[Cardiac involvement in tuberous sclerosis in the first months of life: physiopathologic and clinical aspects of cardiac rhabdomyoma].
Cardiac rhabdomyomas are frequent in association with tuberous sclerosis and they are the first symptoms of Bourneville disease in fetal and neonatal period. Clinical findings of cardiac rhabdomiomas are quite heterogeneous: asymptomatic, cardiac murmur, cardiomegaly, heart failure or arrhythmias. Echocardiography can determine site, dimensions, numbers and haemodynamic consequences of cardiac tumours and their clinical evolution at follow-up. In this study the Authors report clinical findings in 9 cases of cardiac rhabdomyomas in newborn infants: 3 has familiarity for tuberous sclerosis and two of these had prenatal echocardiographic diagnosis; in the other 6 cases diagnosis was casual (1 in fetal period). All patients with prenatal diagnosis were asymptomatic. In other cases 1 had extrasystolic arrhythmias and five had only cardiac murmurs. Echocardiographic follow-up (6 months to 5 years) showed regression of number and dimension of cardiac masses in all cases. In 7 cases cardiac rhabdomiomas were associated with tuberous sclerosis.
Distefano
G
G
Cattedra di Neonatologia, Università di Catania, Italia.
Sciacca
P
P
Mattia
C
C
Tornambene
G
G
D004452:Echocardiography; D005260:Female; D006331:Heart Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D012207:Rhabdomyoma / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D018233:Rhabdomyosarcoma, Embryonal / Q000000981:diagnostic imaging / Q000503:physiopathology; D014402:Tuberous Sclerosis / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D016216:Ultrasonography, Prenatal
Il coinvolgimento cardiaco nella sclerosi tuberosa nei primi mesi di vita: aspetti fisiopatologici e clinici dei rabdomiomi cardiaci.