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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

FOLLOW-UP

  • As appropriate for specific cardiac problem

COMPLICATIONS

  • Endocarditis

  • Heart failure

  • Pericardial tamponade

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