Ankylosing Spondylitis and the Heart

KEY FEATURES

ESSENTIALS OF DIAGNOSIS

• Characteristic lumbar spine and sacroiliac arthritis

• Positive HLA-B27 assay

• Aortic root sclerosis and dilation, aortic valve leaflet thickening, and subaortic bump on echocardiography

• Aortic valve regurgitation

GENERAL CONSIDERATIONS

• Ankylosing spondylitis is an inflammatory disease that mainly affects the vertebral and sacroiliac joints

• Less frequently, it affects other organs, including the heart

• Its incidence is 1 per 2000 people and occurs predominantly in white males < 40 years old

• Cardiac involvement usually follows articular manifestations by 1–2 decades

• The most common cardiovascular manifestations are:

  • – Aortitis with or without aortic valve regurgitation

  • – Conduction disturbances

  • – Mitral regurgitation

  • – Myocardial dysfunction

  • – Pericardial disease

• Aortitis usually occurs in the proximal ascending aorta, frequently involving the sinuses of Valsalva and resulting in dilatation and thickening of the aortic root and annulus; the resulting leaflet retraction leads to mild to moderate aortic regurgitation

• Extension of this process into the anterior mitral valve leaflet may result in mitral regurgitation

• Extension of aortic fibrosis into the basal septum may interfere with the conduction system

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

• Usually asymptomatic

• Dyspnea can occur

• Syncope can occur

PHYSICAL EXAM FINDINGS

• Typical decrescendo diastolic murmur of aortic regurgitation

• Holosystolic murmur of mitral regurgitation

• Bradycardia

DIFFERENTIAL DIAGNOSIS

• Other causes of aortic valve disease

• Other connective tissue diseases

DIAGNOSTIC EVALUATION

LABORATORY TESTS

• Positive test result for histocompatibility antigen HLA-B27

ELECTROCARDIOGRAPHY

• High-degree atrioventricular (AV) block can occur

IMAGING STUDIES

• Echocardiography findings:

  • – Thickened, dilated aortic root with characteristic extension into the proximal anterior mitral leaflet (subaortic bump)

  • – Aortic valve leaflet nodules

• Doppler echocardiography findings:

  • – Color-flow Doppler evidence of aortic and mitral regurgitation

TREATMENT

CARDIOLOGY REFERRAL

• Cardiac symptoms

• Significant heart murmur

HOSPITALIZATION CRITERIA

• Heart failure

• Pericarditis

• Syncope

• High-degree AV block with bradycardia

MEDICATIONS

• Specific anti-inflammatory pharmacotherapy

• Heart failure medications as appropriate

• Medications to control blood pressure as appropriate

THERAPEUTIC PROCEDURES

• Pacemaker

SURGERY

• Rarely valve replacement

MONITORING

• ECG monitoring in hospital as appropriate

DIET AND ACTIVITY

• Diet and activity guidelines as appropriate for the cardiac condition

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

• Resolution of problem

• Successful pacemaker placement

• Successful surgery