Skip to Main Content

KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Prominent left parasternal impulse, soft S1, loud single S2 along the upper left sternal border

  • Absence of left-sided aortic knob on chest x-ray

  • Ventricular inversion (ie, ventriculoarterial and ventriculoatrial discordance) apparent on cardiac imaging

  • Atria usually in normal position

GENERAL CONSIDERATIONS

  • Rare condition with male predominance

  • Usually associated with other abnormalities such as:

    • – Ventriculoseptal defects

    • – Pulmonic stenosis

    • – Tricuspid valve abnormalities

  • When a ventricular septal defect is present, there is a risk of developing pulmonary vascular disease in the absence of pulmonary stenosis because of increased pulmonary blood flow

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • May be asymptomatic until the third or fourth decade of life

  • Patients who develop systemic ventricular failure may experience fatigue, dyspnea, orthopnea, paroxysmal nocturnal dyspnea, and edema

  • Patients with heart block or tachyarrhythmias may have palpitations and/or syncopal episodes

PHYSICAL EXAM FINDINGS

  • Prominent right ventricular impulse

  • Loud single S2 along the upper left sternal border

  • Murmurs caused by associated defects (ventricular septal defect, pulmonic stenosis)

  • Adults who develop systemic right ventricular failure may have a gallop and/or pulmonary rales

DIFFERENTIAL DIAGNOSIS

  • Other causes of complete heart block

  • Other causes of heart failure

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • No specific tests

ELECTROCARDIOGRAPHY

  • Right atrial enlargement

  • P-R prolongation

  • Variable degrees of atrioventricular (AV) block

  • Right ventricular hypertrophy

  • Right-axis deviation

  • Q waves in the right precordial leads

IMAGING STUDIES

  • Chest x-ray findings:

    • – Absence of left-sided aortic knob

    • – Prominent right atrium and right ventricle

  • Echocardiography:

    • – Atria are in the normal position with the morphologic left ventricle situated between the right atrium and pulmonary artery, and the morphologic right ventricle between the left atrium and aorta

    • – The systemic right ventricle is hypertrophied and may be dilated with reduced contractile function

    • – Associated abnormalities may be present, such as a ventriculoseptal defect, abnormalities of the tricuspid valve with variable degrees of tricuspid regurgitation, and/or pulmonary valve stenosis

DIAGNOSTIC PROCEDURES

  • In the adult with associated ventricular septal defect, cardiac catheterization may be indicated to determine pulmonary vascular resistance or severity of pulmonic stenosis

TREATMENT

CARDIOLOGY REFERRAL

  • All patients with transposition of the great arteries should be followed up regularly by a cardiologist who specializes in congenital heart disease

HOSPITALIZATION CRITERIA

  • Atrial or ventricular tachyarrhythmias

  • Symptomatic bradycardia

  • High-grade AV block

  • Heart failure

MEDICATIONS

  • Endocarditis prophylaxis for patients with a ventriculoseptal defect or valvular abnormalities

  • Drugs to control atrial tachyarrhythmias

  • Appropriate ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.