Poland syndrome: A contraindication to the use of the internal thoracic artery in coronary artery bypass grafting?

Abstract

Poland syndrome (PS) is a rare congenital disorder that is present in 1:30,000 people. It is primarily characterized by hypoplasia of the pectoralis muscles, with associated ipsilateral syndactyly of the upper extremity. Although the true cause of PS remains unknown, the prevailing theory attributes these sequelae to hypoplasia of the subclavian artery or its branches as the result of an in utero vascular accident. 1,2 Contemplating how to perform coronary artery bypass grafting (CABG) in a patient with left-sided PS is therefore of particular interest, because hypoplasia of the subclavian artery would theoretically render the left internal thoracic artery (LITA) inadequate for use. Consistent with yet exacerbating this clinical conundrum, no cases of CABG in patients with PS have previously been reported. We recently encountered a patient with undiagnosed left-sided PS with significant coronary artery disease involving the left anterior descending coronary artery. Our preoperative workup included diagnostic imaging of the heart and great vessels to assess the adequacy of the LITA as a bypass conduit. Surprisingly, our evaluation demonstrated normal left subclavian artery and LITA anatomy, thus calling into question the currently accepted theory for this unusual syndrome. Clinical Summary A 70-year-old man was seen with anginal symptoms that had worsened during the previous 2 weeks. Physical examination revealed syndactyly of the left hand and hypoplasia of the left breast (Figure 1). Cardiac catheterization demonstrated three-vessel coronary artery disease, including a severely occluded left anterior descending coronary artery. After surgical consultation, the presence of the aforementioned phenotype was noted, and select subclavian artery catheterization was therefore performed. Interestingly, a widely patent subclavian artery and LITA were seen. Because of the possibility of chest wall deformity that might alter our sternotomy and closure, a computed tomographic angiographic study was also performed (Figure 2). This scan confirmed the absence of pectoralis musculature and demonstrated a normal bony thoracic cage. Moreover, normal caliber subclavian artery and LITA were confirmed. Intraoperative assessment of the LITA also revealed a normal artery with adequate flow by visual inspection. Four-vessel CABG was therefore performed, which included a LITA conduit to the left anterior descending coronary artery. The remainder of the operative and postoperative course was unremarkable.