Mitral and tricuspid valve repair in hypereosinophilic syndrome

Abstract

Hypereosinophilic syndrome (HES) is a rare systemic condition, defined as a persistently elevated eosinophil count associated with end organ damage and the absence of a primary cause. Cardiac involvement occurs in about 50% of patients with HES. Myocardial infiltration results in endomyocardial fibrosis, valve dysfunction and mural thrombus. The atrioventricular valves are almost always involved, resulting in regurgitation due to leaflet restriction, most commonly affecting the posterior mitral valve leaflet. Surgical management remains challenging in patients with HES with limited data on the choice of valve surgery. We describe the case of a 17-year-old woman with HES complicated by congestive cardiac failure secondary to severe mitral and tricuspid regurgitation. Because of refractory heart failure despite medical therapy, surgical mitral and tricuspid valve repair was performed, and an excellent 24-month outcome was achieved. We believe this is the first report of double valve repair in this rare condition.

Keywords: heart failure.

Figure 1

Preoperative transthoracic echocardiographic images showing mitral valve thickening and restriction, with encasement of the posterior mitral leaflet and severe mitral regurgitation (A and C). Restricted tricuspid valve with annular dilatation and severe regurgitation (B and D).

Figure 2

Postoperative transthoracic echocardiographic images of the mitral valve repair. An annuloplasty ring is in situ and there is improved coaptation of the anterior mitral leaflet with the augmented posterior leaflet (A). No mitral regurgitation is apparent on colour flow Doppler (B).