1 / 3

Case of the week 08-23: A tricky diagnostic challenge: (page 1 of 3)

Case of the week 08-23: A tricky diagnostic challenge: (page 1 of 3).

jory
Download Presentation

Case of the week 08-23: A tricky diagnostic challenge: (page 1 of 3)

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Case of the week 08-23: A tricky diagnostic challenge: (page 1 of 3) History: A man in his 40s presenting to respiratory medicine with 6 months breathlessness and hemoptysis. Past medical history: athleticism, proteinuria, hematuria, carpal tunnel syndrome and sinusitis. Medication: No current medication. Respiratory/general investigations: Detailed work up negative apart from mild anaemia* Cardiology:ECG: see below, Echo: concentric LVH, good systolic function, grade 2 diastolic dysfunctionETT: positive for ischemia (but note rest ECG, centre), so angiography: Angiography: normal.The patient then presents to ER with the tachycardia below right: EP study non-inducible. Question: What is the diagnosis? Where to next? Anna Herrey, Kikkiri Naresh, Julian Gillmore, Chiara Bucciarelli-Ducci, Jaspal Kooner Imperial College Healthcare, Royal Brompton Hospital and University College London, UK *CXR, V:Q scan, bronchoscopy normal.U+E/coag/LFT’s/CRP/ESR/ANA/ANCA/antiphospholipid/complement/α1-AT/GBM Ab/RF: normal.

  2. Case of the week 08-23: A tricky diagnostic challenge (page 2 of 3) Cine CMR: Marked biventricular long axis reduction with preserved radial function. Mild concentric LVH. Contrast CMR: Remarkable, extensive LGE in a non-ischemic pattern, most prominent subendocardiallyCMR diagnosis: Likely cardiac amyloidosis

  3. Case of the week 08-23: A tricky diagnostic challenge (page 3 of 3) Subsequent investigation: Cardiac biopsy (right) showed green birefringence on Congo red staining confirming cardiac amyloid (note thickened arteriolar wall). A bone marrow biopsy showed plasma cells (left) and Lambda light chains (middle), and urine showed excessive lambda light chains. Management and follow-up: ‘light’ chemotherapy (prednisolone /melphalan). Serial echo demonstrated progression to restrictive physiology and systolic dysfunction. Work-up for cardiac transplant commenced with a plan for intensive chemo & stem cell transplant, but events overtook this plan with end stage renal and cardiac failure. Conclusion: CMR frequently solves the most complex diagnostic conundrums, here cardiac amyloidosis, although in this case, the outcome was poor.

More Related