Young Adult With Congenital Heart Disease Presenting With Anasarca
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 One year previously, a degenerated Carpentier-Edwards bioprosthesis had been replaced by a Bicarbon mechanical valve (Sorin Biomedica, Saluggia, Italy).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 α1–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.
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).
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.
Source of Funding
Dr Paelinck was supported by a grant from the Fund for Scientific Research Flanders, Brussels, Belgium.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/118/12/1304/DC1.