SHM Converge Scientific Abstract ePoster Gallery-A Race Against Time: Cardiac Infiltration in Plasma Cell Dyscrasia

Pdf Summary

This case highlights the importance of considering infiltrative cardiac disease, especially cardiac amyloidosis, when ischemic causes of heart failure have been excluded. A 46-year-old man presented with recurrent heart failure symptoms, including bilateral leg swelling, shortness of breath, fatigue, and orthopnea. He had similar symptoms seven months earlier and was diagnosed with HFpEF, but prior coronary evaluation showed no evidence of coronary artery disease, including a zero calcium score on CCTA and a negative outpatient cardiac catheterization.<br /><br />On current examination, he was hypotensive and tachypneic with edema, crackles, decreased breath sounds at the bases, and abdominal distension. ECG showed low-voltage limb leads, and transthoracic echocardiography revealed reduced ejection fraction (35–40%), severe pulmonary hypertension, diffuse hypokinesis, and markedly increased left ventricular wall thickness. These findings raised suspicion for infiltrative cardiac disease.<br /><br />Workup demonstrated a plasma cell dyscrasia with an M-spike on serum electrophoresis, elevated lambda light chains, and monoclonal lambda light chains in urine immunofixation. Bone marrow biopsy showed 10–20% plasma cells, though Congo red staining was negative. The patient was started on CyBorD therapy for lambda-restricted monoclonal plasma cell dyscrasia.<br /><br />Despite treatment, his condition worsened rapidly, progressing to cardiogenic shock and ultimately cardiac arrest and death. The case underscores that lambda-restricted plasma cell dyscrasias can cause cardiac infiltration and fatal amyloidosis. Delayed diagnosis of infiltrative cardiac disease is associated with increased mortality, emphasizing the need for early evaluation in patients with HFpEF and increased ventricular wall thickness. Recommended diagnostic tools include cardiac MRI, serum and urine studies, free light chain assays, tissue biopsy, nuclear scintigraphy, and, in selected cases, genetic testing.

Asset Subtitle

Navin Bhatt

Meta Tag

Author List Maryam Hajiabbasi, Nagma Shah, Navin Bhatt, Ranjit Sapkota, Shristi Nepal, Sunil Bogati

Category Clinical Vignettes

Concept Heart Failure

Concept Cardiac Amyloidosis

Concept Infiltrative Cardiac Disease

Concept Myocardial Infiltration

Concept Low-Voltage ECG

Distinguished Non-Finalist

Presenter Organization Icahn School of Medicine at Mount Sinai / Elmhurst

Presenting Author Navin Bhatt

Track Adult

Keywords

cardiac amyloidosis

infiltrative cardiac disease

heart failure with preserved ejection fraction

plasma cell dyscrasia

lambda light chains

low-voltage ECG

ventricular wall thickening

cardiogenic shock

Congo red staining

cardiac MRI

Heart Failure

Cardiac Amyloidosis

Infiltrative Cardiac Disease

Myocardial Infiltration

Low-Voltage ECG