Intracoronary ultrasound guided percutaneous coronary angioplasty using a drug eluting stent in a patient with pseudoxanthoma elasticum

Successful revascularization of advanced coronary artery disease associated with pseudoxanthoma elasticum

“…Consistent with those reports, the findings of IVUS were a high echo component without acoustic shadowing around vessels that reflect a fragmented elastic fiber with calcification response to coronary arteries [5]. In addition to those findings reported in the two previous reports of similar cases [4,5], we found several coronary stenotic lesion morphologies such as plaque rich and shrinkage lesions by observing throughout length of the RCA and LAD. Each stenotic lesion morphology also had high echo components without acoustic shadowing around the vessels, therefore fragmented elastic fiber with calcification might affect the progression of stenosis.…”

“…Spatial resolution of coronary CT might not be enough to detect coronary calcification because calcific degeneration of the vascular internal elastic membrane in PXE leads to microcalcification that is much smaller than that of arteriosclerosis. To our knowledge, this is only the third reported case with coronary IVUS in PXE [4,5]. Consistent with those reports, the findings of IVUS were a high echo component without acoustic shadowing around vessels that reflect a fragmented elastic fiber with calcification response to coronary arteries [5].…”

“…Each stenotic lesion morphology also had high echo components without acoustic shadowing around the vessels, therefore fragmented elastic fiber with calcification might affect the progression of stenosis. In previous studies on coronary lesions in PXE patients, only coronary stenosis with rich plaque and high echoic change around vessel were reported [4,5]. Here, however, our patient had two vessel diseases, and IVUS revealed other coronary stenotic morphology such as rich plaque lesions and negative remodeling lesions.…”

“…Most CAD patients with PXE have been treated by aortocoronary bypass, but this patient refused bypass graft surgery and strongly requested to be treated with PCI, and we finally decided to perform PCI. We know of only three cases that were treated by PCI [3][4][5]. In one of these cases, a heparin-coated balloon was directly deployed at optimal pressure after IVUS observation, and the final angiography showed adequate expansion [4].…”

Stiff Coronary Stenosis in a Young Female With Pseudoxanthoma Elasticum

“…patient with PXE (2). In that report, the stenosis was successfully treated by the conventional techniques.…”