Perioperative management of a patient with profound thrombocytopenia secondary to MYH9-RD presenting for thoracic aortic aneurysm repair and aortic valve replacement

Abstract Preoperative thrombocytopenia is associated with an increased risk of morbidity and mortality following cardiothoracic surgery. We describe a case of a 28-year-old female with a history of myosin heavy chain 9-related disease (MYH9-RD) and a presenting platelet count of 6,000 per microliter undergoing a Bentall procedure for aortic valve insufficiency and a thoracic aortic aneurysm. A multidisciplinary team approach was used in order to optimize the patient for surgery and guide her through the perioperative period. In this report, we discuss the epidemiology and pathophysiology of MYH9-RD and describe the preoperative, intraoperative, and postoperative considerations for such patients presenting for cardiothoracic surgery.