Aneurysm Repair in a Patient With Systemic Mastocytosis

Cardiac Bypass Surgery in a Patient with an Indolent Systemic Mastocytosis

“…the cumulative incidence of anaphylaxis in adult patients with mastocytosis is as high as 49%. Actually, there is a dearth of literature on preoperative management in the setting of systemic mastocytosis, mostly consisting of isolated case reports in cardiac surgery [4][5][6]. In fact, there are no clear guidelines for safe surgery for those patients requiring cardiac surgery.…”

Case report on aortic valve replacement in adult woman with systemic mastocytosis (RCD code: VIII)

“…There is very limited evidence on this filed available in the literature. Four similar case reports have been published, which included cases of an AVR, valve sparing root and ascending aorta repair, heart transplantation and atrial septal defect closure [6][7][8][9]. Wanamaker et al reported event-free AVR in a 72 year-old female with mastocytosis.…”

“…Duggal et al presented a case report on successful management of diffuse allergic reaction in a patient with systemic mastocytosis undergoing ascending aortic aneurysm repair. Hydrocortisone, diphenhydramine, ranitidine and methylene blue were administrated to stabilize the patient [7]. Moro et al performed heart transplantation in a 56-years-old woman with dilated cardiomiopathy and mastocystosis with no perioperative allergic complications [8].…”

Mitral Valve Replacement in a Patient With Systemic Mastocytosis

“…4,8,9 In cardiac surgery patients, physiological stressors such as the use of CPB, ischemia-reperfusion injury, hypothermia, pain, medications, and operative trauma are strong nonimmune stimulators of MC degranulation and potentially could cause a severe hypersensitivity reaction if the procedure is not planned properly or the condition is unknown. 6,9,10 Preoperative management is aimed to avoid trigger stimuli responsible for MC degranulation and block the effects of the mediators eventually released by the MC. Several prophylaxis protocols that include the administration of histamine-1/histamine-2 blockers, corticosteroids, and benzodiazepines are available without having demonstrated the real superiority of any of them.…”