Robotic coronary artery bypass surgery in a patient with haemophilia A

摘要

Improvements in haemophilia comprehensive care, including access to safe recombinant coagulation factor concentrates (CFCs), has resulted in a prolonged life expectancy for persons with haemophilia (PWH). PWH can therefore expect to experience age-related comorbidities including cardiovascular disease 1, 2. Once thought to be resistant to the effects of atherosclerosis, their normalizing lifespan puts them at similar risk as the general population 2. Management of cardiovascular disease in PWH presents a number of challenges including choice of revascularization technique (surgical vs. percutaneous coronary intervention), appropriate peri-procedure CFC prophylaxis, as well as the safety of antiplatelet and anticoagulant drugs. Cardiac surgery involving sternotomy and cardiopulmonary bypass carries significant risk 3, 4, and the existing literature in PWH is limited to case reports and case series 3-6. We sought to mitigate bleeding risk in a PWH requiring coronary artery bypass grafting (CABG) by utilizing a robotic, off-pump, minimally invasive approach. A 48-year-old man with moderate haemophilia A (factor VIII 4%), complicated by contraction of human immunodeficiency virus and hepatitis C virus from previous blood transfusions, developed Canadian Cardiovascular Society class III angina. He had no other risk factors for cardiovascular disease other than a family history of early-onset coronary artery disease. He underwent exercise stress testing which revealed ST changes and reproduction of symptoms at low workload. He was therefore referred for diagnostic coronary angiography. This revealed a complex bifurcation lesion (Medina classification 1,1,1) involving the left anterior descending (LAD) coronary artery and a large, branching first diagonal branch (Fig. 1). Other findings included well-preserved left ventricular function with an ejection fraction of 55% and a dominant right coronary artery with an ostial lesion of 20–25%. Given the lesion characteristics, percutaneous coronary intervention (PCI) would have required bifurcation stenting of the lesion using drug-eluting stents that would have then necessitated prolonged dual antiplatelet therapy. A thorough discussion between the cardiac surgeon, cardiologist, anaesthesiologist and haemophilia treatment centre haematologist yielded a plan for a minimally invasive robotic approach. The patient was administered a bolus dose of 50 IU kg−1 of recombinant FVIII concentrate [Kogenate FS (Bayer)] 12 h prior to the procedure. At 10 min postbolus dose the patients FVIII level was 1.99 U mL−1 (TOP500; Instrumentation Laboratories, Bedford, MA). A continuous infusion was then started through a dedicated line using an infusion pump (AS50 Syringe Pump; Baxter, Deerfield, IL) at a rate of 4 IU kg−1 h−1 and was continued throughout the surgery. Factor VIII level 10 min prior to surgical incision was 1.71 U mL−1. In a 30° right lateral decubitus position the lung was deflated and a 12-mm port was inserted in the left fifth intercostal space anterior to the anterior axillary line, and the intrathoracic cavity was insufflated with carbon dioxide. Endoscopic visualization-assisted creation of two 7-mm ports followed, in the third intercostal space and seventh intercostal space, anterior to the anterior axillary line. The ports were adapted to the da Vinci robotic system (Intuitive Surgical, Sunnyvale, CA) with which the left internal thoracic artery (LITA) was harvested from the first to sixth rib. After systemic heparinization at 300 IU kg−1 and clipping of the distal aspect of the LITA, the pericardium was opened and the LAD was visualized; the third intercostal space was marked to provide best access to the LAD. The robot instruments were removed, and in the third intercostal space a small non-rib-spreading incision was made, with soft tissue retractor applied. The Octopus Nuvo stabilizer (Medtronic, Minneapolis, MN) was inserted through the third intercostal space to stabilize the LAD,