The first case of surgical myocardial revascularization and endarterectomy of the right carotid artery in the same procedure in a patient with haemophilia A
Abstract
Introduction. Haemophilia A is the most common hereditary coagulation disturbance occurring due to the lack of coagulation factor VIII. It is widely accepted that people with haemophilia have a reduced incidence of coronary artery disease, potentially because of the protective effect of the impaired coagulation against the pathogenic mechanisms of the acute coronary syndrome. Case report. A 53-year-old man with mild haemophilia [FVIII 22% (mild form: more than 5%–40% of normal)] was hospitalized because of frequent anginal pain at rest. Selective coronary angiography revealed a severe three-vessel coronary disease. A need for urgent surgical revascularization was indicated. The color duplex scan showed the existence of hemodynamically significant stenosis on the right internal carotid artery. After consulting a haematologist, a cardiac surgeon, and a vascular surgeon, it was concluded that due to high bleeding risk, the patient should undergo an endarterectomy of the right carotid artery and a triple aortocoronary bypass in the same procedure. Procedures were performed with a substitution of FVIII concentrate. The patient firstly underwent the endarterectomy of the right carotid artery. Then, the left mammary artery graft was implanted to the left anterior descending artery as well as the venous grafts to the first obtuse marginal artery and posterior descending branch. There were no complications. During the revascularization, there was no need for blood transfusion, nor was there excessive bleeding in the postoperative period. The patient was discharged with antithrombotic therapy (aspirin, 50 mg). Conclusion. Patients with haemophilia are not protected against the development of atherosclerosis. Cardiac surgery in these patients presents a unique challenge for medical teams in securing haemostasis. Adequate substitution with factor VIII concentrate provides adequate haemostasis and the possibility for treatment with antiplatelet therapy.
References
Srivastava A, Brewer AK, Mauser-Bunschoten EP, Key NS, Kitchen S, Llinas A, et al. Treatment Guidelines Working Group on Behalf of The World Federation Of Hemophilia. Guidelines for the management of hemophilia. Haemophilia 2013; 19(1): e1‒47.
Mafrici A, Baudo F. Hemophilia and percutaneous coronary interventions. Ital Heart J 2003; 4(10): 731‒3.
Bilora F, Dei Rossi C, Girolami B, Casonato A, Zanon E, Bertomoro A, et al. Do hemophilia A and von Willebrand disease protect against carotid atherosclerosis? A comparative study between coagulopathics and normal subjects by means of carotid echo-color Doppler scan. Clin Appl Thromb Hemost 1999; 5(4): 232‒5.
Rosendaal FR, Briët E, Stibbe J, van Herpen G, Leuven JA, Hofman A, et al. Haemophilia protects against ischaemic heart disease: a study of risk factors. Br J Haematol 1990; 75(4): 525‒30.
Aboyans V, Ricco JB, Bartelink MEL, Björck M, Brodmann M, Cohnert T, et al. ESC Scientific Document Group. 2017 ESC Guidelines on the Diagnosis and Treatment of Peripheral Arterial Diseases, in collaboration with the European Society for Vascular Surgery (ESVS): Document covering atherosclerotic disease of extracranial carotid and vertebral, mesenteric, renal, upper and lower extremity arteries. Endorsed by: the European Stroke Organization (ESO)The Task Force for the Diagnosis and Treatment of Peripheral Arterial Diseases of the European Society of Cardiology (ESC) and of the European Society for Vascular Surgery (ESVS). Eur Heart J 2018; 39(9): 763‒816.
Neumann FJ, Sousa-Uva M, Ahlsson A, Alfonso F, Banning AP, Benedetto U, et al. ESC Scientific Document Group. 2018 ESC/EACTS Guidelines on myocardial revascularization. Eur Heart J 2019; 40(2): 87‒165.
Mannucci PM, Schutgens RE, Santagostino E, MauserBunschoten EP. How I treat age-related morbidities in elderly patients with hemophilia. Blood 2009; 114(26): 5256‒63.
Soucie JM, Nuss R, Evatt BG, Abdelhak A, Cowan L, Hill H, et al. Mortality among males with hemophilia: relations with source of medical care. The Hemophilia Surveillance system Project Investigators. Blood 2000; 96(2): 437‒42.
Darby SC, Kan SW, Spooner RJ, Giangrande PL, Hill FG, Hay CR, et al. Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV. Blood 2007; 110(3): 815‒25.
Plug I, Van Der Bom JG, Peters M, Mauser-Bunschoten EP, de Goede-Bolder A, Heijnen L, et al. Mortality and causes of death in patients with hemophilia, 1992-2001: a prospective cohort study. J Thromb Haemost 2006; 4: 510‒6.
Triemstra M, Rosendaal FR, Smit C, Van der Ploeg HM, Briët E. Mortality in patients with hemophilia. Changes in a Dutch population from 1986 to 1992 and 1973 to 1986. Ann Intern Med 1995; 123(11): 823‒7.
Berger K, Schopohl D, Lowe G, Holme PA, Tait RC, Combescure C, et al. How to compare cardiovascular disease and risk factors in elderly patients with haemophilia with general population. Haemophilia 2016; 22(5): e406‒16.
Biere-Rafi S, Zwiers M, Peters M, van der Meer J, Rosendaal FR, Büller HR, et al. The effect of haemophilia and von Willebrand disease on arterial thrombosis: a systematic review. Neth J Med 2010; 68(5): 207‒14.
Srámek A, Reiber JH, Gerrits WB, Rosendaal FR. Decreased coagulability has no clinically relevant effect on atherogenesis: observations in individuals with a hereditary bleeding tendency. Circulation 2001; 104(7): 762‒7.
Girolami A, Ruzzon E, Fabris F, Varvarikis C, Sartori R, Girolami B. Myocardial infarction and other arterial occlusions in hemophilia a patients. A cardiological evaluation of all 42 cases reported in the literature. Acta Haematol 2006; 116(2): 120‒5.
Smolka G, Kulach A, Dabek J, Szulc A, Gasior Z. Percutaneous coronary intervention with stent implantation in haemophilic A patient with unstable angina. Haemophilia 2007; 13(4): 428‒31.
Bhave P, McGiffin D, Shaw J, Walsh M, McCarthy P, Tran H, et al. Guide to performing cardiac surgery in patients with hereditary bleeding disorders. J Card Surg 2015; 30(1): 61‒9.
Kim DK, Kim DI, Kim MS, Lee EJ, Kim YB, Cho HJ, et al. Successful percutaneous coronary intervention for acute coronary syndrome in a patient with severe hemophilia a. Korean Circ J 2010; 40(10): 527‒9.
Tang M, Wierup P, Terp K, Ingerslev J, Sørensen B. Cardiac surgery in patients with haemophilia. Haemophilia 2009; 15(1): 101‒7.
MacKinlay N, Taper J, Renisson F, Rickard K. Cardiac surgery and catheterization in patients with haemophilia. Haemophilia 2000; 6(2): 84‒8.
Rodriguez V, Burkhart HM, Schmidt KA, Pruthi RK. Hemostatic management of an infant with severe hemophilia A and tetralogy of Fallot for cardiac bypass surgery. Pediatr Blood Cancer 2010; 55(7): 1399‒401.
Gasparović H, Zupancic-Salek S, Brida V, Dulić G, Jelić I. Aortic valve replacement in a patient with severe hemophilia. Coll Antropol 2007; 31(1): 355‒7.
Kaminishi Y, Aizawa K, Saito T, Misawa Y, Madoiwa S, Sakata Y. Modified Bentall operation in a patient with hemophilia A. Jpn J Thorac Cardiovasc Surg 2003; 51(2): 68‒70.
Donahue BS, Emerson CW, Slaughter TF. Case 1--1999. Elective and emergency cardiac surgery on a patient with hemophilia B. J Cardiothorac Vasc Anesth 1999; 13(1): 92‒7.
Meagher PD, Rickard KA, Richards JG, Baird DK. Aortic and mitral valve replacement in a patient with severe haemophilia A. Aust N Z J Med 1981; 11(1): 76‒9.
Murugan SJ, Viswanathan S, Thomson J, Parsons JM, Richards M. Heart surgery in infants with hemophilia. Ann Thorac Surg 2006; 81(1): 336‒9.
Eren A, Friedl R, Hannekum A, Gulbins H. Cardiac surgery in a patient with haemophilia A. Thorac Cardiovasc Surg 2006; 54(3): 212‒4.
Stine KC, Becton DL. Use of factor VIII replacement during open heart surgery in a patient with haemophilia A. Haemophilia 2006; 12(4): 435‒6.
De Bels D, Demeere JL, Dugauquier J, Louis P, Van der Vorst E. Continuous infusion of factor VIIIc during heart surgery in a patient with haemophilia A. Eur J Anaesthesiol 2004; 21(12): 984‒6.
Scharfman WB, Rauch AE, Ferraris V, Burkart PT. Treatment of a patient with factor IX deficiency (hemophilia B) with coronary bypass surgery. J Thorac Cardiovasc Surg 1993; 105(4): 765‒6.
Kanellopoulou T, Nomikou E. Replacement therapy for coronary artery bypass surgery in patients with hemophilia A nnd B. J Card Surg 2018; 33(2): 76‒82.