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Published on: February 4, 2021
Osteitis deformans (Paget's disease) and calcific disease of the heart valves
1Division of Cardiovascular Medicine, Department of Veterans Affairs Health Care System, Palo Alto, California, USA.
Insights
Severe Paget's disease (osteitis deformans) significantly increases the risk of aortic stenosis (AS) and cardiac conduction abnormalities. Moderate Paget's disease showed no increased prevalence of these cardiovascular conditions.
Area of Science:
- Cardiology
- Rheumatology
- Pathology
Background:
- Paget's disease of bone (osteitis deformans) is a chronic condition characterized by abnormal bone remodeling.
- Calcific aortic valve stenosis (AS) is a common valvular heart disease, particularly in older adults.
- The association between Paget's disease and AS has been suggested but requires further investigation.
Purpose of the Study:
- To investigate the prevalence of calcific aortic valve stenosis (AS) in patients with severe and moderate Paget's disease.
- To compare the incidence of AS and related cardiac conduction abnormalities in Paget's disease patients versus age-matched controls.
- To determine if the severity of Paget's disease correlates with cardiovascular complications.
Main Methods:
- Retrospective review of autopsy data from patients with severe (>=75% involvement of >=3 major bones) and moderate (>=75% involvement of 1-2 major bones) Paget's disease.
- Comparison of AS prevalence in Paget's disease cases with age-matched normal controls.
- Analysis of clinical signs of AS and electrocardiogram (ECG) findings (atrioventricular block, bundle branch block, left ventricular hypertrophy) in Paget's disease patients and controls.
Main Results:
- Aortic stenosis (AS) was found in 24% of severe Paget's disease autopsies versus 3.5% in controls (p<0.01).
- Clinical AS signs were present in 39% of severe Paget's patients versus 4% in controls (p<0.101).
- No increased AS prevalence was observed in moderate Paget's disease.
- Significant increases in complete atrioventricular (AV) block (11% vs 2.5%), incomplete AV block (11% vs 1.3%), bundle branch block (20% vs 2.5%), and left ventricular hypertrophy (13% vs 3.8%) were noted in severe Paget's cases compared to controls (all p<0.05).
Conclusions:
- Severe Paget's disease is associated with a significantly higher prevalence of aortic stenosis (AS).
- Patients with severe Paget's disease exhibit increased rates of cardiac conduction abnormalities, including AV block and bundle branch block.
- These cardiovascular complications are not observed in moderate forms of Paget's disease, suggesting a link between disease severity and cardiac involvement.
Abstract:
The prevalence of calcific aortic valve stenosis in Paget's disease (osteitis deformans) was investigated by reviewing autopsy data of severe cases (> or = 75% involvement of > or = 3 major bones, the femur, tibia, skull, and pelvis) and moderate cases (> or = 75% involvement of only 1 or 2 major bones) of Paget's disease. Comparisons were made with normal age-matched controls. Aortic stenosis (AS) was present in 24% of 27 autopsies of severe Paget's disease compared with 3.5% in 201 controls (p <0.01). Clinical signs of AS were present in 39% of 102 patients with severe Paget's disease compared with 4% in 417 controls (p <0.101). The prevalence of AS in 18 cases of moderate Paget's disease was similar to that of controls. Electrocardiograms were reviewed in 45 cases of Paget's disease and compared with 80 controls of similar age. Complete atrioventricular (AV) block, incomplete AV block, bundle branch block, and left ventricular hypertrophy were present in 11%, 11%, 20%, and 13% of the Paget's cases and in only 2.5%, 1.3%, 2.5%, and 3.8% in the control cases (p <0.05, <0.05, <0.01, and <0.05, respectively). It is concluded that in severe Paget's disease there is a high prevalence of AS, heart block, and bundle branch block, but these are not present in moderate degrees of bone involvement.
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