Can Paget's Disease Cause Congestive Heart Failure?
Yes, Paget's disease can cause congestive heart failure, though this is an uncommon complication occurring in approximately 3% of patients, typically only in those with extensive polyostotic disease and high bone turnover. 1, 2
Mechanism of Heart Failure in Paget's Disease
Paget's disease causes heart failure through a high-output state rather than primary cardiac dysfunction. 1, 3
Pathophysiology
The extensive vascular proliferation in pagetic bone creates multiple small arteriovenous shunts throughout the skeleton, functioning similarly to large arteriovenous fistulas. 1, 3
These shunts cause decreased systemic vascular resistance, which threatens arterial blood pressure and triggers neurohormonal activation (renin-angiotensin-aldosterone system and sympathetic nervous system). 3
The compensatory response leads to salt and water retention by the kidneys, resulting in volume overload and the clinical syndrome of congestive heart failure despite normal or elevated cardiac output. 3
The heart itself is structurally normal in pure high-output failure from Paget's disease—it is capable of generating very high cardiac output but becomes overwhelmed by the chronic volume load. 3
Clinical Context and Risk Factors
When CHF Develops
Heart failure from Paget's disease occurs primarily when skeletal involvement is extensive (polyostotic disease) and disease activity is high, as measured by elevated alkaline phosphatase and urinary hydroxyproline. 2, 4
The degree of peripheral vascular resistance reduction correlates with both urinary hydroxyproline excretion (a marker of bone turnover) and patient age. 4
In early or limited Paget's disease, there is only a trend toward reduced peripheral vascular resistance without clinical heart failure. 4
Prevalence
Among unselected community patients with Paget's disease, congestive heart failure attributable to the bone disease occurs in only 3.0% of cases. 2
This is far less common than other complications such as osteoarthritis (73%), hearing loss (61%), or fractures of pagetic bone (9.7%). 2
Subclinical Cardiac Changes
Recent advanced echocardiographic studies reveal that even without overt heart failure, patients with Paget's disease show subclinical myocardial abnormalities:
- Increased left ventricular mass index, interventricular septum thickness, and relative wall thickness. 5
- Reduced left ventricular ejection fraction, stroke volume, and cardiac output compared to matched controls. 5
- Impaired global longitudinal strain and myocardial work efficiency on speckle-tracking echocardiography. 5
- Elevated E/e' ratio suggesting diastolic dysfunction. 5
- Higher prevalence of valvular calcifications and sclerosis. 5
These findings suggest a "pagetic heart disease" may exist as a distinct entity beyond simple high-output failure. 5
Critical Clinical Pitfall
Do not use vasodilators as first-line therapy for heart failure in Paget's disease. 3
Because the underlying problem is decreased peripheral vascular resistance (not increased afterload as in typical heart failure), vasodilator therapy may paradoxically worsen hypotension and neurohormonal activation. 3
The appropriate treatment strategy is to treat the underlying Paget's disease with antiresorptive therapy (bisphosphonates like zoledronic acid or calcitonin), which reduces bone turnover, decreases the arteriovenous shunting, and can reverse the high-output state. 6, 5
Congestive heart failure is listed as one of the less frequent but definite indications for treating Paget's disease with antiresorptive medications. 6
Treatment Approach
When CHF is present in a patient with Paget's disease:
Confirm the diagnosis by documenting high cardiac output with decreased systemic vascular resistance and extensive active Paget's disease (elevated alkaline phosphatase, multiple bone sites involved). 4
Initiate antiresorptive therapy with bisphosphonates (zoledronic acid preferred) or calcitonin to reduce bone turnover and arteriovenous shunting. 6, 5
Manage volume overload with diuretics as needed for symptomatic relief. 3
Avoid vasodilators unless there is coexisting hypertension or other clear indication, as they may worsen the hemodynamic state. 3
Address other cardiac risk factors, as many patients have coexisting conditions (hypertension, valvular disease) that may contribute independently to heart failure. 5