OBJECTIVE: To evaluate the incidence and clinical features of abnormal cardiac hypertrophy during treatment of infantile spasms with corticotropin. DESIGN: A prospective, echocardiographic study. PARTICIPANTS: Eighteen children treated for infantile spasms with high-dose corticotropin at a large tertiary children's hospital. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Abnormal cardiac hypertrophy was seen in 13 (72%) of 18 patients. Five of 18 patients (group 1) developed hypertrophic cardiomyopathy with asymmetric septal hypertrophy; none developed significant left ventricular outflow obstruction. Concentric left ventricular hypertrophy was seen in eight patients (group 2), whereas no changes were observed in five (group 3). CONCLUSION: Abnormal ventricular hypertrophy occurs in the majority of patients treated with corticotropin for infantile spasms. In our study, many of these patients developed hypertrophic cardiomyopathy with dramatic asymmetric septal hypertrophy; however, none developed significant obstruction to left ventricular outflow. All changes were reversible, and did not require premature discontinuation of therapy.
OBJECTIVE: To evaluate the incidence and clinical features of abnormal cardiac hypertrophy during treatment of infantile spasms with corticotropin. DESIGN: A prospective, echocardiographic study. PARTICIPANTS: Eighteen children treated for infantile spasms with high-dose corticotropin at a large tertiary children's hospital. INTERVENTIONS: None. MEASUREMENTS AND RESULTS:Abnormal cardiac hypertrophy was seen in 13 (72%) of 18 patients. Five of 18 patients (group 1) developed hypertrophic cardiomyopathy with asymmetric septal hypertrophy; none developed significant left ventricular outflow obstruction. Concentric left ventricular hypertrophy was seen in eight patients (group 2), whereas no changes were observed in five (group 3). CONCLUSION:Abnormal ventricular hypertrophy occurs in the majority of patients treated with corticotropin for infantile spasms. In our study, many of these patients developed hypertrophic cardiomyopathy with dramatic asymmetric septal hypertrophy; however, none developed significant obstruction to left ventricular outflow. All changes were reversible, and did not require premature discontinuation of therapy.
Authors: Rachel D Torok; Stephanie L Austin; Chanika Phornphutkul; Kathleen M Rotondo; Deeksha Bali; Gregory H Tatum; Stephanie B Wechsler; Anne F Buckley; Priya S Kishnani Journal: J Inherit Metab Dis Date: 2017-08-11 Impact factor: 4.982