P022: ANESTHETIC MANAGEMENT OF AN ORTHOTOPIC HEART TRANSPLANT IN A PATIENT WITH EMERY-DREIFUSS MUSCULAR DYSTROPHY
Matthew T Gunst, MD; Mark Bleiweis, MD; Gregory M Janelle, MD, FASE, FASA; University of Florida
Introduction: Muscular Dystrophies cause progressive muscle weakness and wasting, usually beginning in childhood and often affecting the cardiac muscle. Emery-Dreifuss Muscular Dystrophy (EDMD) is a rare group of muscular dystrophies, causing proximal muscle weakness and dilated cardiomyopathy. Here we present the case of a 14-year-old with EDMD necessitating cardiac transplantation.
Case Presentation: A 14-year-old male with a PMH of EDMD with a family history of the disease in his father and older sister presented with intermittent chest pain and nausea. He was found to be in acute on chronic severe biventricular heart failure with TTE showing global hypokinesis with an LVEF of 10-15% and a RVEF < 20%. He was placed on epinephrine and milrinone infusions and listed 1A for transplant, shortly after which a donor was found. A preinduction arterial line was placed and GETA induced with midazolam, fentanyl, and rocuronium. A right internal jugular introducer was placed with a pulmonary artery catheter floated to wedge depth. Maintenance of anesthesia was achieved via TIVA with propofol and sufentanil. Intraoperative TEE confirmed global hypokinesis and an EF of 10%. The patient was placed on CPB and the donor heart was transplanted successfully. The patient was weaned off CPB on epinephrine, milrinone, and nitroprusside infusions and needed no blood products. Post-transplant TEE confirmed a vigorous heart with normal EF and function. The patient was extubated in the OR at the end of the case and transferred to the congenital heart ICU in stable condition.
Discussion: Emery-Dreifuss Muscular Dystrophy was once mistaken for a mild case of Becker or Duchene Muscular Dystrophies but has been recognized as a separate group of diseases caused by mutations in lamin or emerin nuclear membrane proteins. EDMD can be either X-linked or Autosomal Dominant with a variable penetrance, as illustrated in our patient’s family. TIVA was utilized for concerns of rhabdomyolysis and potential MH susceptibility. Succinylcholine was avoided due to hyperkalemia concerns. Anesthesiologists should be aware of these considerations in uncommon muscular dystrophies.