Spontaneous coronary artery dissection in cutis laxa

Abstract

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.