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Enlargement of cardiac rhabdomyoma and myocardial ischaemia during corticotropin treatment for infantile spasm

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Multiple large cardiac tumours were detected by fetal echocardiography (panel A); tuberous sclerosis was also diagnosed. During the first 3 months of the infant girl's life these tumours in the left and right ventricles decreased to less than half their circumference at birth (panel B). However, the patient developed infantile spasms at the age of 4 months and corticotropin treatment was initiated (once a day for two weeks, tapering off over three months). At 6 month of age a heart murmur was detected in the patient. Echocardiography showed pronounced enlargement of the tumours in both ventricles and a small tumour extending from the upper portion of the interventricular septum into the left ventricular outflow tract (panel C), with a 30 mm Hg gradient by Doppler evaluation. An ECG at the time showed 2–3 mm horizontal ST segment depressions in leads I, aVL, and V4–V6. ECG gated single photon emission computed tomography (using 99mTc-tetrofosmin) revealed low perfusion at the lateral and inferior regions of the left ventricle, indicating myocardial ischaemia. Corticotropin treatment was stopped. Three months later, the patient was asymptomatic. An echocardiogram (panel D) showed the tumours had reduced in size, and there was a concomitant improvement in the depressed ST segment of the ECG.