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+A 23-year-old woman with a history of carnitine deficiency presented with exertional dyspnea and fatigue.
+She was first diagnosed with nonischemic cardiomyopathy at age 10 years, when she presented with symptoms of congestive heart failure and transthoracic echocardiography (TTE) revealed severe left ventricular (LV) dysfunction.
+At that time, her total carnitine level was <3 μmol/L (normal range, 25–69 μmol/L), and her free carnitine level was <3 μmol/L (normal range, 16–60 μmol/L).
+The cardiomyopathy was attributed to carnitine deficiency.
+After 5 months of therapy with carnitine supplements, her total carnitine and free carnitine levels had increased to 86 μmol/L and 48 μmol/L, respectively, and her left ventricular systolic function had normalized, with fractional shortening of 34.7%.
+At the current admission, the patient reported that, over the past 10 years, she had only been intermittently compliant with her carnitine supplement regimen, and that, approximately 6 months before presentation, her total carnitine and free carnitine levels had dropped to 18 μmol/L and 17 μmol/L, respectively.
+She underwent TTE followed by cardiac magnetic resonance (CMR) with gadolinium enhancement.
+The TTE showed normal LV function and mild, concentric LV hypertrophy (Fig.1).
+Results of CMR confirmed normal LV size and function but showed focal increased wall thickness at the basal and mid lateral wall (end-diastolic thickness, 1.6 cm) (Fig.2) with patchy delayed gadolinium enhancement (Fig.3).
+On the basis of the patient's family history of sudden cardiac death, abnormal myocardial structure, and underlying disease process, a dual-chamber implantable cardioverter-defibrillator was placed for primary prevention of sudden cardiac death from both bradyarrhythmias and tachyarrhythmias.