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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Infant with cyanosis or congestive heart failure, depending on the size of the interatrial communication and whether the pulmonary venous channel is obstructed

  • Hyperdynamic right ventricular impulse with wide fixed split S2 and an S3; systolic ejection murmur along the left sternal border and a diastolic flow rumble across the tricuspid valve

  • Right-heart chamber enlargement on ECG

  • Right-heart enlargement and pulmonary vascular engorgement on chest x-ray

GENERAL CONSIDERATIONS

  • Congenital anomaly in which all pulmonary veins drain into the right atrium

  • Condition is usually incompatible with life unless a large atrial septal defect (ASD), patent foramen ovale, patent ductus arteriosus, or ventricular septal defect exists

  • Severity depends on whether the pulmonary veins are obstructed

  • Pulmonary veins may drain into the superior vena cava (most common), right atrium, coronary sinus, portal vein, of sinus venosus, or they may have multiple connections

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Infant with cyanosis, lethargy, poor feeding, tachypnea, frequent respiratory infections

PHYSICAL EXAM FINDINGS

  • Cyanosis

  • Hyperdynamic right ventricular impulse

  • Wide fixed split S2 and S3

  • Systolic ejection murmur along the left sternal border

  • Diastolic and loud rumble across the tricuspid valve

DIFFERENTIAL DIAGNOSIS

  • ASD

  • Pentalogy of Fallot (tetralogy plus ASD)

  • Other causes of cyanosis and heart failure

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • No specific tests

ELECTROCARDIOGRAPHY

  • Right atrial enlargement

  • Right ventricular enlargement

IMAGING STUDIES

  • Chest x-ray:

    • – Right atrial and ventricular enlargement

    • – Pulmonary vascular engorgement

  • Echocardiography:

    • – Right heart enlargement

    • – Absence of pulmonary venous flow into the left atrium

    • – Associated lesions such as an atrial septal defect or ventricular septal defect may be visualized

  • Transesophageal echocardiography may disclose the site of anomalous drainage of all 4 pulmonary veins leading to right-sided structures

DIAGNOSTIC PROCEDURES

  • Cardiac catheterization:

    • – Oxygen saturation step-up usually in the superior vena cava

    • – Contrast dye reveals the pulmonary veins draining into the right-sided structures instead of the left atrium

TREATMENT

CARDIOLOGY REFERRAL

  • Infants are often cyanotic and seen by a cardiologist immediately

HOSPITALIZATION CRITERIA

  • Infants are often cyanotic and critically ill in the hospital after birth

MEDICATIONS

  • Aggressive medical therapy for palliation

THERAPEUTIC PROCEDURES

  • Balloon atrial septostomy for palliation

SURGERY

  • Early complete surgical repair with rerouting of all 4 pulmonary veins into the left atrium is recommended

MONITORING

  • ECG monitoring, ...

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