Robotic coronary artery bypass surgery in a patient with haemophilia A

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, after which the LITA was anastomosed. Flow through the artery was measured with the Medistim (Medtronic, Minneapolis, MN) at 23 mL min−1. The heparin was reversed with protamine sulphate at a 1:1 dose based on the amount of heparin given. Two chest tubes were placed in the left pleural cavity, and the left lung was reinflated. Intraoperative cardiac catheterization in the hybrid operating theatre demonstrated a patent LITA anastomosis (Fig. 2). The minimally invasive robotic approach avoided the use of sternotomy and cardiopulmonary bypass. The continuous infusion of FVIII was maintained for a total of 3 days with a FVIII level ranging from 1.05 to 2.11 U mL−1. On postoperative day 3, the patient's continuous infusion was stopped and FVIII dosing was changed to intermittent dosing at 25 IU kg−1 every 12 h for 3 days followed by 25 IU kg−1 every 24 h for 6 days. The trough FVIII level during intermittent dosing was 0.41 U mL−1. There were no bleeding complications associated with the procedure or in the postoperative period and the patient did not require transfusion with any blood products. The patient only required a 5 day total length of stay in hospital. Postoperatively he was started on aspirin 81 mg daily and has continued on this medication with no bleeding complications at 5-months follow-up. The patient is currently free of angina and has only mild dyspnoea with vigorous exertion. A repeat FVIII level at 3-months follow-up was at the patients baseline 0.05 U mL−1 with no evidence of inhibitor development. Coronary artery revascularization for patients with clotting factor deficiencies is becoming increasingly common 3, 4, 6. The diagnosis of haemophilia should not postpone invasive diagnostic or therapeutic procedures, which would benefit such patients 6. Successful conventional on-pump CABG for such patients has been reported 3-6 as well as one case report of an off-pump procedure using a standard sternotomy approach 7. We describe, to our knowledge, the first robotically assisted minimally invasive CABG in a PWH. The robotic approach causes less chest wall trauma during ITA harvesting, and is considered a feasible treatment option, with an acceptable learning curve 8. A 2008 prospective observational study comparing open off-pump CABG and minimally invasive off-pump CABG, found that minimally invasive CABG decreased intubation time, hospital stay, minimized major adverse cardiac and cerebrovascular event risk at 1 year, and, most relevant to our case, reduced intraoperative blood loss (mean 547 mL vs. 1230 mL), transfusion requirements (mean 0.16 U vs. 1.37 U) and improved postoperative clot strength (1% decline vs. 10%) 9. In addition, minimally invasive CABG has other advantages such as reduced rates of wound infection, avoidance of a median sternotomy and reduced postoperative pain. A further advantage is the ability to complete multi-vessel arterial revascularization utilizing bilateral ITA during a MID-CABG procedure 9. Mackinlay et al. stress the importance of a multi-disciplinary team approach, requiring collaboration of haematologists, cardiologists, anaesthesiologists and cardiac surgeons, and our case has underlined this. Our team considered multiple revascularization strategies for this patient including a standard CABG approach as well as a PCI approach. The use of PCI was considered unappealing as coronary stent insertion would necessitate postprocedure dual antiplatelet therapy, and this could be complicated by increased bleeding complications in a patient with an underlying bleeding disorder. Continuous infusion (CI) therapy is complicated, labour intensive and requires a great deal of planning and coordination especially as our centre usually uses bolus dose replacement rather than CI. We felt it necessary to maintain stable factor levels through the procedure and postoperatively without the peaks and troughs associated with bolus dosing in an attempt to avoid bleeding or thrombotic complications. The bleeding disorder program nurse practitioner had a vital role in setting up the CI, training the cardiovascular surgery nurses on its use, arranging proper FVIII level monitoring and coordinating the treatment protocol between the haemophilia treatment team and the surgical, anaesthesia and intensive care teams. This was integral in our case as cardiovascular surgery is done off-site from our haemophilia treatment centre and haemostasis laboratory. In conclusion, we report the first case of a PWH treated with a minimally invasive off-pump CABG with continuous infusion factor prophylaxis. We feel that PWH are ideal candidates for such a minimally invasive technique, and the potential to reduce perioperative complications is well worth the extensive planning. Lori Laudenbach, Nurse practitioner for the Bleeding Disorder Program. Dr. Kiaii is a consultant with Medtronic.

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