title: Extracorporeal membrane oxygenation as bridge-to-decision in acute heart failure due to systemic light-chain amyloidosis.
abstract: METHODS
  Female, 58.
  
  
  METHODS
  Acute hear failure.
  
  
  METHODS
  Dispnoea • edema • fatigue.
  
  
  METHODS
  —
  
  
  METHODS
  Bone marrow biopsy • endomyocardial biopsy • abdominal subcutaneous fat biopsy under ECMO support.
  
  
  METHODS
  Cardiology.
  
  
  OBJECTIVE
  Rare disease.
  
  
  BACKGROUND
  Cardiac amyloidosis results from the amyloid deposition in heart tissue, either in the context of a systemic disease or as a localized form. Several pro-amyloid proteins can produce amyloid deposits in the heart. Each of these amyloidoses has characteristic clinical (cardiac and extracardiac) features, and a specific diagnosis and treatment.
  
  
  METHODS
  A 58-year-old woman who presented with acute heart failure and echocardiographic findings strongly suggestive of infiltrative cardiomyopathy needed percutaneous veno-arterial extracorporeal membrane oxygenation (ECMO) as bridge-to-decision. Amyloid deposition was found on endomyocardial and bone marrow biopsies. Bone marrow plasma cell infiltrate with acute renal lesion and hypercalcemia confirmed the diagnosis of multiple myeloma-associated systemic light-chain amyloidosis (AL). Refractory shock with multi-organic failure syndrome persisted and no improvements in left ventricular function and structure were seen. After extensive discussion by a multidisciplinary team, and with the patients' family, she was not considered eligible for high-dose chemotherapy and/or autologous stem cell transplantation, heart transplantation, or sequential heart with autologous stem cell transplantation. The patient died a few hours after ECMO withdrawal. During the 14 days of ECMO support no major bleeding or thrombotic complications occurred.
  
  
  CONCLUSIONS
  The clinician must consider a diagnosis of cardiac amyloidosis in patients with heart failure, a restrictive type of cardiomyopathy with ventricular hypertrophy in the absence of valve abnormalities, or uncontrolled arterial hypertension. Although developments in chemotherapy have greatly improved the outcomes in AL amyloidosis, the prognosis of patients with severe cardiac involvement remains very poor. ECMO is potentially a reliable bridge-to-diagnosis and bridge-to-decision in these patients. An experienced ECMO team, careful patient selection, and rigorous management protocols with objective criteria to wean or stop ECMO are needed.
authors: Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.
authors: Jennifer
authors: J
authors: Mancio Silva
authors: Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.
authors: Ricardo
authors: R
authors: Fontes-Carvalho
authors: Department of Internal Medicine, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.
authors: Dília
authors: D
authors: Valente
authors: Department of Internal Medicine, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.
authors: Cristiana
authors: C
authors: Almeida
authors: Department of Internal Medicine, Centro Hospitalar Entre-Douro e Vouga, Santa Maria da Feira, Portugal.
authors: Antonio José
authors: AJ
authors: Cruz
authors: Department of Pathology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.
authors: David
authors: D
authors: Tente
authors: Department of Haematology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.
authors: Henrique
authors: H
authors: Coelho
authors: Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.
authors: Marco
authors: M
authors: Oliveira
authors: Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.
authors: Aníbal
authors: A
authors: Albuquerque
authors: Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.
authors: Vasco Gama
authors: VG
authors: Ribeiro
mesh_terms: D000208:Acute Disease; D000686:Amyloidosis / Q000150:complications* / Q000276:immunology; D003937:Diagnosis, Differential; D015199:Extracorporeal Membrane Oxygenation / Q000379:methods; D017809:Fatal Outcome; D005260:Female; D006333:Heart Failure / Q000000981:diagnostic imaging / Q000235:genetics* / Q000628:therapy*; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications*; D009102:Multiple Organ Failure / Q000209:etiology; D012307:Risk Factors; D014463:Ultrasonography
chemical_list: D007147:Immunoglobulin Light Chains