Osteitis deformans (Paget's disease) and calcific disease of the heart valves.

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.