A long-term follow-up of a girl with dilated cardiomyopathy after mitral valve replacement and septal anterior ventricular exclusion

Dilated cardiomyopathy (DCM) is one of the most serious prognostic factors in heart disease [1,2]. Batista et al. described left ventriculectomy in 1996 which has become one of the most important surgical therapies for adults with DCM [3-6]. However, in patients with both damaged intraventricular septum (IVS) and damaged left ventricular (LV) free wall, cardiac function worsens following this procedure. The Dor procedure and Septal Anterior Ventricular Exclusion (SAVE) procedures have recently been recommended in these patients [7-9].In November 2001, a 10 year, 11 month old girl was admitted to our hospital with dyspnea on mild exertion and pretibial and palpebral edema.At 2 months, a heart murmur was detected. One year later, she was diagnosed with congenital mitral valve stenosis (MS) and mitral valve regurgitation (MR) by cardiac echogram and catheterization. Despite treatment with digitoxin and diuretics, her left ventricular end-diastolic diameter (LVDd) gradually increased and MR worsened. She received mitral valve replacement (MVR) at age 6, but her cardiac function continued to worsen and her LVDd increased despite of 9 years optimal medical treatment.At the time of her hospitalization, a chest X-ray revealed pulmonary congestion and cardiomegaly (cardio-thoracic ratio 79.0%). Echocardiogram showed dilated LVDd, of 71.5 mm (188% of normal), reduced left ventricular fractional shortening (LVFS) (7.6%) and closure of one of the artificial mechanical valves. Left ventricular ejection fraction (LVEF) was also measured by cardiac catheterization, and the LVEF was 11.0% at this time. Serum BNP was elevated at 2217.5 pg/ml. Decreased up-takes of 201Tl and 123I-MIBG were detected in the anterior IVS and anterior LV wall by cardiac scintigraphy (Figure 1). A cardiac muscle biopsy revealed fibrous and vacuolar degeneration in the IVS area (Figure 2). Both the left and right coronary arteries were normal and there was no evidence of ischemic cardiomyopathy by an angio