Amyloidosis in Critical Care: Case of the Month
Overview
Theme: Cardiac amyloidosis, cardiogenic shock, multi-organ dysfunction.
Key words: Cardiac amyloidosis, Cardiogenic shock, Restrictive cardiomyopathy, Macroglossia, Multi-organ failure, Periorbital purpura, Light chain disease, Coagulopathy, Arrhythmia, Protein misfolding
A 62-year-old male presented to the Emergency Department with progressive dyspnea, bilateral lower limb oedema, and a 20kg unintentional weight loss over six months. The patient reported increasing orthopnea requiring three pillows at night and episodes of presyncope when standing.
On examination, he was hypotensive with an irregular pulse. Cardiovascular examination revealed an elevated JVP, irregular rhythm, soft S1, and S3 gallop. Respiratory examination showed bilateral basal crepitations. Abdominal examination revealed hepatomegaly 4cm below costal margin and mild ascites. Notable findings included macroglossia with scalloped edges, periorbital purpura, waxy skin papules, and significant bilateral pitting oedema extending to thighs.
Initial investigations showed mild anemia, elevated urea and creatinine, low albumin, elevated cardiac biomarkers, and mildly prolonged PT. ECG showed low voltage QRS complexes and poor R wave progression. Echocardiogram revealed biventricular hypertrophy with a "speckled" myocardial appearance and preserved LVEF with restrictive filling pattern. Immunological tests showed a small monoclonal band, elevated lambda free light chains, and a significantly abnormal Lambda/Kappa ratio.
The patient was initially stabilized in the acute medical unit but developed worsening hypotension and oliguria despite careful fluid resuscitation. He was transferred to ICU with a presumptive diagnosis of cardiogenic shock secondary to cardiac amyloidosis.