Ann Thorac Surg 2004;78:691-693
© 2004 The Society of Thoracic Surgeons
Case report
Long-term left internal mammary artery graft patency for coronary artery disease associated with pseudoxanthoma elasticum
Howard K. Song, MD, PhDa,
Erez Sharoni, MDa,
Byron Williams, Jr, MDa,
Robert A. Guyton, MDa,
John D. Puskas, MDa*
a Section of Cardiothoracic Surgery and Division of Cardiology, Emory University, Atlanta, Georgia, USA
Accepted for publication August 19, 2003.
* Address reprint requests to Dr Puskas, Carlyle Fraser Heart Center, 6th Floor, Cardiothoracic Surgery, Crawford Long Hospital of Emory University, 550 Peachtree St, NE, Atlanta, GA, USA 30308
e-mail: john_puskas{at}emoryhealthcare.org
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Abstract
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Pseudoxanthoma elasticum (PXE) is a rare, inherited connective tissue disorder with numerous systemic manifestations that include premature coronary artery disease. Coronary artery bypass grafting (CABG) is known to be beneficial in patients with PXE-related coronary artery disease. In these patients, however, the suitability of arterial conduits, including the internal mammary artery, has been controversial. We present a patient with PXE-related coronary artery disease who has had long-term patency of a left internal mammary artery (LIMA) graft after an off-pump CABG procedure in which LIMA and bilateral radial artery conduits were used.
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Introduction
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Pseudoxanthoma elasticum (PXE) is a rare, inherited connective tissue disorder associated with numerous manifestations affecting the ocular (angioid streaks and retinal choroiditis), dermal (yellow xanthoma-like papules), and cardiovascular systems [1]. The prevalence of this disorder is approximately 1/100,000 people with a female preponderance of 2:1. The cardiovascular manifestations of PXE include premature coronary artery disease, calcific vascular lesions, and endocardial fibrosis [2]. Although coronary artery bypass grafting (CABG) for patients with PXE-related coronary artery disease has been reported previously, the infrequency of these cases has precluded any standardization of the surgical management of these patients [2–8]. In particular, the selection of suitable conduits for these patients with a systemic illness affecting medium-sized arteries has been controversial [2, 7, 8]. We present a patient with longstanding PXE and coronary artery disease who has had long-term patency of a left internal mammary artery (LIMA) graft after off-pump CABG in which LIMA and bilateral radial artery conduits were used.
The patient was a frail, 67-year-old woman with a past medical history significant for PXE, subretinal hemorrhage, coronary artery disease, recent myocardial infarction, and severe peripheral vascular disease that required multiple previous lower-extremity revascularization procedures. Cardiac catheterization demonstrated severe three-vessel coronary artery disease and moderately reduced left ventricular function. Because of the severity of the disease and the patient's ongoing unstable angina, CABG was recommended and the patient agreed to proceed.
The patient had no remaining saphenous vein conduit in either leg as a result of the previous multiple lower-extremity revascularization procedures. Allen's test examinations of both arms revealed intact palmar arches and good ulnar arterial inflow bilaterally. When the chest was opened, the sternum was noted to be very thin and narrow. The LIMA was harvested as a pedicle and prepared with papaverine. It was determined that bilateral mammary harvesting was contraindicated due to the fragility of the sternum and concern about its healing following devascularization. Bilateral radial artery harvesting was performed, and the radial artery conduits were skeletonized and inspected closely. Although both conduits had intermittent calcification throughout their lengths, they were widely patent. The LIMA graft was also patent with good flow. Lacking available saphenous vein or right internal mammary artery conduits, we elected to proceed with CABG by using the LIMA and both radial artery grafts.
Bypass grafting of the left anterior descending (LAD), middle obtuse marginal (MOM), and posterior descending arteries was performed off-pump with an Octopus 3 (Medtronic, Inc, Minneapolis, MN) stabilizing device and 8-0 Surgipro (United States Surgical Corp, Norwalk, CT) suture. Good back-bleeding was noted from the radial artery grafts after completion of the distal anastomoses, and Doppler examination demonstrated good biphasic signals in all of the grafts at the end of the procedure.
The patient's initial postoperative course was unremarkable and she was discharged to home on postoperative day 4. At 30-day follow-up, the patient had no angina and no wound healing or upper extremity complications. One year after her operation, atypical chest pain developed and the patient underwent cardiac catheterization. This demonstrated a widely patent LIMA graft to the LAD, but an occluded radial artery graft to the MOM artery and stenosis of the radial artery graft to the posterior descending artery (see Fig 1). The stenosed radial artery graft was stented, and the patient had relief of symptoms after this procedure.
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Fig 1. Angiogram demonstrating patency of a left internal mammary artery graft to the left anterior descending artery of a patient with pseudoxanthoma elasticum-related coronary artery disease 1 year following bypass surgery.
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Comment
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The pathologic process underlying the clinical manifestations of PXE involves the calcification and fragmentation of elastic fibers. In the cardiovascular system, this occurs mainly in the internal elastic lamina of medium-sized arteries and frequently leads to early coronary artery disease [1]. The predilection of this pathologic process for medium-sized arteries has led some authors to recommendation that the use of internal mammary artery conduits be avoided altogether during CABG [2, 4, 6, 7]. Recently, this sentiment has come under question, as the LIMA has been found to be free of stenosis in a number of patients being considered for CABG [2, 4, 8]. The effect of harvesting and anastomosis on the disease process within the LIMA has been unclear and has led to continued debate regarding the usefulness of this graft in the treatment of PXE-related coronary artery disease [8]. The use of radial artery conduits and off-pump CABG for patients with PXE have not been previously reported in the literature.
The controversy surrounding the suitability of arterial grafts for patients with PXE may be related to the wide variability in the disease's degree of penetrance and expression [1]. Indeed, while premature coronary artery disease has been reported in teenage patients with PXE, the patient presented here had no coronary symptoms until her seventh decade. Similarly, internal mammary artery involvement by PXE is not likely to be uniform across the population and therefore, these conduits may be suitable in a subset of these patients. In fact, PXE is widely thought to be underdiagnosed because of physicians' lack of familiarity with the disease, and it is therefore likely that arterial conduits have been used successfully for coronary artery bypass in a number of patients with undiagnosed PXE.
In the case presented here, saphenous vein grafts were unavailable because they had been previously used in lower-extremity revascularization procedures. After careful examination for patency, arterial grafts were used and yielded good short-term results. Over time, however, the radial artery grafts developed aggressive arteriosclerotic disease that resulted in the loss of one graft and the need for repeat revascularization in the other. The LIMA graft remains widely patent at 1-year follow-up. Our experience in this unique circumstance has led us to recommend that left or bilateral internal mammary grafts be used preferentially, after they have been carefully evaluated for patency, in patients with PXE-related coronary artery disease. Radial artery grafts do not appear to be spared by the systemic disease process and should only be used in the absence of other alternatives, including saphenous vein grafts.
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References
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Abstract
Introduction
