Isolated cardiac amyloidosis with normal interventricular septum thickness: a case report

Cardiol Young. 2022 Dec;32(12):2029-2031. doi: 10.1017/S1047951122000993. Epub 2022 Apr 25.

Abstract

Cardiac amyloidosis presented with normal interventricular septum is an extremely rare entity, and diagnosis may be difficult. This report discusses a 44-year-old female who presented with worsening dyspnoea on exertion, orthopnoea, and lower-extremity oedema. Electrocardiogram depicted low voltage in limb leads and a pseudoinfarct pattern. Echocardiogram revealed biatrial dilatation without changes of ventricular chambers and restrictive filling physiology. A diagnosis of cardiac amyloidosis was considered. Cardiac MRI was pursued, showing delayed gadolinium enhancement, and this ultimately led to the myocardial biopsy confirming the diagnosis of cardiac amyloidosis. The case suggests that patients who present with heart failure of uncertain aetiology, amyloidosis could be a cause of cardiomyopathy despite the absence of "classical" echocardiographic features of amyloid deposition such as an increased interventricular septum thickness or"brilliant sparkled"appearance of the myocardium.

Keywords: Cardiac MRI; Cardiac amyloidosis; Echocardiography; Interventricular septum; Light-chain amyloidosis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Amyloidosis* / diagnosis
  • Amyloidosis* / pathology
  • Cardiomyopathies* / diagnostic imaging
  • Cardiomyopathies* / etiology
  • Contrast Media
  • Female
  • Gadolinium
  • Humans
  • Myocardium / pathology
  • Ventricular Septum* / diagnostic imaging

Substances

  • Contrast Media
  • Gadolinium