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A fatal case of constrictive pericarditis due to a marked, selective pericardial accumulation of amyloid
J P Daubert1, J Gaede, H J Cohen
1Department of Medicine, Duke University Medical Center, Durham, North Carolina.
Insights
Amyloidosis can cause constrictive pericarditis by infiltrating the pericardium, not just the heart muscle. This finding is crucial for diagnosing heart conditions, especially when symptoms overlap.
Area of Science:
- Cardiology
- Pathology
- Medical Diagnostics
Background:
- Distinguishing constrictive pericarditis from restrictive cardiomyopathy is a common diagnostic challenge.
- Amyloidosis is a frequent cause of restrictive cardiomyopathy.
Observation:
- This report details a unique case of constrictive pericarditis caused by extensive pericardial amyloid infiltration.
- The patient exhibited minimal myocardial amyloid and a normal heart weight.
Findings:
- Amyloid deposition can predominantly affect the pericardium, leading to constrictive pericarditis.
- Pericardial amyloidosis can mimic the hemodynamic presentation of myocardial amyloidosis.
Implications:
- A diagnosis of amyloidosis via biopsy does not exclude constrictive pericarditis due to pericardial amyloid infiltration.
- This case broadens the understanding of amyloid heart disease manifestations and diagnostic considerations.
Abstract:
Distinguishing constrictive pericarditis from restrictive cardiomyopathy, usually due to amyloidosis, is a relatively frequent and difficult diagnostic problem. This report describes, for the first time, a patient with constrictive pericarditis caused by direct, extensive infiltration of the pericardium by amyloid, with only minimal amyloid in the myocardium, and a normal heart weight of 320 g. This patient demonstrates that amyloid may be predominantly deposited in the pericardium and actually cause constrictive pericarditis, as well as simulate its hemodynamic presentation by myocardial deposition. Given a clinical and hemodynamic presentation compatible with either constrictive or restrictive disease, an endomyocardial biopsy or other biopsy revealing amyloidosis does not necessarily rule out pericardial constriction that may be due to amyloid infiltration. The relationship between constrictive pericarditis, seen in this patient, and the other more common manifestations of amyloid heart disease, and the hemodynamic profiles of amyloid cardiomyopathy and constrictive pericarditis are reviewed.