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Published on: May 13, 2019
[Ebstein's anomaly associated with left ventricular dysfunction: a case report]
Insights
This case study highlights a 66-year-old man with Ebstein's anomaly, presenting with left ventricular dysfunction and heart failure symptoms. Diffuse fibrosis is suggested as a potential cause for his depressed systolic function.
Area of Science:
- Cardiology
- Cardiovascular Medicine
- Congenital Heart Disease
Background:
- Ebstein's anomaly is a rare congenital heart condition affecting the tricuspid valve.
- Patients may develop progressive heart failure and arrhythmias over time.
- Left ventricular dysfunction in Ebstein's anomaly is less common but can significantly impact prognosis.
Observation:
- A 66-year-old male with known Ebstein's anomaly presented with symptoms of heart failure, including lower extremity edema and orthopnea.
- Physical examination revealed signs of right-sided heart strain and atrial fibrillation.
- Echocardiography demonstrated significant tricuspid valve abnormalities, atrial and ventricular dilation, and paradoxical septal motion.
Findings:
- Left ventriculography revealed diffuse hypokinesis with a reduced ejection fraction of 49%.
- Transesophageal echocardiography showed a hypoplastic tricuspid valve with moderate regurgitation.
- Coronary arteries were normal, ruling out ischemic heart disease.
Implications:
- Diffuse myocardial fibrosis is proposed as a potential mechanism for the observed left ventricular systolic dysfunction in this patient.
- This case underscores the importance of considering ventricular dysfunction in the long-term management of Ebstein's anomaly.
- Further research into the pathophysiology of ventricular dysfunction in Ebstein's anomaly is warranted.
Abstract:
A 66-year-old man presented with Ebstein's anomaly associated with left ventricular dysfunction. He had been followed since 40 years of age for cardiomegaly and arrhythmia, and experienced episodes of orthopnea at the age of 64. He was referred to our hospital in April 1997 because of lower extremity edema. Physical examination revealed dilated external jugular vein, tenderness of the right hypocondorium, and lower extremity edema. Electrocardiography confirmed atrial fibrillation. Transthoracic echocardiography revealed bilateral atrial and ventricular dilation, and paradoxical septal movement. The apical four-chamber view demonstrated 15 mm apical displacement of the septal leaflet. Color Doppler echocardiography revealed moderate tricuspid regurgitation. Transesophageal echocardiography revealed low echoic and hypoplastic tricuspid valve. Left ventriculography showed diffuse hypokinesis, and the ejection fraction was 49%. The coronary artery was normal. Atrial septal defect was not detected. Diffuse fibrosis, which may be found in the hearts of patients with Ebstein's anomaly at autopsy may have been responsible for the left ventricular depressed systolic function in this patient.
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