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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Young adult with dyspnea, recurrent respiratory infections, hemoptysis, and palpitations

  • Physical findings of atrial septal defect (ASD):

    • – Right ventricular heave

    • – Systolic ejection murmur at the left sternal border

    • – Wide fixed split S2 and a diastolic flow rumble over the tricuspid area

  • Incomplete right bundle branch block on ECG

  • Pulmonary vascular congestion, enlarged right heart, and occasionally evidence of the anomalous vein on chest radiograph

  • Cardiac imaging evidence of anomalous pulmonary venous connection and ASD in many patients

GENERAL CONSIDERATIONS

  • This condition is present in approximately 10% of patients with ASD (usually of the sinus venous type)

  • Excessive pulmonary venous return to the right heart over years causes right atrial and ventricular dilation

  • Atrial arrhythmias, right heart failure, and rarely pulmonary hypertension may develop

  • Symptomatic patients or those with a large shunt (Qp/Qs ratio > 2.0) should have surgical repair, including repair of any associated congenital heart defects

  • Surgical repair is contraindicated in patients with an associated ASD who have developed irreversible pulmonary vascular disease

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Dyspnea

  • Signs of right heart failure

  • Recurrent respiratory infections

  • Hemoptysis

  • Palpitations

PHYSICAL EXAM FINDINGS

  • Right ventricular heave

  • Systolic ejection murmur at the left sternal border

  • Wide fixed split S2 consistent with an associated ASD

  • Diastolic flow rumble over the tricuspid valve

DIFFERENTIAL DIAGNOSIS

  • Simple ASD

  • Right heart failure from other causes

  • Pulmonary hypertension from other causes

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • No specific tests

ELECTROCARDIOGRAPHY

  • Incomplete right bundle branch block

  • Right-axis deviation

  • Right ventricular hypertrophy

  • Right atrial enlargement

IMAGING STUDIES

  • Chest x-ray:

    • – Enlarged right atrium and right ventricle

    • – Pulmonary vascular congestion, rarely enlarged pulmonary vein

  • Echocardiography:

    • – Enlarged right atrium and right ventricle, sometimes color-flow Doppler evidence of an ASD

    • – Transesophageal echocardiography can usually demonstrate anomalous drainage of 1 or more pulmonary veins into the right atrium (usually the right upper pulmonary vein)

    • – A sinus venous type ASD may be present at the junction of the superior vena cava and right atrium

DIAGNOSTIC PROCEDURES

  • Cardiac catheterization:

    • – Oxygen saturation step-up in the right atrium (usually the high right atrium). Qp/Qs ratio may be calculated

    • – Pulmonary artery pressures are often elevated owing to increased pulmonary blood flow

    • – Pulmonary vascular resistance may be elevated

TREATMENT

CARDIOLOGY REFERRAL

  • Signs and symptoms of right heart failure

  • Suspected ASD and/or overwhelmingly positive agitated saline study

HOSPITALIZATION CRITERIA

  • Right heart failure

  • Tachyarrhythmias

MEDICATIONS

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