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.

Figure 1.

CT coronary angiogram demonstrating a right coronary artery dissection. 1 .a—coronal slice demonstrating the right coronary artery within the atrioventricular groove. The proximal right coronary artery is normal calibre and opacified with contrast suggesting patency (thin arrow). The patent vessel then tapers into a non-opacified dissected vessel expanded with thrombus (stars). There is distal refill of the PDA branch (thick arrow) via collaterals. 1.b—four chamber view demonstrating the right coronary artery expanded with thrombus (arrow). The left circumflex coronary artery is normal calibre and opacified with contrast suggesting patency (arrow head). Ao, aorta; LA, left atrium; LV, left ventricle.

Figure 2.

2 .a—Curved mutiplanar reconstruction of the RCA. Patent proximal RCA (star). Mid RCA is dissected and expanded with thrombosis with no intraluminal contrast (arrows). Distal vessel (PDA) is opacified from filling via collaterals (circle). 2.b—Volume rendered three dimensional reconstruction of the coronary arteries. Dissected RCA (arrow) has poor definition (due to lack of intraluminal enhancement) compared to well defined and normally opacified left sided coronary arteries (star). RCA, right coronary artery.