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European Heart Journal 1988 9(7):811-818;
Copyright © 1988 by the European Society of Cardiology.
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© 1988 The European Society of Cardiology

Value of radionuclide assessment with thallium 201 scintigraphy in carnitine deficiency cardiomyopathy

F. TAILLARD*, O. MUNDLER{dagger}, I. TILLOUS-BORDE{ddagger}, J.-C. DESBOIS* and M. PATURENEAU-JOUAS§

*Service de Pédiatrie Hôpital Hérold 7, Place Rhin et Danube 75935 Paris Cedex 19
{dagger}Service central de Biophysique et de Médecine Nucléaire, Hôpital Lariboisière 75010 Paris
{ddagger}Laboratoire, d'Echocardiographie, Clinique de Pédiatrie, H{circumflex}pital Trousseau 75571 Paris Cedex 12
§Laboratoire de Neurochimie, INSERM U 134, Hôpital de la Salpêtrière 75651 Paris Cedex 13, France

Received 27 May 1987; revised 20 November 1987; .

Abstract

The case of a 30 month-old boy who presented with isolated severe dilated cardiomyopathy is reported. The diagnosis of systemic carnitine deficiency was confirmed by low serum and tissue carnitine levels. During oral L-carnitine therapy, dramatic improvement of the cardiac function was assessed by radionuclide methods. Myocardial thallium 201 uptake was closely correlated with cardiac function studied by angiosintigraphy. These methods are simple, easily reproducible, non-ivasive and involve little radiation. In a case of cardiomyopathy, we suggest an immediate trial of oral carnitine treatment; the efficacy of the therapy can be confirmed by isotopic tests with thallium 201 scintigraphy.

Key Words: Cardiomyopathy • carnitine deficiency • thallium 201 scintigraphy.


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