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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • History of dyspnea, atypical chest pain, or intermittent cyanosis

  • Palpitations associated with supraventricular arrhythmias and preexcitation syndrome (Wolff-Parkinson-White [WPW])

  • Right parasternal lift, widely split S1, systolic clicks, and systolic murmur of tricuspid regurgitation (without inspiratory accentuation)

  • Right atrial enlargement, right bundle branch block, posteriorly directed delta waves with accessory pathway by ECG; frequent first-degree atrioventricular block

  • Normal or reduced pulmonary vascularity without pulmonary artery enlargement, right atrial enlargement, normal left-sided cardiac silhouette on chest radiograph

  • Apical displacement of septal tricuspid valve leaflet, variable degrees of tricuspid regurgitation originating from apical portion of right ventricle, and enlarged right atrium on echocardiography

GENERAL CONSIDERATIONS

  • Ebstein’s anomaly is characterized by deformity of the tricuspid valve with apical displacement of the septal and posterior leaflets

  • A variable degree of tricuspid regurgitation is present and is dependent on the extent of apical displacement and malcoaptation of the tricuspid leaflets

  • Right ventricular pump function may be inadequate if the proportion of right ventricle proximal to the leaflets (atrialized right ventricle) is substantial and the remaining right ventricle is inadequate

  • Cyanosis may be present as a result of right-to-left shunting across a patent foramen ovale in the presence of significant tricuspid regurgitation or elevated right atrial pressures

  • Atrial septal defect is the most common associated anomaly

  • Twenty-five percent to 30% of patients have an accessory pathway with preexcitation (WPW) and a tendency for supraventricular tachyarrhythmias

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Tremendous variability in clinical presentation exists, depending on the morphology and physiologic consequences of the tricuspid valve disorder and associated anomalies

  • Dyspnea

  • Arrhythmias, including WPW syndrome

  • Decreased exercise tolerance

PHYSICAL EXAM FINDINGS

  • Variable cyanosis

  • Right parasternal lift

  • Widely split S1

  • Systolic clicks from delayed tricuspid valve closure

  • Systolic murmur of tricuspid regurgitation that does not increase in intensity during inspiration owing to the inability of the right ventricle to accept an increase in venous return

  • S3 and S4 gallops may be present

  • An early diastolic snap may be present from the opening of an elongated anterior tricuspid leaflet

DIFFERENTIAL DIAGNOSIS

  • Other causes of primary and secondary tricuspid regurgitation

  • Other causes of exertional dyspnea, chest pressure, and intermittent cyanosis

  • Other causes of supraventricular arrhythmias

  • Other causes of congestive heart failure

DIAGNOSTIC EVALUATION

ELECTROCARDIOGRAPHY

  • Right atrial enlargement

  • Right bundle branch block

  • PR interval may be prolonged, except in the presence of an accessory pathway when the PR interval is shortened

  • WPW syndrome

  • Atrial fibrillation may be present in older patients

IMAGING STUDIES

  • Chest x-ray:

    • – Normal or reduced pulmonary vasculature without pulmonary artery enlargement

    • – Right atrial enlargement, normal/small left atrium and left ventricle size

  • Echocardiography:

    • – Elongated ...

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