Circulation. 2008;118:1304-1305
doi: 10.1161/CIRCULATIONAHA.108.767632
(Circulation. 2008;118:1304-1305.)
© 2008 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Young Adult With Congenital Heart Disease Presenting With Anasarca
Riet Dierckx, MD;
Steven E. Haine, MD;
Christiaan J. Vrints, MD, PhD;
Bernard P. Paelinck, MD, PhD
From the Department of Cardiology, University Hospital Antwerp, Edegem, Belgium.
Correspondence to Bernard P. Paelinck, Department of Cardiology, University Hospital Antwerp, Wilrijkstraat 10, 2650 Edegem, Belgium. E-mail Bernard.Paelinck{at}uza.be
A 28-year-old man, diagnosed at birth with Ebstein’s anomaly, was admitted because of generalized edema. He had a history of successive tricuspid valve replacements. One year previously, a degenerated Carpentier-Edwards bioprosthesis had been replaced by a Bicarbon mechanical valve (Sorin Biomedica, Saluggia, Italy). Metallic heart sounds were absent. In addition to manifest peripheral edema, he had hepatomegaly and prominent jugular veins. He had severe hypoproteinemia (2.8 g/dL, normal 6.3–8.6 g/dL) and hypoalbuminemia (1.3 g/dL, normal 3.7–5.6 g/dL). Thorax radiograph demonstrated an enlarged right atrium, a hump-shaped infundibulum, and epicardial pacemaker leads (Figure 1). ECG showed atrial pacing and intraventicular conduction delay (Figure 2). The echocardiogram revealed the presence of poor right ventricular function and immobile prosthetic tricuspid valve leaflets. These findings were confirmed by transesophageal echocardiography (Loop 1). Fecal 
1–antitrypsin clearance was 128 mL/d (normal <12 mL/d), indicating protein-losing gastroenteropathy. Coumadin had been therapeutic (international normalized ratio >3), except for about 2 weeks (international normalized ratio 2.7) some months previously.
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Figure 1. Chest radiograph showing enlarged right atrium, hump-shaped infundibulum (arrow), and epicardial leads. Note Bicarbon mechanical valve in tricuspid position.
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Figure 2. Surface ECG demonstrates atrial pacing, extremely delayed intraventricular conduction with right bundle-branch block morphology, and marked repolarization abnormalities.
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The patient was treated with thrombolytic therapy (15-mg bolus and 50 mg over 90 minutes, followed by 20 mg per hour up to a total of 100-mg recombinant tissue plasminogen activator). This thrombolytic regimen had to be repeated 3 times to obtain complete restoration of valve leaflet mobility (Loop 2). Intravenous heparin was administered while oral anticoagulation was intensified (target international normalized ratio level 3.5 to 4.5) together with 100 mg acetylsalicylic acid. For several days afterward, a gradual rise in serum protein and albumin levels was noted, and, without administration of diuretics, the patient’s edema resolved completely (Figure 3). At 1-year follow-up, prosthetic valve function remained normal (Loop 3).
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Figure 3. Graph plotting total serum protein and albumin together with body weight against time. Total serum protein and albumin rose while body weight decreased after thrombolysis.
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Ebstein’s anomaly is a rare congenital disorder characterized by apical displacement of the tricuspid valve due to adherence of the septal and posterior leaflets to the interventricular septum, resulting in valve insufficiency and partial atrialization of the right ventricle.1 Right-sided mechanical valve placement is hemodynamically unfavorable because of the low flow state.2 Right-sided prosthetic valve thrombosis is often insidious as opposed to left-sided valve thrombosis, which often presents as thromboembolism, pulmonary edema, and/or cardiogenic shock.
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Source of Funding
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Dr Paelinck was supported by a grant from the Fund for Scientific
Research Flanders, Brussels, Belgium.
Disclosures
None.
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Footnotes
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The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/118/12/1304/DC1.
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References
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1. Attenhofer JCH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein’s anomaly.
Circulation. 2007; 115: 277–285.
[Free Full Text]2. Chang BC, Lim SH, Yi G, Hong YS, Lee S, Yoo KJ, Kang MS, Cho BK. Long-term clinical results of tricuspid valve replacement. Ann Thorac Surg. 2006; 81: 1317–1323.[Abstract/Free Full Text]
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References