Cardiac surgery of aortic valve and ascending aorta (Bentall procedure) in a patient with Fabry disease without enzyme replacement therapy: an overview of aortic and aortic valve surgical tips

Introduction: Fabry disease (FD) is a rare disease in which cardiovascular events are one of its manifestations. Aortic and mitral valve involvement are among its manifestations, but due to the concern of suture failure without enzyme replacement therapy (ERT), the desire for its surgery has been very low, and its reported cases have been few. After the introduction of ERT, cardiovascular surgery for such patients has been gradually reported from different places, but due to the high cost of ERT, it is not practically available to patients in the third world and the backward countries. Reports of the experiences of patients with FD who underwent cardiac surgery without ERT can help improve cardiac surgery in these patients. Case Report: A 34-year-old man diagnosed with FD due to chronic renal failure and underwent dialysis 3 times a week for 1.5 years became a candidate for kidney transplant. In pre-transplant examinations, dilatation of aortic root and aortic valve insufficiency were diagnosed, and the patient’s kidney transplant surgery was postponed until after cardiac surgery. The patient underwent cardiac surgery, aortic valve replacement, and ascending aortic replacement, as well as re-implantation of both right and left coronary buttons on the ascending aorta (Bentall procedure). The post-surgery period passed without any special event. The patient’s dialysis continued; after 4 months, he underwent kidney transplant surgery, and the one-year follow-up period also was good. Conclusion: Although there is very little evidence of cardiovascular surgery in patients with FD without ERT, and surgeons have always been worried about suture failure and complications of cardiac surgery, due to the high cost of ERT, this treatment is not available to everyone. The introduced patient underwent cardiac surgery by Bentall procedure without receiving ERT and was discharged without any special complications. By reporting and collecting similar cases, guidance may be provided for patients with FD for whom ERT is not available.