Tags

Type your tag names separated by a space and hit enter

Extracorporeal membrane oxygenation as bridge-to-decision in acute heart failure due to systemic light-chain amyloidosis.
Am J Case Rep. 2015 Mar 24; 16:174-81.AJ

Abstract

PATIENT

Female, 58.

FINAL DIAGNOSIS

Acute hear failure.

SYMPTOMS

Dispnoea • edema • fatigue.

MEDICATION

CLINICAL PROCEDURE

Bone marrow biopsy • endomyocardial biopsy • abdominal subcutaneous fat biopsy under ECMO support.

SPECIALTY

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.

CASE REPORT

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+Show Affiliations

Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.Department of Internal Medicine, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.Department of Internal Medicine, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.Department of Internal Medicine, Centro Hospitalar Entre-Douro e Vouga, Santa Maria da Feira, Portugal.Department of Pathology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.Department of Haematology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

25803181

Citation

Mancio Silva, Jennifer, et al. "Extracorporeal Membrane Oxygenation as Bridge-to-decision in Acute Heart Failure Due to Systemic Light-chain Amyloidosis." The American Journal of Case Reports, vol. 16, 2015, pp. 174-81.
Mancio Silva J, Fontes-Carvalho R, Valente D, et al. Extracorporeal membrane oxygenation as bridge-to-decision in acute heart failure due to systemic light-chain amyloidosis. Am J Case Rep. 2015;16:174-81.
Mancio Silva, J., Fontes-Carvalho, R., Valente, D., Almeida, C., Cruz, A. J., Tente, D., Coelho, H., Oliveira, M., Albuquerque, A., & Ribeiro, V. G. (2015). Extracorporeal membrane oxygenation as bridge-to-decision in acute heart failure due to systemic light-chain amyloidosis. The American Journal of Case Reports, 16, 174-81. https://doi.org/10.12659/AJCR.892772
Mancio Silva J, et al. Extracorporeal Membrane Oxygenation as Bridge-to-decision in Acute Heart Failure Due to Systemic Light-chain Amyloidosis. Am J Case Rep. 2015 Mar 24;16:174-81. PubMed PMID: 25803181.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Extracorporeal membrane oxygenation as bridge-to-decision in acute heart failure due to systemic light-chain amyloidosis. AU - Mancio Silva,Jennifer, AU - Fontes-Carvalho,Ricardo, AU - Valente,Dília, AU - Almeida,Cristiana, AU - Cruz,Antonio José, AU - Tente,David, AU - Coelho,Henrique, AU - Oliveira,Marco, AU - Albuquerque,Aníbal, AU - Ribeiro,Vasco Gama, Y1 - 2015/03/24/ PY - 2015/3/25/entrez PY - 2015/3/25/pubmed PY - 2015/7/28/medline SP - 174 EP - 81 JF - The American journal of case reports JO - Am J Case Rep VL - 16 N2 - PATIENT: Female, 58. FINAL DIAGNOSIS: Acute hear failure. SYMPTOMS: Dispnoea • edema • fatigue. MEDICATION: — CLINICAL PROCEDURE: Bone marrow biopsy • endomyocardial biopsy • abdominal subcutaneous fat biopsy under ECMO support. SPECIALTY: 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. CASE REPORT: 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. SN - 1941-5923 UR - https://www.unboundmedicine.com/medline/citation/25803181/Extracorporeal_membrane_oxygenation_as_bridge_to_decision_in_acute_heart_failure_due_to_systemic_light_chain_amyloidosis_ L2 - https://www.amjcaserep.com/download/index/idArt/892772 DB - PRIME DP - Unbound Medicine ER -