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(Circulation. 2009;120:e1-e2.)
© 2009 American Heart Association, Inc.

Images in Cardiovascular Medicine

Uncommon Variant of Ebstein Anomaly With Tricuspid Stenosis

Niloufar Tabatabaei, MD; Poomiporn Katanyuwong, MD; Jerome F. Breen, MD; James Glockner, MD, PhD; Joseph A. Dearani, MD; Rami N. Khouzam, MD; Naser M. Ammash, MD

From the Divisions of Cardiovascular Disease (N.T., N.M.A.), Pediatric Cardiology (P.K.), Vascular and Interventional Radiology (J.F.B., J.G.), and Cardiovascular Surgery (J.A.D.), Mayo Clinic, St. Mary’s Hospital, Rochester, Minn; and Farmington Heart Center, Farmington, Minn (R.N.K.).

Correspondence to Nasser M. Ammash, MD, FACC, Associate Professor of Medicine, Mayo Clinic, St. Mary’s Hospital, 2nd St SW GO-138SE, Rochester, MN 55902. E-mail ammash.naser@mayo.edu

An extract of the first 250 words of the full text is provided, because this article has no abstract.

A 27-year-old previously healthy woman with no prior history of exercise limitations, shortness of breath, cyanosis, or arrhythmia became short of breath 10 months after her second successful pregnancy. Her shortness of breath was at rest accompanied by progressive edema, ascites, and later anasarca. An ECG revealed normal sinus rhythm with right atrial enlargement (Figure 1A), and chest x-ray revealed normal lung parenchyma with mildly enlarged cardiac silhouette (Figure 1B). A transthoracic echocardiogram demonstrated reduced left ventricular size with an ejection fraction estimated at 50%, severe right atrial enlargement, and severe right ventricular enlargement with dysfunction and was suspicious for severe displacement of the tricuspid valve leaflets toward the right ventricular apex, suggestive of Ebstein anomaly (EA) (Figure 2). She responded to intravenous diuresis with improvement in her right-sided heart failure. In light of her dilated, poorly functioning right ventricle, she was started on Coumadin. Repeat transthoracic echocardiogram 2 months later demonstrated displacement of the tricuspid valve into the right ventricular outflow tract with associated tricuspid stenosis (Figure 3, online-only Data Supplement Movie IA). The spectral Doppler analysis demonstrated a mean gradient of 4 to 5 mm Hg. Cardiac magnetic resonance imaging performed to assess right ventricular function demonstrated tricuspid valve displacement into the right ventricular outflow tract below the pulmonary valve with extensive mural thrombus/mass in the right ventricular apex extending into the right ventricular outflow tract (Figure 4, online-only Data Supplement Movie IIA and IIB).

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