Abstract
We treated a 10 year 11 month old girl with severe mitral valve regurgitation, stenosis and dilated cardiomyopathy, presented with New York Heart Association (NYHA) functional classification IV. She acutely developed cardiogenic shock with a dyskinetic anterior-septal left ventricle and entered a shock state during our consultation about heart transplantation. Septal-anterior ventricular exclusion and mitral valve replacement were performed emergently. She successfully recovered from cardiogenic shock. Left ventricular end-diastolic diameter and fractional shortening improved from 71.5 mm (188.0% of normal) to 62.5 mm (144.2% of normal) and 7.6% to 18.3% respectively. Furthermore, her serum BNP decreased from 2217.5 pg/ml to 112.0 pg/ml. Her cardiac function has remained stable for 7 years since the procedures were performed.
Highlights
Dilated cardiomyopathy (DCM) is one of the most serious prognostic factors in heart disease [1,2]
Severe heart failure in children is commonly treated with diuretics, ACE inhibitors, calcium blockers, β-blockers and vasodilators [10,11]
Patients with DCM and New York Heart Association (NYHA) functional class, who do not respond to medical therapy, are candidates for heart transplantation
Summary
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 [3,4,5,6]. 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 [7,8,9]
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