Related Experiment Video
Updated: Jan 20, 2026
Author Spotlight: Development of a Minimally Invasive Large-Animal Model for Reliable and Reproducible Cardiovascular Research
Published on: October 20, 2023
Rapid progression of aortic and mitral stenosis in a patient with AA amyloidosis: a case report
David Frumkin1, Eliane Tabea Taube2, Karl Stangl1
1Department of Cardiology and Angiology, Campus Mitte, Charité-Universitätsmedizin Berlin, Charitéplatz 1, Berlin, Germany.
Insights
Cardiac amyloidosis (CA) combined with chronic dialysis can rapidly worsen aortic valve stenosis, even in young, asymptomatic patients. Regular echocardiograms are crucial for early detection and management of this accelerated valve disease.
Area of Science:
- Cardiology
- Nephrology
- Pathology
Background:
- Aortic stenosis is frequently observed in cardiac amyloidosis (CA).
- Younger patients with CA may be asymptomatic, delaying diagnosis and leading to heart failure.
- Chronic kidney disease and dialysis can accelerate aortic stenosis progression.
Purpose of the Study:
- To highlight the accelerated progression of aortic valve stenosis in a patient with cardiac amyloidosis and chronic dialysis.
- To emphasize the increased perioperative risks in such patients.
Main Methods:
- Case report of a 40-year-old male with AA amyloidosis and end-stage renal failure on chronic dialysis.
- Echocardiography revealed severe aortic and mitral valve stenosis.
- Surgical aortic and mitral valve replacement was performed.
- Histological examination confirmed amyloid presence in excised valves.
Main Results:
- Rapid progression of severe aortic and mitral valve stenosis was identified.
- Histology confirmed significant amyloid infiltration of the native valves.
- Post-operative echocardiography showed prosthetic valve function and preserved left ventricular ejection fraction.
- The patient experienced a complicated post-operative course and died from multiorgan failure.
Conclusions:
- Concomitant cardiac amyloidosis and chronic dialysis accelerate severe aortic valve stenosis onset.
- Asymptomatic young patients are at risk, with perioperative risk increasing with dialysis duration and stenosis severity.
- Regular, short-term echocardiographic monitoring is essential, even in clinically stable patients.
Background:
Aortic stenosis is a common finding in cardiac amyloidosis (CA). Younger patients often remain asymptomatic. If unrecognized, this can lead to serious complications such as heart failure. Progression of aortic stenosis can be accelerated in patients with chronic kidney disease and need for dialysis. Perioperative risk in these patients is often high due to the underlying systemic disease.
Case Summary:
A 40-year-old Caucasian man with known AA amyloidosis, highly active Ankylosing Spondylitis and need for chronic dialysis due to end-stage chronic renal failure presented for echocardiographic routine exam without reporting any cardiac symptoms. At the last visit 4 years ago, a normal heart valve function was noted and no echocardiographic follow-up was performed in the following. Now, rapid progression with severe aortic valve and mitral valve stenosis was stated and the patient underwent combined aortic and mitral surgical valve replacement following discussion in the multidisciplinary cardiology meeting. Macroscopic examination of the valves revealed significant calcification and histological examination showed the high presence of amyloid by Congo-red staining and immunohistological staining for AA-Amyloid. Both valve prosthetic devices showed normal function as well as a normal left ventricular ejection fraction in initial post-operative transoesophageal echocardiography. After prolonged and complicated post-operative course in the intensive care unit the patient died 3 months after surgery due to intractable multiorgan failure in combined severe abdominal septic and cardiogenic shock.
Discussion:
Concomitant CA and chronic dialysis can accelerate the onset of severe aortic valve stenosis. Young patients, as in this case, often stay asymptomatic, perioperative risk increases with duration of chronic dialysis and severity of valve stenosis. This increases the need for regular short-term echocardiographic examinations even in clinical stable patients.
Related Concept Videos
01:22Mitral Stenosis I: Introduction
01:27Mitral Stenosis IV: Nursing Management
01:26Mitral Stenosis III: Medical Management
01:23Mitral Stenosis II: Clinical features and Diagnostic Tests
01:24Data Reporting and Recording
01:20Mitral Regurgitation I: Introduction