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

H N Hultgren1

  • 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.

Related Concept Videos

Osteoclasts in Bone Remodeling01:31

Osteoclasts in Bone Remodeling

Osteoclasts are cells responsible for bone resorption and remodeling. They originate from hematopoietic progenitor cells present in the bone marrow. Numerous progenitor cells fuse to form multinucleated cells, each with 10-20 nuclei. A single osteoclast has a diameter of 150 to 200 µM. These cells have ruffled borders that break down the underlying bone tissue and release minerals such as calcium into the blood in bone resorption. Osteoclasts cling to bones with their ruffled edges during bone...
Bone Disorders01:29

Bone Disorders

Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
Mitral Valve Prolapse I: Introduction01:27

Mitral Valve Prolapse I: Introduction

IntroductionThe mitral valve, one of the heart's four valves, regulates blood flow. These valves have flaps that open and close to direct blood properly through the heart and body. During each heartbeat, the flaps open for blood to pass through and seal shut to prevent backflow. Specifically, the mitral valve opens to allow blood flow from the heart's upper left chamber to the lower left chamber. It then closes securely as the lower left chamber contracts to pump blood to the body, preventing...
Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...