有结缔组织疾病的人、动脉病变患者、孕妇、使用避孕药者和使用可卡因者。很少报告有动脉粥样硬化危险因素。由于在这一人群中症状可能不会引起高度的临床怀疑，所以该病诊断不足。传统上通过有创冠状动脉造影进行诊断，尽管该操作在SCAD中有风险，对于适当选择的患者队列应考虑使用无创CTCA，或作为评估冠状动脉外血管异常的辅助措施。我们的患者通过CTCA确诊，避免了进行有创导管血管造影的需要。
