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[Cardiac amyloidosis with atrioventricular valve thickening and left atrial dysfunction: a case report]
N Fukuda1, N Takeichi, T Soeki
1Clinical Research Institute, National Hospital, Kagawa.
Insights
Cardiac amyloidosis can cause angina pectoris due to amyloid infiltration of small coronary arteries. Key diagnostic clues include atrioventricular valve thickening and left atrial dysfunction.
Area of Science:
- Cardiology
- Cardiovascular Pathology
Background:
- Cardiac amyloidosis is a progressive disease characterized by amyloid protein deposition in the heart.
- It can lead to diastolic dysfunction, heart failure, and arrhythmias.
Observation:
- A 70-year-old man with cardiac amyloidosis presented with exertional chest pain and ischemic electrocardiogram changes.
- Echocardiography revealed characteristic "granular sparkling" myocardium, thickened valves, and left atrial dysfunction.
- Coronary angiography showed normal arteries, but impaired coronary flow reserve was noted.
Findings:
- Amyloid infiltration of intramural coronary arteries was identified as the likely cause of angina pectoris.
- Rectal biopsy confirmed amyloidosis via Congo red staining.
- Impaired coronary flow reserve despite normal coronary arteries suggests microvascular dysfunction.
Implications:
- This case highlights the importance of considering cardiac amyloidosis in patients with unexplained angina and ischemic changes.
- Atrioventricular valve thickening and left atrial dysfunction are crucial diagnostic indicators.
- Early diagnosis and management of cardiac amyloidosis are essential to prevent adverse cardiovascular events.
Abstract:
A 70-year-old man with cardiac amyloidosis was referred to our hospital because of exertional chest pain accompanied by ischemic changes on electrocardiography on April 2, 1997. Transthoracic echocardiography revealed a normal size and normally contracted left ventricle without hypertrophy and "granular sparkling" quality of the myocardium, thickening of the mitral and tricuspid valves, and enlargement of the left atrium with reduced booster pump function. Pulsed Doppler mitral inflow velocity wave showed a pseudonormalized pattern, and pulmonary venous flow velocity wave showed a non-compliant pattern. Transesophageal echocardiography revealed thickening and reduced movement of the interatrial septum and reduced flow velocity in the left atrial appendage, suggesting left atrial dysfunction. Adenosine triphosphate (ATP) stress thallium-201 myocardial scintigraphy showed reversible patchy defect mainly in the posterolateral wall. Left ventricular end-diastolic and pulmonary capillary wedge pressures were mildly elevated. Angiography showed normal coronary arteries, but coronary flow reserve measured by administering intravenous ATP in the left anterior descending artery was severely impaired. A rectal biopsy specimen was positive by Congo red staining. Thus, angina pectoris in this patient may be due to amyloid infiltration of the small intramural coronary arteries. Atrioventricular valve thickening and left atrial dysfunction are important clues to diagnose cardiac amyloidosis.