The constellation of heart failure with preserved ejection fraction, low-voltage ECG despite ventricular hypertrophy, nephrotic syndrome, macroglossia, periorbital purpura, and neuropathy should raise suspicion for amyloidosis.
Patients with cardiac amyloidosis are exquisitely sensitive to alterations in preload, contractility, and heart rate. Standard heart failure protocols may be detrimental.
Multi-organ dysfunction is common due to the infiltrative nature of amyloidosis affecting multiple organ systems simultaneously.
Factor X deficiency and vascular fragility from amyloid deposits significantly increase bleeding risk, particularly during invasive procedures.
Advanced cardiac involvement (Stage III/IV) carries a poor prognosis with median survival less than 6 months despite treatment.
While disease-modifying therapy can effectively reduce light chain production, patients with advanced organ involvement may not survive long enough to benefit from treatment.
Management requires collaboration between intensive care, cardiology, hematology, and renal specialists.
