Amyloid cardiomyopathy – a diagnostic challenge

Authors

  • Gustavo Barbosa Serviço de Cardiologia do Hospital de São João e Serviço de Medicina Interna do Hospital do Padre Américo
  • Inês Rangel Serviço de Cardiologia do Hospital de São João e Serviço de Medicina Interna do Hospital do Padre Américo
  • Teresa Pinho Serviço de Cardiologia do Hospital de São João e Serviço de Medicina Interna do Hospital do Padre Américo
  • Luísa Lobato Serviço de Cardiologia do Hospital de São João e Serviço de Medicina Interna do Hospital do Padre Américo
  • Cristina Gavina Serviço de Cardiologia do Hospital de São João e Serviço de Medicina Interna do Hospital do Padre Américo
  • Paulo Bettencourt Serviço de Cardiologia do Hospital de São João e Serviço de Medicina Interna do Hospital do Padre Américo
  • Maria Júlia Maciel Serviço de Cardiologia do Hospital de São João e Serviço de Medicina Interna do Hospital do Padre Américo

Keywords:

Restrictive cardiomyopathy, Amyloidosis, Endomyocardial biopsy, Cardiovascular magnetic resonance imaging, Late gadolinium enhancement

Abstract

The authors present the case of a 71-year-old male patient, with
previous history of arterial hypertension and diabetes mellitus,
admitted with dyspnoea and anasarca. The echocardiogram
presented biatrial dilation, severe left ventricle (LV) hypertrophy
and mild LV systolic dysfunction. Abdominal computed tomography
scan revealed hepatosplenomegaly and large volume ascites.
Readmitted two months later with symptoms worsening. A new
echocardiogram was performed, revealing, besides the referred
changes, a transmitral flow with a restrictive pattern. Cardiac
catheterization excluded coronary disease. Endomyocardial biopsy
revealed fibroelastosis lesions and nuclear hypertrophy, without
initial evidence of amyloid deposition. Cardiovascular magnetic
resonance imaging (CMRI) revealed bi-ventricular hypertrophy
and a pattern of late gadolimium enhancement (LGE) suggestive
of restrictive cardiomyopathy (amyloidosis). Abdominal fat biopsy
identified amyloid substance by Congo red-stain.
The diagnosis of amyloid cardiomyopathy is difficult. Although
endomyocardial biopsy is the gold standard, CMRI arises as
a non-invasive method useful to determine cardiomyopathies
etiology due to its capacity of characterizing tissue through LGE.

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References

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Additional Files

Published

2012-12-31

How to Cite

1.
Barbosa G, Rangel I, Pinho T, Lobato L, Gavina C, Bettencourt P, Maciel MJ. Amyloid cardiomyopathy – a diagnostic challenge. RPMI [Internet]. 2012 Dec. 31 [cited 2024 Nov. 23];19(4):205-10. Available from: https://revista.spmi.pt/index.php/rpmi/article/view/1176

Issue

Vol. 19 No. 4 (2012): Outubro/ Dezembro

Section

Case Reports

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