Mixed Connective Tissue Disease and the Heart

KEY FEATURES

ESSENTIALS OF DIAGNOSIS

• Raynaud’s phenomenon, sclerodactyly

• Myopathy with high titers of ribonucleoprotein antibodies

• Pericarditis, pulmonary hypertension

GENERAL CONSIDERATIONS

• Patients with mixed connective tissue disease have clinical features that resemble other connective tissue diseases

• Characteristically, affected patients have high titers of antibodies to ribonucleoprotein and speckled antinuclear antibodies

• Rheumatoid agglutinins occur in about 50%

• Predominantly, females of all ages are affected

• Cardiac involvement is infrequent and usually related to pericarditis or pulmonary hypertension

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

• Chest pain characteristic of pericarditis is common

• Symptoms of heart failure

  • – Fatigue

  • – Dyspnea

  • – Edema

PHYSICAL EXAM FINDINGS

• Pulmonary hypertension

  • – Right ventricular (RV) lift

  • – Loud P₂

  • – Edema

• Pericarditis

  • – Friction rub

  • – Elevated jugular venous pressure (JVP)

DIFFERENTIAL DIAGNOSIS

• Other connective tissue diseases

• Other causes of cardiac disease

DIAGNOSTIC EVALUATION

LABORATORY TESTS

• Antinuclear antibody titer, ribonucleoprotein level, rheumatoid factor

• Brain natriuretic peptide

ELECTROCARDIOGRAPHY

• RV and right atrial hypertrophy

• Diffuse ST elevation of pericarditis

• Supraventricular arrhythmias

IMAGING STUDIES

• Echocardiography:

  • – Pericardial effusion

  • – Right heart chamber enlargement

  • – Verrucous mitral valve thickening

• Doppler echocardiography:

  • – Elevated pulmonary pressure estimates

  • – Mitral regurgitation

• Chest x-ray: enlarged cardiac silhouette

DIAGNOSTIC PROCEDURES

• Cardiac catheterization: may be required in some cases to evaluate possible coronary artery disease or cardiac tamponade

TREATMENT

CARDIOLOGY REFERRAL

• Suspected cardiac disease

HOSPITALIZATION CRITERIA

• Heart failure

• Pericarditis

• Rapid atrial fibrillation

MEDICATIONS

• Specific anti-inflammatory pharmacotherapy

• Appropriate therapy for pulmonary hypertension

• Steroids for pericarditis, eg, prednisone 5–50 mg PO daily

THERAPEUTIC PROCEDURES

• Pericardiocentesis if tamponade

MONITORING

• ECG in hospital as appropriate

• Blood pressure and JVP with pericarditis

• Pulmonary artery pressures during drug treatment of pulmonary hypertension

DIET AND ACTIVITY

• Low-sodium diet

• Restricted activity if heart failure

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

• Resolution of problem

FOLLOW-UP

• Cardiology follow-up as appropriate to problem

COMPLICATIONS

• Atrial and ventricular arrhythmias

• Myocarditis

• Cardiac conduction disturbances

PROGNOSIS

• Mortality is 13% at 10 years

RESOURCES

PRACTICE GUIDELINES