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Left heart matters in Ebstein's anomaly: the importance of a closer follow up-a case report

Maria Victoria Ordoñez1, Radwa Bedair1, Stephanie L Curtis1

  • 1Bristol Heart Institute, University Hospitals Bristol & Weston NHS Foundation Trust, Bristol, UK.

European Heart Journal. Case Reports
|September 25, 2020
PubMed

Insights

Ebstein's anomaly (EA) can affect the left ventricle, causing outflow obstruction, especially during exertion. Regular exercise echocardiography is recommended for patients with EA and severe tricuspid regurgitation to detect this acquired dynamic left ventricular outflow obstruction.

Area of Science:

  • Cardiology
  • Congenital Heart Disease
  • Echocardiography

Background:

  • Ebstein's anomaly (EA) is primarily considered a right heart condition.
  • Congenital left-sided cardiac lesions can coexist with EA.
  • Routine investigation of EA patients should include the left heart.

Observation:

  • A 57-year-old woman with severe EA presented with exertional dyspnea and chest pain.
  • Symptoms were attributed to progressive tricuspid regurgitation (TR) and right ventricle dilation.
  • This led to dynamic compression of the left ventricular outflow tract (LVOTO).

Findings:

  • Acquired left ventricular outflow obstruction (LVOTO) can occur in patients with EA.
  • LVOTO secondary to severe TR and right ventricle dilation may be exertional.
  • The obstruction can be silent at rest but significant during physical activity.

Implications:

  • Patients with EA and significant TR require regular monitoring for acquired dynamic LVOTO.
  • Exercise echocardiography is crucial for identifying exertional LVOTO in these patients.
  • Early detection and management of LVOTO can improve patient outcomes.
Abstract

Keywords:
Case reportEbstein’s anomalyLeft ventricular outflow obstruction (LVOTO)Tricuspid regurgitation

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