Case of the Month #56 The Protein Puzzle: A Case of Progressive Multi-Organ Dysfunction

Structural changes

• Extracellular amyloid deposition between myocytes
• Progressive wall thickening with reduced chamber volume
• Increased ventricular stiffness and reduced compliance

Functional consequences

• Diastolic dysfunction (initially with preserved systolic function)
• Progressive systolic dysfunction as disease advances
• Restrictive physiology with elevated filling pressures
• Atrial dysfunction and enlargement with thrombosis risk

Conduction system

• Infiltration of conduction pathways
• Bundle branch blocks, AV blocks
• Increased risk of atrial and ventricular arrhythmias

Microvascular dysfunction

• Coronary microvascular amyloid deposition
• Reduced coronary flow reserve
• Angina despite normal epicardial coronary arteries

Autonomic dysfunction

• Orthostatic hypotension
• Impaired baroreceptor response
• Increased sensitivity to vasodilators and negative inotropes