Ventricular outflow tract obstruction by cardiac tumors during ACTH therapy of an infant with tuberous sclerosis
Yoshimi, S.; Osako, Y.; Nishibatake, M.; Kamuro, K.
No to Hattatsu 31(2): 165-170
1999
ISSN/ISBN: 0029-0831 PMID: 10191640 Document Number: 509946
In an infant with tuberous sclerosis and West syndrome, ACTH treatment was interrupted because of augmentation of the biventricular outflow tract obstruction. A 5-month-old boy, who had been diagnosed to have multiple cardiac tumors since in utero, manifested West syndrome with typical hypsarhythmia in EEG. Several days after starting low dose ACTH treatment, a systolic murmur due to biventricular outflow tract obstruction intensified with ventricular arrhythmia. A tumor in the left ventricular outflow tract was enlarged. Discontinuation of daily administration of ACTH and administration of a beta-blocking agent improved the cardiac problems. Acceleration of blood velocity in the outflow tracts disappeared two months later. Intracardiac morphology and hemodynamic performance should be monitored to avoid critical complications during ACTH treatment in patients with tuberous sclerosis.