title: Total Artificial Heart Implantation as a Bridge to Heart Transplantation in an Active Duty Service Member With Amyloid Cardiomyopathy.
abstract: Cardiac involvement by light-chain (AL) amyloid occurs in up to 50% of patients with primary AL amyloidosis. The prognosis of amyloid heart disease is poor with 1-year survival rates of 35 to 40%. Historically, heart transplantation was considered controversial for patients with AL amyloid cardiomyopathy (CM) given the systemic nature of the disease and poor survival. We present a case report of an active duty service member diagnosed with advanced cardiac amyloid who underwent total artificial heart transplant as a bridge to heart transplant and eventual autologous stem cell transplant.
  
  
  
  A 47-year-old active duty male initially evaluated for atypical chest pain was found to have severe concentric left ventricular hypertrophy on echocardiogram but normal voltage on electrocardiogram. Cardiac magnetic resonance imaging, laboratory studies, and bone marrow biopsy established the diagnosis of cardiac amyloidosis. At the time of diagnosis, the patient's prognosis was very poor with a median survival of 5 months on the basis of the Mayo Clinic revised prognostic staging system for amyloidosis. The patient developed rapidly progressive left ventricular dysfunction and heart failure leading to cardiac arrest. The patient received a total artificial heart as a bridge to orthotopic heart and kidney transplantation and eventual stem cell transplant. He continues to be in remission and has a fair functional capacity without restriction in activities of daily living or moderate exercise.
  
  
  
  Amyloid CM is a rare and devastating disease. The natural course of the disease has made heart transplant in these patients controversial. Modern advancements in chemotherapies and advanced heart failure treatments have improved outcomes for select patients with AL amyloid CM undergoing heart transplantation. There is ongoing research seeking improvement in treatment options and outcomes for patients with this deadly disease.
authors.affiliation: Department of Cardiology, Naval Medical Center San Diego, 34730 Bob Wilson Drive, Suite 303, San Diego, CA 92134-3303.
authors.forename: Michael S
authors.initials: MS
authors.lastname: Scully
authors.affiliation: Department of Cardiology, Naval Medical Center San Diego, 34730 Bob Wilson Drive, Suite 303, San Diego, CA 92134-3303.
authors.forename: Dylan E
authors.initials: DE
authors.lastname: Wessman
authors.affiliation: Department of Radiology, Naval Medical Center San Diego, 34730 Bob Wilson Drive, Suite 303, San Diego, CA 92134-3303.
authors.forename: James M
authors.initials: JM
authors.lastname: McKee
authors.affiliation: Department of Cardiology, Naval Medical Center San Diego, 34730 Bob Wilson Drive, Suite 303, San Diego, CA 92134-3303.
authors.forename: Gregory M
authors.initials: GM
authors.lastname: Francisco
authors.affiliation: Department of Cardiology, Naval Medical Center San Diego, 34730 Bob Wilson Drive, Suite 303, San Diego, CA 92134-3303.
authors.forename: Keshav R
authors.initials: KR
authors.lastname: Nayak
authors.affiliation: Cedars-Sinai Medical Center, Heart Institute, 127 South San Vincente Boulevard, Los Angeles, CA 90048.
authors.forename: Jon A
authors.initials: JA
authors.lastname: Kobashigawa
mesh_terms: D000686:Amyloidosis / Q000150:complications*; D000893:Anti-Inflammatory Agents / Q000494:pharmacology / Q000627:therapeutic use; D000069286:Bortezomib / Q000494:pharmacology / Q000627:therapeutic use; D009202:Cardiomyopathies / Q000150:complications*; D003520:Cyclophosphamide / Q000494:pharmacology / Q000627:therapeutic use; D003907:Dexamethasone / Q000494:pharmacology / Q000627:therapeutic use; D006333:Heart Failure / Q000150:complications / Q000209:etiology / Q000601:surgery; D016027:Heart Transplantation; D006354:Heart, Artificial; D006801:Humans; D007166:Immunosuppressive Agents / Q000494:pharmacology / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D008889:Military Personnel; D020097:Natriuretic Peptide, Brain / Q000032:analysis / Q000097:blood; D010446:Peptide Fragments / Q000032:analysis / Q000097:blood; D020107:Troponin T / Q000032:analysis / Q000097:blood
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D010446:Peptide Fragments; D020107:Troponin T; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain; D000069286:Bortezomib; D003907:Dexamethasone; D003520:Cyclophosphamide