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Interact CardioVasc Thorac Surg 2007;6:403-404. doi:10.1510/icvts.2006.141994 © 2007 European Association of Cardio-Thoracic Surgery
Patients with Klippel-Feil syndrome should have imaging of the subclavian artery and its branches prior to coronary artery bypass graftingDepartment of Cardiac Surgery, Royal Victoria Hospital, Grosvenor Road, Belfast BT12 6BA, Northern Ireland, UK Received 17 August 2006; received in revised form 22 January 2007; accepted 23 January 2007
*Corresponding author. Tel.: +44 28 9024 0503.
Patients with Klippel-Feil syndrome have increased incidence of subclavian artery anomalies. We report a case of a patient with this syndrome undergoing coronary artery bypass grafting. Intra-operatively, the patient was found to have the left internal mammary artery arising from the second intercostal space and the decision was taken to use it as a free graft. As other anomalies of the subclavian arteries and their branches are recognised in patients with Klippel-Feil syndrome, we propose that imaging of the internal mammary arteries is carried out pre-operatively in such patients to assess the suitability for use as conduit in coronary artery bypass grafting.
Key Words: Internal mammary artery; Coronary disease; Klippel-Feil syndrome
Anatomical variations are not infrequently encountered in all aspects of surgery. In the majority of occasions they are not of any great significance and are merely only of interest in their variety. Klippel-Feil syndrome is defined as congenital fusion of two or more cervical vertebrae and is believed to result from faulty segmentation along the embryo's developing axis during weeks 3–8 of gestation. The classic clinical triad of short neck, low hair line, and restricted neck motion is present in less than 50% of patients with this syndrome. Three types of Klippel-Feil syndrome have been described [1].
Other systemic findings include, renal agenesis, different types of hearing loss, cardiac pathologies, epilepsy [2], and other organ system anomalies [3]. Normally, the left internal mammary artery (LIMA) originates as a branch of the first part of the left subclavian artery, passing deep to the costal cartilages just lateral to the manubrium and sternum. It supplies branches to the intercostals' spaces and terminates by dividing into the musculophrenic and superior epigastric arteries. The standard fashion for harvesting the LIMA is to disconnect it at its terminal bifurcation and mobilise it as a pedicle, dividing all the intercostals' branches to avoid the risk of steal phenomenon. We report an unexpected and significant finding of anomalous origin of the left internal mammary artery (LIMA) in a patient with Klippel-Feil syndrome undergoing coronary artery bypass grafting (CABG).
A 62-year-old gentleman with Klippel-Feil syndrome was referred by the cardiologists for consideration of CABG to treat his increasing angina pectoris (Canadian Cardiac Score II) despite maximal medical therapy. The patient was offered CABG for symptomatic relief with a pre-operative plan for a pedicle LIMA to the left anterior descending artery and long saphenous vein graft from ascending aorta to obtuse marginal branch of the circumflex artery. At operation, the LIMA was harvested and was found to have an aberrant origin from the 2nd intercostal space on the left (Fig. 1). The decision was taken to harvest it as a free graft as the proximal course was unknown. The free LIMA was grafted to the left anterior descending artery (LAD) and long saphenous vein was grafted to the obtuse marginal branch of the circumflex. The operation was completed in a standard fashion without complication and his recovery was uneventful. He was discharged home on the eighth post-operative day.
In this case, the LIMA was found to be originating from the 2nd intercostal space. The anatomy of the vessel, proximal to this, was unclear. The decision was taken to use it as a free graft as opposed to a pedicle as there was potential for a steal phenomenon to occur. This patient had Klippel-Feil syndrome, a rare congenital disorder characterised by congenital fusion of two or more cervical vertebrae and may be associated with other organ system anomalies [3]. There are no reports in the literature about coronary artery bypass grafting in such patients. There are some case reports highlighting the presence of subclavian artery anomalies in patients with Klippel-Feil syndrome [4, 5]. Bavinck and Weaver use the term subclavian artery supply disruption sequence (SASDS) and hypothesise that this is a common pathogenesis for a number of recognised birth defects. In a disruption sequence, a developmentally normal embryo or fetus experiences a destructive process, in this case partial or complete blockage of blood flow in the subclavian artery, with cascading consequences [5]. The exact nature of the defect will depend upon the exact site and timing of the disruption sequence. A number of similar defects have been described of which Klippel-Feil syndrome is well recognised. This theory relies in the subclavian artery and its branches develop by the process of sprouting angiogenesis. More recent research in the zebrafish animal model suggests the vasculature in the trunk develops by the process termed vasculogenesis [6]. This is the process of blood vessel formation occurring by a de novo production of endothelial cells. Coalescence of these segmental angioblasts results in the formation of the trunk vessels. The left internal mammary artery could be seen as a vessel not ‘sprouting’ from the subclavian artery and giving intercostal branches, rather as a vessel segmentally fused to the subclavian artery. Abnormalities of vertebral fusion/segmentation may be linked to abnormal segmentation of the trunk vasculature thus explaining the phenotype seen in our patient. Evidence for faulty segmentation rather than fusion of the vertebrae is provided in a genetic study of Klippel-Feil syndrome families by Clarke et al. [7]. For these reasons, we propose that any patient with Klippel-Feil syndrome for coronary artery bypass grafting, where the use of the internal mammary artery is being considered for conduit, should have imaging of the subclavian and internal mammary arteries pre-operatively as there is likely to be an associated anomaly. This will allow better planning of the operation in terms of conduit choice.
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Abstract
1. Introduction
